Quarterly Review of Literature 3rd & 4th Quarter 2016

Table of Contents Long QT Syndrome (LQTS) ...... 2 Acquired Long QT Syndrome ...... 10 BRS – (BrS) ...... 13 Catecholaminergic Polymorphic Ventricular (CPVT) ...... 21 / ...... 23 Implantable Cardioverter Defibrillator (ICD) ...... 27 Sudden Cardiac Death (SCD) ...... 32 Screening ...... 37 CPR ...... 39 Automated External Defibrillator (AED) ...... 40 Hypertrophic (HCM) ...... 42 Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/ARVC) ...... 46 (TS) ...... 49 Short QT (SQT) ...... 49 Epilepsy ...... 50 Exercise ...... 50 Genetics & ...... 50 ...... 51 Miscellaneous ...... 51

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 1 Quarterly Review of Literature 3rd & 4th Quarter 2016

Long QT Syndrome (LQTS)

Difficult management of Jervell and Lange-Nielsen syndrome: An endless search. Schwartz PJ. Rhythm. 2016 Nov;13(11):2193-2194. doi: 10.1016/j.hrthm.2016.08.019. No abstract available. PMID:27520542

Beta-blocker therapy for long QT syndrome and catecholaminergic polymorphic : Are all beta- blockers equivalent? Ackerman MJ, Priori SG, Dubin A, Kowey P, Linker NJ, Slotwiner D, Triedman J, Van Hare GF, Gold MR. Heart Rhythm. 2016 Sep 19. pii: S1547-5271(16)30760-3. doi: 10.1016/j.hrthm.2016.09.012. [Epub ahead of print] No abstract available. PMID:27659101

Clinical and genetic features of Australian families with long QT syndrome: A registry-based study. Burns C, Ingles J, Davis AM, Connell V, Gray B, Hunt L, McGaughran J, Semsarian C. J Arrhythm. 2016 Dec;32(6):456-461. PMID:27920829 | PMCID:PMC5129121

Systematic ajmaline challenge in patients with long QT 3 syndrome caused by the most common mutation: a multicentre study. Hohmann S, Rudic B, Konrad T, Duncker D, König T, Tülümen E, Rostock T, Borggrefe M, Veltmann C. Europace. 2016 Dec 2. pii: euw214. [Epub ahead of print] PMID:27915266

Ventricular tachycardia and prolonged QT interval presenting as seizure-like activity: A case report. Wentlandt M, Morris SC, Mitchell SH. Am J Emerg Med. 2016 Nov 22. pii: S0735-6757(16)30879-8. doi: 10.1016/j.ajem.2016.11.051. [Epub ahead of print] PMID:27914889

In brief: PPIs and . [No authors listed] Med Lett Drugs Ther. 2016 Dec 5;58(1509):153. No abstract available. PMID:27906148

Long QT Syndrome and Duodenal Ampullary Adenoma: A New Association. Asad-Ur-Rahman FN, Hughes L, Talha Khan M, Khalid Hasan M, Inayat I. ACG Case Rep J. 2016 Nov 23;3(4):e163. PMID:27921062 | PMCID:PMC5126501

Reproducibility of corrected QT interval in pediatric genotyped long QT syndrome. Ogawa Y, Tanaka T, Kido S. Pediatr Int. 2016 Nov;58(11):1246-1248. doi: 10.1111/ped.13120. PMID:27882731

Considerations when using next-generation sequencing for genetic diagnosis of long-QT syndrome in the clinical testing laboratory. Chae H, Kim J, Lee GD, Jang W, Park J, Jekarl DW, Oh YS, Kim M, Kim Y. Clin Chim Acta. 2016 Nov 18;464:128-135. doi: 10.1016/j.cca.2016.11.013. [Epub ahead of print] PMID:27871843

An elderly Jervell and Lange-Nielsen patient heterozygous compound for two new KCNQ1 mutations. Coto E, García-Fernández FJ, Calvo D, Salgado-Aranda R, Martín-González J, Alonso B, Iglesias S, Gómez J. Am J Med Genet A. 2016 Nov 21. doi: 10.1002/ajmg.a.38062. [Epub ahead of print] PMID:27868350

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 2 Quarterly Review of Literature 3rd & 4th Quarter 2016

Management of Patients with Long QT Syndrome. Cho Y. Korean Circ J. 2016 Nov;46(6):747-752. Review. PMID:27826330 | PMCID:PMC5099327

Contribution of a KCNH2 variant in genotyped long QT syndrome: Romano-Ward syndrome under double mutations and acquired long QT syndrome under heterozygote. Fujii Y, Matsumoto Y, Hayashi K, Ding WG, Tomita Y, Fukumoto D, Wada Y, Ichikawa M, Sonoda K, Ozawa J, Makiyama T, Ohno S, Yamagishi M, Matsuura H, Horie M, Itoh H. J Cardiol. 2016 Nov 2. pii: S0914-5087(16)30219-2. doi: 10.1016/j.jjcc.2016.09.010. [Epub ahead of print] PMID:27816319

Induction of autoimmune response to the extracellular loop of the HERG channel pore induces QTc prolongation in guinea-pigs. Fabris F, Yue Y, Qu Y, Chahine M, Sobie E, Lee P, Wieczorek R, Jiang XC, Capecchi PL, Laghi-Pasini F, Lazzerini PE, Boutjdir M. J Physiol. 2016 Nov 1;594(21):6175-6187. doi: 10.1113/JP272151. PMID:27296897 | PMCID:PMC5088251

Readthrough-Promoting Drugs Gentamicin and PTC124 Fail to Rescue Nav1.5 Function of Human-Induced Pluripotent Stem Cell-Derived Cardiomyocytes Carrying Nonsense Mutations in the Sodium Channel Gene SCN5A. Kosmidis G, Veerman CC, Casini S, Verkerk AO, van de Pas S, Bellin M, Wilde AA, Mummery CL, Bezzina CR. Circ Arrhythm Electrophysiol. 2016 Nov;9(11). pii: e004227. PMID:27784737

Fatty acid analogue N-arachidonoyl taurine restores function of I(Ks) channels with diverse long QT mutations. Liin SI, Larsson JE, Barro-Soria R, Bentzen BH, Larsson HP. Elife. 2016 Sep 30;5. pii: e20272. doi: 10.7554/eLife.20272. PMID:27690226 | PMCID:PMC5081249

Experience with bisoprolol in long-QT1 and long-QT2 syndrome. Steinberg C, Padfield GJ, Al-Sabeq B, Adler A, Yeung-Lai-Wah JA, Kerr CR, Deyell MW, Andrade JG, Bennett MT, Yee R, Klein GJ, Green M, Laksman ZW, Krahn AD, Chakrabarti S. J Interv Card Electrophysiol. 2016 Nov;47(2):163-170. PMID:27394160

The Jervell and Lange-Nielsen syndrome; atrial pacing combined with ß-blocker therapy, a favorable approach in young high-risk patients with long QT syndrome? Früh A, Siem G, Holmström H, Døhlen G, Haugaa KH. Heart Rhythm. 2016 Nov;13(11):2186-2192. doi: 10.1016/j.hrthm.2016.07.020. PMID:27451284

Convergence of models of human ventricular myocyte electrophysiology after global optimization to recapitulate clinical long QT phenotypes. Mann SA, Imtiaz M, Winbo A, Rydberg A, Perry MD, Couderc JP, Polonsky B, McNitt S, Zareba W, Hill AP, Vandenberg JI. J Mol Cell Cardiol. 2016 Sep 20;100:25-34. doi: 10.1016/j.yjmcc.2016.09.011. [Epub ahead of print] PMID:27663173

A Precision Medicine Approach to the Rescue of Function on Malignant Calmodulinopathic Long QT Syndrome. Limpitikul WB, Dick IE, Tester D, Boczek NJ, Limphong P, Yang W, Choi MH, Babich J, DiSilvestre D, Kanter RJ, Tomaselli GF, Ackerman MJ, Yue D. Circ Res. 2016 Oct 20. pii: CIRCRESAHA.116.309283. [Epub ahead of print] PMID:27765793

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 3 Quarterly Review of Literature 3rd & 4th Quarter 2016

Spontaneous initiation of premature ventricular complexes and in type 2 long QT syndrome. Huang X, Kim TY, Koren G, Choi BR, Qu Z. Am J Physiol Heart Circ Physiol. 2016 Dec 1;311(6):H1470-H1484. doi: 10.1152/ajpheart.00500.2016. PMID:27765749

Characterization of the novel mutant A78T-HERG from a long QT syndrome type 2 patient: Instability of the mutant protein and stabilization by heat shock factor 1. Kondo T, Hisatome I, Yoshimura S, Mahati E, Notsu T, Li P, Iitsuka K, Kato M, Ogura K, Miake J, Aiba T, Shimizu W, Kurata Y, Sakata S, Nakasone N, Ninomiya H, Nakai A, Higaki K, Kawata Y, Shirayoshi Y, Yoshida A, Yamamoto K. J Arrhythm. 2016 Oct;32(5):433-440. PMID:27761169 | PMCID:PMC5063263

Molecular pathogenesis of long QT syndrome type 1. Wu J, Ding WG, Horie M. J Arrhythm. 2016 Oct;32(5):381-388. Review. PMID:27761162 | PMCID:PMC5063268

Molecular pathogenesis of long QT syndrome type 2. Smith JL, Anderson CL, Burgess DE, Elayi CS, January CT, Delisle BP. J Arrhythm. 2016 Oct;32(5):373-380. Review. PMID:27761161 | PMCID:PMC5063260

A type 2 ryanodine receptor variant associated with reduced Ca(2+) release and short-coupled torsades de pointes ventricular arrhythmia. Fujii Y, Itoh H, Ohno S, Murayama T, Kurebayashi N, Aoki H, Blancard M, Nakagawa Y, Yamamoto S, Matsui Y, Ichikawa M, Sonoda K, Ozawa T, Ohkubo K, Watanabe I, Guicheney P, Horie M. Heart Rhythm. 2016 Oct 15. pii: S1547-5271(16)30916-X. doi: 10.1016/j.hrthm.2016.10.015. [Epub ahead of print] PMID:27756708

Long QT syndrome and torsades de pointes complicating replacement. Gilbert S, Singh D, Jesuraj ML. Indian Heart J. 2016 Sep;68 Suppl 2:S210-S211. doi: 10.1016/j.ihj.2015.07.015. PMID:27751291 | PMCID:PMC5067385

Genetic biomarkers for the risk of seizures in long QT syndrome. Auerbach DS, McNitt S, Gross RA, Zareba W, Dirksen RT, Moss AJ. Neurology. 2016 Oct 18;87(16):1660-1668. PMID:27466471 | PMCID:PMC5085072

Finding a Needle in a QT Interval Big Data Haystack: The Role for Orthogonal Datasets. Roden DM, Mosley JD, Denny JC. J Am Coll Cardiol. 2016 Oct 18;68(16):1765-1768. doi: 10.1016/j.jacc.2016.08.012. No abstract available. PMID:27737743

Ranolazine for Congenital Long-QT Syndrome Type III: Experimental and Long-Term Clinical Data. Chorin E, Hu D, Antzelevitch C, Hochstadt A, Belardinelli L, Zeltser D, Barajas-Martinez H, Rozovski U, Rosso R, Adler A, Benhorin J, Viskin S. Circ Arrhythm Electrophysiol. 2016 Oct;9(10). pii: e004370. PMID:27733495 | PMCID:PMC5119553

QTc interval lengthening in first-episode schizophrenia (FES) patients in the earliest stages of antipsychotic treatment. Zhai D, Lang Y, Dong G, Liu Y, Wang X, Zhou D, Cui T, Yang Y, Zhang W, Zhao Y, Zhang R. Schizophr Res. 2016 Oct 7. pii: S0920-9964(16)30449-2. doi: 10.1016/j.schres.2016.10.006. [Epub ahead of print] PMID:27727006

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 4 Quarterly Review of Literature 3rd & 4th Quarter 2016

Antiarrhythmic effect of vernakalant in an experimental model of Long-QT-syndrome. Frommeyer G, Clauss C, Ellermann C, Bogossian H, Dechering DG, Kochhäuser S, Reinke F, Pott C, Eckardt L. Europace. 2016 Oct 4. pii: euw182. [Epub ahead of print] PMID:27702859

Early somatic mosaicism is a rare cause of long-QT syndrome. Priest JR, Gawad C, Kahlig KM, Yu JK, O'Hara T, Boyle PM, Rajamani S, Clark MJ, Garcia ST, Ceresnak S, Harris J, Boyle S, Dewey FE, Malloy-Walton L, Dunn K, Grove M, Perez MV, Neff NF, Chen R, Maeda K, Dubin A, Belardinelli L, et al. Proc Natl Acad Sci U S A. 2016 Oct 11;113(41):11555-11560. PMID:27681629 | PMCID:PMC5068256

Clinical pharmacists' opportunities to reduce inappropriate prescription of QT-prolonging : calls to action. Dhanani TC, Mantovani EH, Turner JR. Int J Pharm Pract. 2016 Sep 28. doi: 10.1111/ijpp.12303. [Epub ahead of print] PMID:27677250

Epidural Analgesia with Ropivacaine during Labour in a Patient with a SCN5A Gene Mutation. van der Knijff-van Dortmont AL, Dirckx M, Duvekot JJ, Roos-Hesselink JW, Gonzalez Candel A, van der Marel CD, Scoones GP, Adriaens VF, Dons-Sinke IJ. Case Rep Anesthesiol. 2016;2016:9278409. doi: 10.1155/2016/9278409. PMID:27668095 | PMCID:PMC5030394

The Effects of Pharmacological Compounds on Beat Rate Variations in Human Long QT-Syndrome Cardiomyocytes. Kuusela J, Kim J, Räsänen E, Aalto-Setälä K. Stem Cell Rev. 2016 Dec;12(6):698-707. PMID:27646833 | PMCID:PMC5106508

Variants in the SCN5A Promoter Associated With Various Arrhythmia Phenotypes. Yagihara N, Watanabe H, Barnett P, Duboscq-Bidot L, Thomas AC, Yang P, Ohno S, Hasegawa K, Kuwano R, Chatel S, Redon R, Schott JJ, Probst V, Koopmann TT, Bezzina CR, Wilde AA, Nakano Y, Aiba T, Miyamoto Y, Kamakura S, Darbar D, Donahue BS, et al. J Am Heart Assoc. 2016 Sep 13;5(9). pii: e003644. doi: 10.1161/JAHA.116.003644. PMID:27625342 | PMCID:PMC5079027

Mutation analysis for the detection of long QT-syndrome (LQTS) associated SNPs. J E, T D, M S, M KN, J D, M NW. Int J Legal Med. 2016 Sep 9. [Epub ahead of print] PMID:27613431

Long QT syndrome and life-threatening anaphylaxis: Is epinephrine safe? White JL, Kahoud RJ, Li JT, Campbell RL, Ackerman MJ. Ann Allergy Asthma Immunol. 2016 Oct;117(4):444-446. doi: 10.1016/j.anai.2016.08.004. No abstract available. PMID:27590635

Clinical Aspects of Type 3 Long-QT Syndrome: An International Multicenter Study. Wilde AA, Moss AJ, Kaufman ES, Shimizu W, Peterson DR, Benhorin J, Lopes C, Towbin JA, Spazzolini C, Crotti L, Zareba W, Goldenberg I, Kanters JK, Robinson JL, Qi M, Hofman N, Tester DJ, Bezzina CR, Alders M, Aiba T, Kamakura S, Miyamoto Y, et al. Circulation. 2016 Sep 20;134(12):872-82. doi: 10.1161/CIRCULATIONAHA.116.021823. PMID:27566755 | PMCID:PMC5030177

A new experimentally validated formula to calculate the QT interval in the presence of left holds true in the clinical setting. Bogossian H, Frommeyer G, Ninios I, Pechlivanidou E, Hasan F, Nguyen QS, Mijic D, Kloppe A, Karosiene Z, Margkarian A, Bandorski D, Schultes D, Erkapic D, Seyfarth M, Lemke B, Eckardt L, Zarse M. Ann Noninvasive Electrocardiol. 2016 Aug 26. doi: 10.1111/anec.12393. [Epub ahead of print] PMID:27562181

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 5 Quarterly Review of Literature 3rd & 4th Quarter 2016

miRNAs Regulate hERG. Lian J, Guo J, Huang X, Yang XI, Huang G, Mao H, Sun HH, Ba Y, Zhou J. J Cardiovasc Electrophysiol. 2016 Dec;27(12):1472-1482. doi: 10.1111/jce.13084. PMID:27558843

Sevoflurane-associated torsade de pointes in a patient with congenital long QT syndrome genotype 2. Kumakura M, Hara K, Sata T. J Clin Anesth. 2016 Sep;33:81-5. doi: 10.1016/j.jclinane.2016.03.011. PMID:27555138

Is Symptomatic Long QT Syndrome Associated with Depression in Women and Men? Wesołowska K, Elovainio M, Koponen M, Tuiskula AM, Hintsanen M, Keltikangas-Järvinen L, Määttänen I, Swan H, Hintsa T. J Genet Couns. 2016 Aug 23. [Epub ahead of print] PMID:27553078

Early and risk of arrhythmia events in long QT syndrome. Hasegawa K, Watanabe H, Hisamatsu T, Ohno S, Itoh H, Ashihara T, Hayashi H, Makiyama T, Minamino T, Horie M. Int J Cardiol. 2016 Nov 15;223:540-542. doi: 10.1016/j.ijcard.2016.08.215. No abstract available. PMID:27552577

The natural history of fetal long QT syndrome. Cuneo BF, Strasburger JF, Wakai RT. J Electrocardiol. 2016 Jul 28. pii: S0022-0736(16)30101-7. doi: 10.1016/j.jelectrocard.2016.07.023. [Epub ahead of print] PMID:27539165

Genetic Modifiers for the Long-QT Syndrome: How Important Is the Role of Variants in the 3' Untranslated Region of KCNQ1? Crotti L, Lahtinen AM, Spazzolini C, Mastantuono E, Monti MC, Morassutto C, Parati G, Heradien M, Goosen A, Lichtner P, Meitinger T, Brink PA, Kontula K, Swan H, Schwartz PJ. Circ Cardiovasc Genet. 2016 Aug;9(4):330-9. doi: 10.1161/CIRCGENETICS.116.001419. PMID:27531917

B-Blocker Adherence in Familial Long QT Syndrome. Waddell-Smith KE, Li J, Smith W, Crawford J, Skinner JR; Cardiac Inherited Disease Group New Zealand. Circ Arrhythm Electrophysiol. 2016 Aug;9(8). pii: e003591. doi: 10.1161/CIRCEP.115.003591. PMID:27516460

T-wave morphology can distinguish healthy controls from LQTS patients. Immanuel SA, Sadrieh A, Baumert M, Couderc JP, Zareba W, Hill AP, Vandenberg JI. Physiol Meas. 2016 Sep;37(9):1456-73. doi: 10.1088/0967-3334/37/9/1456. PMID:27510854

A multidisciplinary approach to prenatal treatment of congenital long QT syndrome. Crimmins S, Vashit S, Doyle L, Harman C, Turan O, Turan S. J Clin Ultrasound. 2016 Aug 5. doi: 10.1002/jcu.22386. [Epub ahead of print] PMID:27492745

KCNE4 and KCNE5: K(+) channel regulation and cardiac arrhythmogenesis. Abbott GW. Gene. 2016 Nov 30;593(2):249-60. doi: 10.1016/j.gene.2016.07.069. Review. PMID:27484720

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 6 Quarterly Review of Literature 3rd & 4th Quarter 2016

Evaluation and Management of Athletes With Long QT Syndrome: An Evolved Paradigm. Gomez AT, Prutkin JM, Rao AL. Sports Health. 2016 Aug 1. pii: 1941738116660294. [Epub ahead of print] PMID:27480102 | PMCID:PMC5089354 hERG long QT syndrome type 2 mutants need more than a chaperone to dance. Fedida D, Macdonald L. J Physiol. 2016 Aug 1;594(15):4095-6. doi: 10.1113/JP272417. No abstract available. PMID:27477605 | PMCID:PMC4967728

Long QT Manifested as Giant and Inverted T Waves With a SCN5A Mutation. Suarez K, Blazo M, Banchs JE. J Cardiovasc Electrophysiol. 2016 Dec;27(12):1490-1491. doi: 10.1111/jce.13053. No abstract available. PMID:27471188

A Neonate with Susceptibility to Long QT Syndrome Type 6 who Presented with Ventricular and Sudden Unexpected Infant Death. Sauer CW, Marc-Aurele KL. Am J Case Rep. 2016 Jul 28;17:544-8. PMID:27465075 | PMCID:PMC4968432

Coexistence of congenital long QT syndrome and autonomic dysregulation in children. Ogawa Y, Aiba T, Kamei N, Tominaga K, Fujita H, Miyamoto Y, Tanaka T, Kido S. Pediatr Int. 2016 Jul;58(7):672-4. doi: 10.1111/ped.13015. PMID:27460404

The role of known variants of KCNQ1, KCNH2, KCNE1, SCN5A, and NOS1AP in water-related deaths. Tzimas I, Zingraf JC, Bajanowski T, Poetsch M. Int J Legal Med. 2016 Nov;130(6):1575-1579. PMID:27460199

The QT Scale: A Weight Scale Measuring the QTc Interval. Couderc JP, Beshaw C, Niu X, Serrano-Finetti E, Casas O, Pallas-Areny R, Rosero S, Zareba W. Ann Noninvasive Electrocardiol. 2016 Jul 15. doi: 10.1111/anec.12378. [Epub ahead of print] PMID:27422673

Identification of Concealed and Manifest Long QT Syndrome Using a Novel Analysis Program. Sugrue A, Noseworthy PA, Kremen V, Bos JM, Qiang B, Rohatgi RK, Sapir Y, Attia ZI, Brady P, Asirvatham SJ, Friedman PA, Ackerman MJ. Circ Arrhythm Electrophysiol. 2016 Jul;9(7). pii: e003830. doi: 10.1161/CIRCEP.115.003830. PMID:27406603

Prominent QTc prolongation in a patient with a rare variant in the cardiac ryanodine receptor gene. Taniguchi Y, Miyazaki A, Sakaguchi H, Hayama Y, Ebishima N, Negishi J, Noritake K, Miyamoto Y, Shimizu W, Aiba T, Ohuchi H. Heart Vessels. 2016 Jul 11. [Epub ahead of print] PMID:27401738

Calcium Revisited: New Insights Into the Molecular Basis of Long-QT Syndrome. Giudicessi JR, Ackerman MJ. Circ Arrhythm Electrophysiol. 2016 Jul;9(7). pii: e002480. doi: 10.1161/CIRCEP.116.002480. No abstract available. PMID:27390209

Improved Clinical Risk Stratification in Patients with Long QT Syndrome? Novel Insights from Multi-Channel ECGs. Samol A, Gönes M, Zumhagen S, Bruns HJ, Paul M, Vahlhaus C, Waltenberger J, Schulze-Bahr E, Eckardt L, Mönnig G. PLoS One. 2016 Jul 5;11(7):e0158085. doi: 10.1371/journal.pone.0158085. PMID:27379800 | PMCID:PMC4933388

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 7 Quarterly Review of Literature 3rd & 4th Quarter 2016

Novel calmodulin mutations associated with congenital long QT syndrome affect calcium current in human cardiomyocytes. Pipilas DC, Johnson CN, Webster G, Schlaepfer J, Fellmann F, Sekarski N, Wren LM, Ogorodnik KV, Chazin DM, Chazin WJ, Crotti L, Bhuiyan ZA, George AL Jr. Heart Rhythm. 2016 Oct;13(10):2012-9. doi: 10.1016/j.hrthm.2016.06.038. PMID:27374306 | PMCID:PMC5035189

Patient Recall, Interpretation, and Perspective of an Inconclusive Long QT Syndrome Genetic Test Result. Predham S, Hathaway J, Hulait G, Arbour L, Lehman A. J Genet Couns. 2016 Jul 2. [Epub ahead of print] PMID:27369690

TECRL, a new life-threatening inherited arrhythmia gene associated with overlapping clinical features of both LQTS and CPVT. Devalla HD, Gélinas R, Aburawi EH, Beqqali A, Goyette P, Freund C, Chaix MA, Tadros R, Jiang H, Le Béchec A, Monshouwer-Kloots JJ, Zwetsloot T, Kosmidis G, Latour F, Alikashani A, Hoekstra M, Schlaepfer J, Mummery CL, Stevenson B, Kutalik Z, de Vries AA, Rivard L, et al. EMBO Mol Med. 2016 Dec 1;8(12):1390-1408. doi: 10.15252/emmm.201505719. PMID:27861123

Potassium Channelopathies and Gastrointestinal Ulceration. Han J, Lee SH, Giebisch G, Wang T. Gut Liver. 2016 Nov 15;10(6):881-889. doi: 10.5009/gnl15414. Review. PMID:27784845 | PMCID:PMC5087926

A new hERG allosteric modulator rescues genetic and drug-induced long-QT syndrome phenotypes in cardiomyocytes from isogenic pairs of patient induced pluripotent stem cells. Sala L, Yu Z, Ward-van Oostwaard D, van Veldhoven JP, Moretti A, Laugwitz KL, Mummery CL, IJzerman AP, Bellin M. EMBO Mol Med. 2016 Sep 1;8(9):1065-81. doi: 10.15252/emmm.201606260. PMID:27470144 | PMCID:PMC5009811

The Promise and Peril of Precision Medicine: Phenotyping Still Matters Most. Ackerman JP, Bartos DC, Kapplinger JD, Tester DJ, Delisle BP, Ackerman MJ. Mayo Clin Proc. 2016 Oct 8. pii: S0025-6196(16)30463-3. doi: 10.1016/j.mayocp.2016.08.008. [Epub ahead of print] Review. PMID:27810088

Electrocardiographic surveillance in a psychiatric institution: avoiding iatrogenic cardiovascular death. Rodríguez-Leal CM, López-Lunar E, Carrascosa-Bernáldez JM, Provencio-Arranz RM. Int J Psychiatry Clin Pract. 2016 Sep 30:1-3. [Epub ahead of print] PMID:27686282

Female gender as independent risk factor of torsades de pointes during acquired . Chorin E, Hochstadt A, Viskin S, Rozovski U, Havakuk O, Baranchuk A, Enriquez A, Strasberg B, Guevara-Valdivia ME, Márquez MF, González-Pacheco H, Hasdemir C, Rosso R. Heart Rhythm. 2016 Sep 17. pii: S1547-5271(16)30796-2. doi: 10.1016/j.hrthm.2016.09.013. [Epub ahead of print] PMID:27650425

Elevated local [Ca2+] and CaMKII promote spontaneous Ca2+ release in ankyrin-B-deficient . Popescu I, Galice S, Mohler PJ, Despa S. Cardiovasc Res. 2016 Aug 1;111(3):287-94. doi: 10.1093/cvr/cvw093. PMID:27131508 | PMCID:PMC4957489

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 8 Quarterly Review of Literature 3rd & 4th Quarter 2016

Update on the Diagnosis and Management of Familial Long QT Syndrome. Waddell-Smith KE, Skinner JR; members of the CSANZ Genetics Council Writing Group.. Heart Lung Circ. 2016 Aug;25(8):769-76. doi: 10.1016/j.hlc.2016.01.020. PMID:27262388

The evolution of sports participation guidelines and the influence of genotype-phenotype correlation in long QT syndrome. Furst ML, Aziz PF. Trends Cardiovasc Med. 2016 Nov;26(8):690-697. doi: 10.1016/j.tcm.2016.04.013. Review. PMID:27260221

Semiconductor Whole Exome Sequencing for the Identification of Genetic Variants in Colombian Patients Clinically Diagnosed with Long QT Syndrome. Burgos M, Arenas A, Cabrera R. Mol Diagn Ther. 2016 Aug;20(4):353-62. doi: 10.1007/s40291-016-0207-2. PMID:27251404

Transgenic rabbit models to investigate the cardiac disease long QT syndrome. Lang CN, Koren G, Odening KE. Prog Biophys Mol Biol. 2016 Jul;121(2):142-56. doi: 10.1016/j.pbiomolbio.2016.05.004. Review. PMID:27210307

Quinine and the ABCs of Long QT: A Patient's Misfortune with Arthritis, (Alcoholic) Beverages, and Cramps. Sheehan ET, Frizzell JD, Gabaldon J, West MB. J Gen Intern Med. 2016 Oct;31(10):1254-7. doi: 10.1007/s11606-016-3738-7. PMID:27173501 | PMCID:PMC5023602

A neonatal prolonged QT syndrome due to maternal use of oral tricyclic antidepressants. Fukushima N, Nanao K, Fukushima H, Namera A, Miura M. Eur J Pediatr. 2016 Aug;175(8):1129-32. doi: 10.1007/s00431-016-2722-x. PMID:27068648

Probability of diagnosing long QT syndrome in children and adolescents according to the criteria of the HRS/EHRA/APHRS expert consensus statement. Yoshinaga M, Kucho Y, Nishibatake M, Ogata H, Nomura Y. Eur Heart J. 2016 Aug;37(31):2490-7. doi: 10.1093/eurheartj/ehw072. PMID:27026747

Rescue of protein expression defects may not be enough to abolish the pro-arrhythmic phenotype of long QT type 2 mutations. Perry MD, Ng CA, Phan K, David E, Steer K, Hunter MJ, Mann SA, Imtiaz M, Hill AP, Ke Y, Vandenberg JI. J Physiol. 2016 Jul 15;594(14):4031-49. doi: 10.1113/JP271805. PMID:26958806 | PMCID:PMC4945714

The impact of recent advances in genetics in understanding disease mechanisms underlying the long QT syndromes. Harmer SC, Tinker A. Biol Chem. 2016 Jul 1;397(7):679-93. doi: 10.1515/hsz-2015-0306. PMID:26910742

Effect of Left Cardiac Sympathetic Denervation on the Electromechanical Window in Patients with either Type 1 or Type 2 Long QT Syndrome: A Pilot Study. Schneider AE, Bos JM, Ackerman MJ. Congenit Heart Dis. 2016 Sep;11(5):437-443. doi: 10.1111/chd.12332. PMID:26887900

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 9 Quarterly Review of Literature 3rd & 4th Quarter 2016

Extracorporeal CPR and therapeutic hypothermia for out-of-hospital in a patient with congenital long QT syndrome. Lee JW, Yoo SW, Jeong WJ, Ryu S, Yoo YH, Yoo IS, Park SI, Kim JH. Am J Emerg Med. 2016 Jul;34(7):1321.e1-3. doi: 10.1016/j.ajem.2015.11.061. No abstract available. PMID:26698681

Asymmetry of parental origin in long QT syndrome: preferential maternal transmission of KCNQ1 variants linked to channel dysfunction. Itoh H, Berthet M, Fressart V, Denjoy I, Maugenre S, Klug D, Mizusawa Y, Makiyama T, Hofman N, Stallmeyer B, Zumhagen S, Shimizu W, Wilde AA, Schulze-Bahr E, Horie M, Tezenas du Montcel S, Guicheney P. Eur J Hum Genet. 2016 Aug;24(8):1160-6. doi: 10.1038/ejhg.2015.257. PMID:26669661 | PMCID:PMC4970673

The prevalence of long QT interval in post-operative intensive care unit patients. Pham JC, Banks MC, Narotsky DL, Dorman T, Winters BD. J Clin Monit Comput. 2016 Aug;30(4):437-43. doi: 10.1007/s10877-015-9736-1. PMID:26169292 Acquired Long QT Syndrome

Electrophysiological Characteristics of Human iPSC-Derived Cardiomyocytes for the Assessment of Drug-Induced Proarrhythmic Potential. Yamamoto W, Asakura K, Ando H, Taniguchi T, Ojima A, Uda T, Osada T, Hayashi S, Kasai C, Miyamoto N, Tashibu H, Yoshinaga T, Yamazaki D, Sugiyama A, Kanda Y, Sawada K, Sekino Y. PLoS One. 2016 Dec 6;11(12):e0167348. doi: 10.1371/journal.pone.0167348. PMID:27923051

Influence of steroid hormones on ventricular repolarization. Salem JE, Alexandre J, Bachelot A, Funck-Brentano C. Pharmacol Ther. 2016 Jul 22. pii: S0163-7258(16)30125-5. doi: 10.1016/j.pharmthera.2016.07.005. [Epub ahead of print] PMID:27452340

Editiorial Commentary: Genomics and drug discovery: The next frontier in precision medicine. Roden DM. Trends Cardiovasc Med. 2016 Sep 20. pii: S1050-1738(16)30148-7. doi: 10.1016/j.tcm.2016.09.004. [Epub ahead of print] No abstract available. PMID:27771237

Early drug development: assessment of proarrhythmic risk and cardiovascular safety. Lester RM, Olbertz J. Expert Rev Clin Pharmacol. 2016 Dec;9(12):1611-1618. PMID:27718759

Marked QTc Prolongation and Torsades de pointes in Patients with Chronic Inflammatory Arthritis. Lazzerini PE, Capecchi PL, Bertolozzi I, Morozzi G, Lorenzini S, Simpatico A, Selvi E, Bacarelli MR, Acampa M, Lazaro D, El-Sherif N, Boutjdir M, Laghi-Pasini F. Front Cardiovasc Med. 2016 Sep 21;3:31. PMID:27703966 | PMCID:PMC5029147

Effect of Ondansetron on QT Interval in Patients Cared for in the PICU. Trivedi S, Schiltz B, Kanipakam R, Bos JM, Ackerman MJ, Ouellette Y. Pediatr Crit Care Med. 2016 Jul;17(7):e317-23. doi: 10.1097/PCC.0000000000000776. PMID:27387786

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 10 Quarterly Review of Literature 3rd & 4th Quarter 2016

QT interval prolongation and torsades de pointes with oxaliplatin. Hancox JC, Caves RE, Choisy SC, James AF. Ther Adv Drug Saf. 2016 Dec;7(6):261-263. No abstract available. PMID:27904744 | PMCID:PMC5117153

The analysis of QT interval and repolarization morphology of the heart in chronic exposure to lead. Kiełtucki J, Dobrakowski M, Pawlas N, Średniawa B, Boroń M, Kasperczyk S. Hum Exp Toxicol. 2016 Nov 30. pii: 0960327116680277. [Epub ahead of print] PMID:27903879

Investigating the Additive Interaction of QT-Prolonging Drugs in Older People Using Claims Data. Meid AD, von Medem A, Heider D, Adler JB, Günster C, Seidling HM, Quinzler R, König HH, Haefeli WE. Drug Saf. 2016 Nov 29. [Epub ahead of print] PMID:27896662

Reckless administration of QT interval-prolonging agents in elderly patients with drug-induced torsade de pointes. Jackobson G, Carmel NN, Lotan D, Kremer A, Justo D. Z Gerontol Geriatr. 2016 Nov 22. [Epub ahead of print] PMID:27878411

Life threatening torsades de pointes due to abiraterone-induced hypokaelemia in a patient with metastatic prostate cancer. Khan A, Kneale B. N Z Med J. 2016 Nov 18;129(1445):124-127. PMID:27857247

Electrocardiographic Parameters Indicating Worse Evolution in Patients with Acquired Long QT Syndrome and Torsades de Pointes. Kukla P, Jastrzębski M, Fijorek K, Stec S, Bryniarski L, Czarnecka D, Baranchuk A. Ann Noninvasive Electrocardiol. 2016 Nov;21(6):572-579. doi: 10.1111/anec.12355. PMID:27018992

Phenytoin in Treatment of Methadone-Induced Torsades de Pointes: A Case Report. Altheeb Z, Alziadat M, Shamoon F. Am J Ther. 2016 Oct 12. [Epub ahead of print] No abstract available. PMID:27754988

Coupling Data Mining and Laboratory Experiments to Discover Drug Interactions Causing QT Prolongation. Lorberbaum T, Sampson KJ, Chang JB, Iyer V, Woosley RL, Kass RS, Tatonetti NP. J Am Coll Cardiol. 2016 Oct 18;68(16):1756-1764. doi: 10.1016/j.jacc.2016.07.761. PMID:27737742 | PMCID:PMC5082283

Time-to-Onset Analysis of Drug-Induced Long QT Syndrome Based on a Spontaneous Reporting System for Adverse Drug Events. Sasaoka S, Matsui T, Hane Y, Abe J, Ueda N, Motooka Y, Hatahira H, Fukuda A, Naganuma M, Hasegawa S, Kinosada Y, Nakamura M. PLoS One. 2016 Oct 10;11(10):e0164309. doi: 10.1371/journal.pone.0164309. PMID:27723808 | PMCID:PMC5056697

In vivo analysis of torsadogenic potential of an antipsychotic drug paliperidone using the acute atrioventricular block rabbit as a proarrhythmia model. Hagiwara M, Kambayashi R, Aimoto M, Nagasawa Y, Takahara A. J Pharmacol Sci. 2016 Sep;132(1):48-54. doi: 10.1016/j.jphs.2016.05.001. PMID:27262905 Cardiac Arrest in Pediatric Patients Receiving Azithromycin. Valdés SO, Kim JJ, Niu MC, de la Uz CM, Miyake CY, Moffett BS. J Pediatr. 2016 Nov 29. pii: S0022-3476(16)31240-9. doi: 10.1016/j.jpeds.2016.11.012. [Epub ahead of print] PMID:27912925

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 11 Quarterly Review of Literature 3rd & 4th Quarter 2016

Pharmacological Conversion of a Cardiac Inward Rectifier into an Outward Rectifier Channel. Moreno-Galindo EG, Sanchez-Chapula JA, Tristani-Firouzi M, Navarro-Polanco RA. Mol Pharmacol. 2016 Sep;90(3):334-40. doi: 10.1124/mol.116.104950. PMID:27247338

Exposure-QT analysis for sonidegib (LDE225), an oral inhibitor of the hedgehog signaling pathway, for measures of the QT prolongation potential in healthy subjects and in patients with advanced solid tumors. Quinlan M, Zhou J, Hurh E, Sellami D. Eur J Clin Pharmacol. 2016 Dec;72(12):1427-1432. PMID:27663457

Two Sudden and Unexpected Deaths of Patients with Schizophrenia Associated with Intramuscular Injections of Antipsychotics and Practice Guidelines to Limit the Use of High Doses of Intramuscular Antipsychotics. Wahidi N, Johnson KM, Brenzel A, de Leon J. Case Rep Psychiatry. 2016;2016:9406813. doi: 10.1155/2016/9406813. PMID:27597919 | PMCID:PMC5002457

Sex hormonal regulation of cardiac ion channels in drug-induced QT syndromes. Kurokawa J, Kodama M, Clancy CE, Furukawa T. Pharmacol Ther. 2016 Dec;168:23-28. doi: 10.1016/j.pharmthera.2016.09.004. Review. PMID:27595633 | PMCID:PMC5140718

Loperamide Induced Life Threatening Ventricular Arrhythmia. Upadhyay A, Bodar V, Malekzadegan M, Singh S, Frumkin W, Mangla A, Doshi K. Case Rep Cardiol. 2016;2016:5040176. doi: 10.1155/2016/5040176. PMID:27547470 | PMCID:PMC4980497

A computational model predicts adjunctive pharmacotherapy for cardiac safety via selective inhibition of the late cardiac Na current. Yang PC, El-Bizri N, Romero L, Giles WR, Rajamani S, Belardinelli L, Clancy CE. J Mol Cell Cardiol. 2016 Oct;99:151-161. doi: 10.1016/j.yjmcc.2016.08.011. PMID:27545042

Domperidone-Associated QT Interval Prolongation in Non-oncologic Pediatric Patients: A Review of the Literature. Morris AD, Chen J, Lau E, Poh J. Can J Hosp Pharm. 2016 May-Jun;69(3):224-30. Review. PMID:27403002 | PMCID:PMC4924943

ADMET Evaluation in Drug Discovery. 16. Predicting hERG Blockers by Combining Multiple Pharmacophores and Machine Learning Approaches. Wang S, Sun H, Liu H, Li D, Li Y, Hou T. Mol Pharm. 2016 Aug 1;13(8):2855-66. doi: 10.1021/acs.molpharmaceut.6b00471. PMID:27379394

A new paradigm for predicting risk of Torsades de Pointes during drug development: Commentary on: "Improved prediction of drug-induced Torsades de Pointes through simulations of dynamics and machine learning algorithms". McCauley MD, Darbar D. Clin Pharmacol Ther. 2016 Oct;100(4):324-6. doi: 10.1002/cpt.408. PMID:27301674

Prescribing pattern of antipsychotic drugs during the years 1996-2010: a population-based database study in Europe with a focus on torsadogenic drugs. Oteri A, Mazzaglia G, Pecchioli S, Molokhia M, Ulrichsen SP, Pedersen L, Poluzzi E, De Ponti F, Garbe E, Schink T, Herings R, Bezemer ID, Sturkenboom MC, Trifirò G. Br J Clin Pharmacol. 2016 Aug;82(2):487-97. doi: 10.1111/bcp.12955. PMID:27061849 | PMCID:PMC4972165

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 12 Quarterly Review of Literature 3rd & 4th Quarter 2016

High prevalence of risk factors in elderly patients using drugs associated with acquired torsades de pointes chronically in Colombia. Moreno-Gutiérrez PA, Gaviria-Mendoza A, Cañón MM, Machado-Alba JE. Br J Clin Pharmacol. 2016 Aug;82(2):504-11. doi: 10.1111/bcp.12969. PMID:27060989 | PMCID:PMC4972167

Drug-Induced QT/QTc Interval Shortening: Lessons from Drug-Induced QT/QTc Prolongation. Malik M. Drug Saf. 2016 Jul;39(7):647-59. doi: 10.1007/s40264-016-0411-3. Review. PMID:26968542

Improved Prediction of Drug-Induced Torsades de Pointes Through Simulations of Dynamics and Machine Learning Algorithms. Lancaster MC, Sobie EA. Clin Pharmacol Ther. 2016 Oct;100(4):371-9. doi: 10.1002/cpt.367. PMID:26950176

Secondly ECG recordings in the emergency room revealed Garenoxacin-induced abnormal QT interval prolongation in a patient with multiple syncopal attacks. Tagawa M, Ochiai S, Nakamura Y, Sato A, Chinushi M. Heart Vessels. 2016 Jul;31(7):1200-5. doi: 10.1007/s00380-015-0693-x. PMID:26022375

On the Potential of Preemptive Genotyping Towards Preventing -Related Adverse Events: Results from the South Korean National Health Insurance Database. Schildcrout JS, Denny JC, Roden DM. Drug Saf. 2016 Nov 21. [Epub ahead of print] No abstract available. PMID:27873192 BRS – Brugada Syndrome (BrS)

Pericarditis mimicking Brugada syndrome: A case report. Monti M, Olivi G, Francavilla F, Borgognoni F. Am J Emerg Med. 2016 Nov 9. pii: S0735-6757(16)30825-7. doi: 10.1016/j.ajem.2016.11.015. [Epub ahead of print] PMID:27863892

Low amplitude ECG and QRS fragmentation in provocable coved-type ST-segment elevation on surface ECG are strong predictors of a continuum between arrhythmogenic cardiomypathy and Brugada syndrome. Peters S. Int J Cardiol. 2016 Jul 1;214:148-50. doi: 10.1016/j.ijcard.2016.03.113. No abstract available. PMID:27061648

Transient left ventricular dysfunction during fever-induced Brugada-like electrocardiographic pattern. Stankovic I, Janicijevic A, Neskovic AN. Europace. 2016 Oct;18(10):1607. No abstract available. PMID:27009188

Simultaneous Non-Invasive Epicardial and Endocardial Mapping in Patients With Brugada Syndrome: New Insights Into Arrhythmia Mechanisms. Rudic B, Chaykovskaya M, Tsyganov A, Kalinin V, Tülümen E, Papavassiliu T, Dösch C, Liebe V, Kuschyk J, Röger S, El- Battrawy I, Akin I, Yakovleva M, Zaklyazminskaya E, Shestak A, Kim S, Chmelevsky M, Borggrefe M. J Am Heart Assoc. 2016 Nov 14;5(11). pii: e004095. PMID:27930354

Brugada syndrome: A merely ion or a structural heart disease or mixed? Jimmy Juang JM, Lin JL. Heart Rhythm. 2016 Dec 2. pii: S1547-5271(16)31160-2. doi: 10.1016/j.hrthm.2016.12.005. [Epub ahead of print] No abstract available. PMID:27919766

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 13 Quarterly Review of Literature 3rd & 4th Quarter 2016

Late gadolinium enhancement in Brugada syndrome: A marker for subtle underlying cardiomyopathy? Bastiaenen R, Cox AT, Castelletti S, Wijeyeratne YD, Colbeck N, Pakroo N, Ahmed H, Bunce N, Anderson L, Moon JC, Prasad S, Sharma S, Behr ER. Heart Rhythm. 2016 Dec 2. pii: S1547-5271(16)31159-6. doi: 10.1016/j.hrthm.2016.12.004. [Epub ahead of print] PMID:27919765

Brugada Syndrome:Risk Stratification And Management. Letsas Md Fesc KP, Georgopoulos Md S, Vlachos Md K, Karamichalakis Md N, Liatakis Md I, Korantzopoulos Md PhD P, Liu Md PhD T, Efremidis Md M, Sideris Md A. J Atr Fibrillation. 2016 Aug 31;9(2):1413. Review. PMID:27909531 | PMCID:PMC5129684

Management of Brugada Syndrome 2016: Should All High Risk Patients Receive an ICD? All High-Risk Patients Should Receive an Implantable Cardiac Defibrillator. Sieira J, Brugada P. Circ Arrhythm Electrophysiol. 2016 Nov;9(11). pii: e004195. No abstract available. PMID:27906652

Management of Brugada Syndrome 2016: Should All High Risk Patients Receive an ICD? Alternatives to Implantable Cardiac Defibrillator Therapy for Brugada Syndrome. Belhassen B. Circ Arrhythm Electrophysiol. 2016 Nov;9(11). pii: e004185. No abstract available. PMID:27906651

A case of Brugada syndrome which developed status epilepticus. Matsui M, Inoue K, Fujimura H, Sakoda S. Rinsho Shinkeigaku. 2016 Nov 25. [Epub ahead of print] PMID:27890882

Brugada Pattern Caused by a Overdose. Tessitore E, Ramlawi M, Tobler O, Sunthorn H. J Emerg Med. 2016 Nov 21. pii: S0736-4679(16)30928-3. doi: 10.1016/j.jemermed.2016.10.045. [Epub ahead of print] PMID:27884575

Near miss in a patient with undiagnosed Brugada syndrome: a case report and literature review. Alzahrani T. J Clin Anesth. 2016 Dec;35:427-429. doi: 10.1016/j.jclinane.2016.09.015. PMID:27871569

Brugada Syndrome. Brugada R, Campuzano O, Sarquella-Brugada G, Brugada P, Brugada J, Hong K. 2005 Mar 31 [updated 2016 Nov 17]. In: Pagon RA, Adam MP, Ardinger HH, Wallace SE, Amemiya A, Bean LJH, Bird TD, Ledbetter N, Mefford HC, Smith RJH, Stephens K, editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2016. PMID:20301690

Sodium channel blockade unmasked Brugada electrocardiographic pattern in a patient with complete right bundle branch block and early repolarization in the lateral leads. Konishi S, Minamiguchi H, Okuyama Y, Sakata Y. Clin Case Rep. 2016 Oct 7;4(11):1061-1064. PMID:27830074 | PMCID:PMC5093164

Modeling and Genome-Editing Brugada Syndrome in a Dish. Cho HC. J Am Coll Cardiol. 2016 Nov 8;68(19):2097-2098. doi: 10.1016/j.jacc.2016.09.911. No abstract available. PMID:27810049

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 14 Quarterly Review of Literature 3rd & 4th Quarter 2016

Patient-Specific and Genome-Edited Induced Pluripotent Stem Cell-Derived Cardiomyocytes Elucidate Single-Cell Phenotype of Brugada Syndrome. Liang P, Sallam K, Wu H, Li Y, Itzhaki I, Garg P, Zhang Y, Vermglinchan V, Lan F, Gu M, Gong T, Zhuge Y, He C, Ebert AD, Sanchez-Freire V, Churko J, Hu S, Sharma A, Lam CK, Scheinman MM, Bers DM, Wu JC. J Am Coll Cardiol. 2016 Nov 8;68(19):2086-2096. doi: 10.1016/j.jacc.2016.07.779. PMID:27810048

Pathogenesis and management of Brugada syndrome. Sieira J, Dendramis G, Brugada P. Nat Rev Cardiol. 2016 Dec;13(12):744-756. doi: 10.1038/nrcardio.2016.143. Review. PMID:27629507

Electrophysiological Mechanisms of Brugada Syndrome: Insights from Pre-clinical and Clinical Studies. Tse G, Liu T, Li KH, Laxton V, Chan YW, Keung W, Li RA, Yan BP. Front Physiol. 2016 Oct 18;7:467. Review. PMID:27803673 | PMCID:PMC5067537

Implantable cardioverter defibrillator and catheter ablation in Brugada syndrome. Pappone C, Santinelli V. J Cardiovasc Med (Hagerstown). 2016 Oct 31. [Epub ahead of print] No abstract available. PMID:27801684

Predictors of Ventricular Arrhythmias and Sudden Death in a Québec Cohort With Brugada Syndrome. Rivard L, Roux A, Nault I, Champagne J, Roux JF, Tadros R, Talajic M, Cadrin-Tourigny J, Shohoudi A, Mondésert B, Roy D, Macle L, Andrade J, Dyrda K, Dubuc M, Guerra PG, Sarrazin JF, Thibault B, Khairy P. Can J Cardiol. 2016 Nov;32(11):1355.e1-1355.e7. doi: 10.1016/j.cjca.2016.03.012. PMID:27378596

Rare Disease and Low Event Rates: Challenges for Refining Risk Stratification in Brugada Syndrome. Roberts JD, Klein GJ. Can J Cardiol. 2016 Nov;32(11):1294.e1-1294.e3. doi: 10.1016/j.cjca.2016.02.050. No abstract available. PMID:27084074

Will we be able to cure brugada syndrome? Veerakul G, Nademanee K. Heart Rhythm. 2016 Nov;13(11):2159-2160. doi: 10.1016/j.hrthm.2016.08.025. No abstract available. PMID:27544747

Characterization of the epicardial substrate for catheter ablation of Brugada syndrome. Zhang P, Tung R, Zhang Z, Sheng X, Liu Q, Jiang R, Sun Y, Chen S, Yu L, Ye Y, Fu G, Shivkumar K, Jiang C. Heart Rhythm. 2016 Nov;13(11):2151-2158. doi: 10.1016/j.hrthm.2016.07.025. PMID:27453126

Autonomic and cardio-respiratory responses to exercise in Brugada Syndrome patients. Chanavirut R, Makarawate P, Macdonald IA, Leelayuwat N. J Arrhythm. 2016 Oct;32(5):426-432. PMID:27761168 | PMCID:PMC5063266

Genetics of Brugada syndrome. Juang JJ, Horie M. J Arrhythm. 2016 Oct;32(5):418-425. Review. PMID:27761167 | PMCID:PMC5063259

Motor Vehicle Collision Secondary to Ventricular Dysrhythmia: A Case Report of Brugada Syndrome. Healey T, Buckley C 2nd, Mollman M. J Emerg Med. 2016 Oct 14. pii: S0736-4679(16)30796-X. doi: 10.1016/j.jemermed.2016.09.022. [Epub ahead of print] PMID:27751697

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 15 Quarterly Review of Literature 3rd & 4th Quarter 2016

Brugada syndrome in the young: an assessment of risk factors predicting future events. Gonzalez Corcia MC, Sieira J, Sarkozy A, de Asmundis C, Chierchia GB, Hernandez Ojeda J, Pappaert G, Brugada P. Europace. 2016 Oct 13. pii: euw206. [Epub ahead of print] PMID:27738063

Heart rate variability and repolarization characteristics in symptomatic and asymptomatic Brugada syndrome. Behar N, Petit B, Probst V, Sacher F, Kervio G, Mansourati J, Bru P, Hernandez A, Mabo P. Europace. 2016 Oct 13. pii: euw224. [Epub ahead of print] PMID:27738060

Numerous Brugada syndrome-associated genetic variants have no effect on J-point elevation, syncope susceptibility, malignant cardiac arrhythmia, and all-cause mortality. Ghouse J, Have CT, Skov MW, Andreasen L, Ahlberg G, Nielsen JB, Skaaby T, Olesen SP, Grarup N, Linneberg A, Pedersen O, Vestergaard H, Haunsø S¸ S, Svendsen JH, Hansen T, Kanters JK, Olesen MS. Genet Med. 2016 Oct 6. doi: 10.1038/gim.2016.151. [Epub ahead of print] PMID:27711072

(Tpeak - Tend)/QRS and (Tpeak - Tend)/(QT × QRS): novel markers for predicting arrhythmic risk in the Brugada syndrome. Tse G. Europace. 2016 Oct 5. pii: euw194. [Epub ahead of print] No abstract available. PMID:27707783

All that looks like "Brugada" is not "Brugada": Case series of Brugada phenocopy caused by hyponatremia. Agrawal Y, Aggarwal S, Kalavakunta JK, Gupta V. J Saudi Heart Assoc. 2016 Oct;28(4):274-7. doi: 10.1016/j.jsha.2016.02.003. PMID:27688678 | PMCID:PMC5034561

Efficacy and safety of dextrose-insulin in unmasking non-diagnostic Brugada ECG patterns. Velázquez-Rodríguez E, Rodríguez-Piña H, Pacheco-Bouthillier A, Jiménez-Cruz MP. J Electrocardiol. 2016 Aug 19. pii: S0022-0736(16)30166-2. doi: 10.1016/j.jelectrocard.2016.08.007. [Epub ahead of print] PMID:27686029

Large Genomic Imbalances in Brugada Syndrome. Mademont-Soler I, Pinsach-Abuin ML, Riuró H, Mates J, Pérez-Serra A, Coll M, Porres JM, Del Olmo B, Iglesias A, Selga E, Picó F, Pagans S, Ferrer-Costa C, Sarquella-Brugada G, Arbelo E, Cesar S, Brugada J, Campuzano Ó, Brugada R. PLoS One. 2016 Sep 29;11(9):e0163514. doi: 10.1371/journal.pone.0163514. PMID:27684715 | PMCID:PMC5042553

Embryonic type Na+ channel β-subunit, SCN3B masks the disease phenotype of Brugada syndrome. Okata S, Yuasa S, Suzuki T, Ito S, Makita N, Yoshida T, Li M, Kurokawa J, Seki T, Egashira T, Aizawa Y, Kodaira M, Motoda C, Yozu G, Shimojima M, Hayashiji N, Hashimoto H, Kuroda Y, Tanaka A, Murata M, Aiba T, Shimizu W, et al. Sci Rep. 2016 Sep 28;6:34198. doi: 10.1038/srep34198. PMID:27677334 | PMCID:PMC5039759

Combined Right Ventricular Outflow Tract Epicardial and Endocardial Late Potential Ablation for Treatment of Brugada Storm: A Case Report and Review of the Literature. Saha SA, Krishnan K, Madias C, Trohman RG. Cardiol Ther. 2016 Dec;5(2):229-243. PMID:27646458

Brugada Syndrome and Early Repolarisation: Distinct Clinical Entities or Different Phenotypes of the Same Genetic Disease? Conte G, Caputo ML, Regoli F, Moccetti T, Brugada P, Auricchio A. Arrhythm Electrophysiol Rev. 2016 Aug;5(2):84-9. doi: 10.15420/AER.2016.23.2. PMID:27617086 | PMCID:PMC5016597 SADS Quarterly Research Literature Review 2016 – Q3&4. Page 16 Quarterly Review of Literature 3rd & 4th Quarter 2016

Atypical electrocardiographic presentation of Brugada syndrome. Porto AG, Dametto E, Piazza R, Dragos A, Perfetti M, Bernardi G. Int J Cardiol. 2016 Nov 15;223:651-653. doi: 10.1016/j.ijcard.2016.08.125. No abstract available. PMID:27567233

A Novel SCN5A Mutation Associated with Drug Induced Brugada Type ECG. Turker I, Makiyama T, Vatta M, Itoh H, Ueyama T, Shimizu A, Ai T, Horie M. PLoS One. 2016 Aug 25;11(8):e0161872. doi: 10.1371/journal.pone.0161872. PMID:27560382 | PMCID:PMC4999187

Prevalence and Clinical Impact of Early Repolarization Pattern and QRS-Fragmentation in High-Risk Patients With Brugada Syndrome. Conte G, de Asmundis C, Sieira J, Ciconte G, Di Giovanni G, Chierchia GB, Casado-Arroyo R, Baltogiannis G, Ströker E, Irfan G, Pappaert G, Auricchio A, Brugada P. Circ J. 2016 Sep 23;80(10):2109-16. doi: 10.1253/circj.CJ-16-0370. PMID:27558008

Low-Dose Effectively Reduced Shocks in Brugada Syndrome Patients with an Implantable Cardioverter Defibrillator: A Chinese Case Series Report. Shen T, Yuan B, Geng J, Chen C, Zhou X, Shan Q. Ann Noninvasive Electrocardiol. 2016 Aug 23. doi: 10.1111/anec.12375. [Epub ahead of print] PMID:27550400

Long-Term Prognosis of Brugada-Type ECG and ECG With Atypical ST-Segment Elevation in the Right Precordial Leads Over 20 Years: Results From the Circulatory Risk in Communities Study (CIRCS). Tsuneoka H, Takagi M, Murakoshi N, Yamagishi K, Yokoyama Y, Xu D, Sekiguchi Y, Yamasaki H, Naruse Y, Ito Y, Igarashi M, Kitamura A, Okada T, Tanigawa T, Kuga K, Ohira T, Tada H, Aonuma K, Iso H; CIRCS Investigators.. J Am Heart Assoc. 2016 Aug 8;5(8). pii: e002899. doi: 10.1161/JAHA.115.002899. PMID:27503848 | PMCID:PMC5015268

Risk stratification in Brugada syndrome: Where is the finish line? Postema PG, Wilde AA. Heart Rhythm. 2016 Oct;13(10):1955-6. doi: 10.1016/j.hrthm.2016.08.008. No abstract available. PMID:27498077

Brugada Syndrome and the Subcutaneous Implantable Cardioverter-Defibrillator. Olde Nordkamp LR, Conte G, Rosenmöller BR, Warnaars JL, Tan HL, Caputo ML, Regoli F, Moccetti T, Auricchio A, Knops RE, Wilde AA. J Am Coll Cardiol. 2016 Aug 9;68(6):665-6. doi: 10.1016/j.jacc.2016.05.058. No abstract available. PMID:27491911

Brugada Syndrome: The Endless Conundrum. Mazzanti A, Priori SG. J Am Coll Cardiol. 2016 Aug 9;68(6):624-5. doi: 10.1016/j.jacc.2016.02.087. No abstract available. PMID:27491906

Long-Term Trends in Newly Diagnosed Brugada Syndrome: Implications for Risk Stratification. Casado-Arroyo R, Berne P, Rao JY, Rodriguez-Mañero M, Levinstein M, Conte G, Sieira J, Namdar M, Ricciardi D, Chierchia GB, de Asmundis C, Pappaert G, La Meir M, Wellens F, Brugada J, Brugada P. J Am Coll Cardiol. 2016 Aug 9;68(6):614-23. doi: 10.1016/j.jacc.2016.05.073. PMID:27491905 hiPSC-derived cardiomyocytes from Brugada Syndrome patients without identified mutations do not exhibit clear cellular electrophysiological abnormalities. Veerman CC, Mengarelli I, Guan K, Stauske M, Barc J, Tan HL, Wilde AA, Verkerk AO, Bezzina CR. Sci Rep. 2016 Aug 3;6:30967. doi: 10.1038/srep30967. PMID:27485484 | PMCID:PMC4971529

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 17 Quarterly Review of Literature 3rd & 4th Quarter 2016

The utility of T-wave alternans during the morning in the summer for the risk stratification of patients with Brugada syndrome. Sakamoto S, Takagi M, Kakihara J, Hayashi Y, Doi A, Sugioka K, Yoshiyama M. Heart Vessels. 2016 Aug 1. [Epub ahead of print] PMID:27480879

Risk factors for cardiac events in patients with Brugada syndrome: A PRISMA-compliant meta-analysis and systematic review. Wu W, Tian L, Ke J, Sun Y, Wu R, Zhu J, Ke Q. Medicine (Baltimore). 2016 Jul;95(30):e4214. doi: 10.1097/MD.0000000000004214. PMID:27472692

Risk stratification of in Brugada syndrome using noninvasive scoring methods. Kawazoe H, Nakano Y, Ochi H, Takagi M, Hayashi Y, Uchimura Y, Tokuyama T, Watanabe Y, Matsumura H, Tomomori S, Sairaku A, Suenari K, Awazu A, Miwa Y, Soejima K, Chayama K, Kihara Y. Heart Rhythm. 2016 Oct;13(10):1947-54. doi: 10.1016/j.hrthm.2016.07.009. PMID:27424075

Early and late manifestation of Brugada syndrome ECG in arrhythmogenic cardiomyopathy. Peters S. Int J Cardiol. 2016 Oct 1;220:260-1. doi: 10.1016/j.ijcard.2016.06.105. No abstract available. PMID:27389450

Type 1 Brugada pattern ECG due to supra-therapeutic phenytoin level. Swe T, Bhattarai B, Dufresne A. BMJ Case Rep. 2016 Jun 28;2016. pii: bcr2016214899. doi: 10.1136/bcr-2016-214899. PMID:27353175

Brugada syndrome is not an ECG. Martini B. Heart Rhythm. 2016 Sep;13(9):e292. doi: 10.1016/j.hrthm.2016.06.022. No abstract available. PMID:27321243

Reply to the Editor-Brugada syndrome is not an ECG. Andorin A, Probst V. Heart Rhythm. 2016 Sep;13(9):e292. doi: 10.1016/j.hrthm.2016.06.023. No abstract available. PMID:27321241

Activation Pattern of the Polymorphic Ventricular Tachycardia and Ventricular Fibrillation on Body Surface Mapping in Patients With Brugada Syndrome. Ueoka A, Morita H, Watanabe A, Nakagawa K, Nishii N, Nagase S, Ohe T, Ito H. Circ J. 2016 Jul 25;80(8):1734-43. doi: 10.1253/circj.CJ-16-0124. PMID:27319581

Dynamic ECG change in Brugada syndrome during coronary angiography. Kadoya Y, Kenzaka T. QJM. 2016 Jul;109(7):495. doi: 10.1093/qjmed/hcw069. No abstract available. PMID:27247430

Surveillance after cardiac arrest in patients with Brugada syndrome without an implantable defibrillator: An alarm effect of the previous syncope. Calvo D, Flórez JP, Valverde I, Rubín J, Pérez D, Vasserot MG, Rodríguez-Reguero J, Avanzas P, de la Hera JM, Gómez J, Coto E, Martínez-Camblor P, Morís C. Int J Cardiol. 2016 Sep 1;218:69-74. doi: 10.1016/j.ijcard.2016.05.018. PMID:27232914

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 18 Quarterly Review of Literature 3rd & 4th Quarter 2016

Brugada Syndrome Diagnosed from the ECG Leads in the High Intercostal Spaces: Searching for Answers from a Higher Source? Kumar S, Kalman JM. J Cardiovasc Electrophysiol. 2016 Aug;27(8):944-6. doi: 10.1111/jce.13016. No abstract available. PMID:27221147

Brugada syndrome in the paediatric population: a comprehensive approach to clinical manifestations, diagnosis, and management. Gonzalez Corcia MC, de Asmundis C, Chierchia GB, Brugada P. Cardiol Young. 2016 Aug;26(6):1044-55. doi: 10.1017/S1047951116000548. PMID:27151277

Clinical Presentation and Outcome of Brugada Syndrome Diagnosed With the New 2013 Criteria. Curcio A, Mazzanti A, Bloise R, Monteforte N, Indolfi C, Priori SG, Napolitano C. J Cardiovasc Electrophysiol. 2016 Aug;27(8):937-43. doi: 10.1111/jce.12997. PMID:27098113

Brugada Syndrome and PKP2: Evidences and uncertainties. Campuzano O, Fernández-Falgueras A, Iglesias A, Brugada R. Int J Cardiol. 2016 Jul 1;214:403-5. doi: 10.1016/j.ijcard.2016.03.194. PMID:27085656

Prognostic significance of fever-induced Brugada syndrome. Mizusawa Y, Morita H, Adler A, Havakuk O, Thollet A, Maury P, Wang DW, Hong K, Gandjbakhch E, Sacher F, Hu D, Amin AS, Lahrouchi N, Tan HL, Antzelevitch C, Probst V, Viskin S, Wilde AA. Heart Rhythm. 2016 Jul;13(7):1515-20. doi: 10.1016/j.hrthm.2016.03.044. PMID:27033637

Pharmacological Provocation of Outflow-Tract Tachycardia in a Patient With Brugada Syndrome. Bürkle G, Schäfer H, Marschall C, Brugada P, Ehrlich JR. Can J Cardiol. 2016 Dec;32(12):1577.e5-1577.e7. doi: 10.1016/j.cjca.2016.01.026. PMID:27032887

Paced QRS fragmentation is associated with spontaneous ventricular fibrillation in patients with Brugada syndrome. Kataoka N, Mizumaki K, Nakatani Y, Sakamoto T, Yamaguchi Y, Tsujino Y, Nishida K, Inoue H. Heart Rhythm. 2016 Jul;13(7):1497-503. doi: 10.1016/j.hrthm.2016.03.050. PMID:27018377

Warm-up Brugada phenocopy associated with takotsubo cardiomyopathy. Kirbas O, Ozeke O, Karabulut O, Unal S, Sen F, Cagli K, Golbasi Z, Topaloglu S, Aras D, Aydogdu S. Am J Emerg Med. 2016 Oct;34(10):2051.e1-2051.e3. doi: 10.1016/j.ajem.2016.02.075. No abstract available. PMID:26997494

Insight into specific pro-arrhythmic triggers in Brugada and early repolarization syndromes: results of long-term follow-up. Talib AK, Sato N, Myojo T, Sugiyama E, Nakagawa N, Sakamoto N, Tanabe Y, Fujino T, Takeuchi T, Akasaka K, Matsuhashi H, Saijo Y, Kawamura Y, Doi A, Hasebe N. Heart Vessels. 2016 Dec;31(12):2035-2044. PMID:26968993

Long-term efficacy of catheter ablation for paroxysmal in patients with Brugada syndrome and an implantable cardioverter-defibrillator to prevent inappropriate shock therapy. Kitamura T, Fukamizu S, Kawamura I, Hojo R, Aoyama Y, Komiyama K, Nishizaki M, Hiraoka M, Sakurada H. Heart Rhythm. 2016 Jul;13(7):1455-9. doi: 10.1016/j.hrthm.2016.03.006. PMID:26961302

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 19 Quarterly Review of Literature 3rd & 4th Quarter 2016

Ventricular arrhythmia during ajmaline challenge for the Brugada syndrome. Dobbels B, De Cleen D, Ector J. Europace. 2016 Oct;18(10):1501-1506. Review. PMID:26941343

Brugada Phenocopy Induced by Electrolyte Disorder: A Transient Electrocardiographic Sign. Hunuk A, Hunuk B, Kusken O, Onur OE. Ann Noninvasive Electrocardiol. 2016 Jul;21(4):429-32. doi: 10.1111/anec.12350. PMID:26910573

Brugada Phenocopy as a Dynamic Electrocardiographic Pattern during Acute Anterior . Ferrando-Castagnetto F, Garibaldi-Remuñan A, Vignolo G, Ricca-Mallada R, Baranchuk A. Ann Noninvasive Electrocardiol. 2016 Jul;21(4):425-8. doi: 10.1111/anec.12351. PMID:26901086

Intermittent Brugada Pattern in Relation to Changes in Body Temperature. Dias A, Franco E, Figueredo VM. Rev Esp Cardiol (Engl Ed). 2016 Jul;69(7):694. doi: 10.1016/j.rec.2015.11.023. English, Spanish. No abstract available. PMID:26850744

Brugada electrocardiogram pattern induced by cannabis; is cannabis safe? Alonso JV, Teo BH, Pozo FJ, Aguayo MA, Sanchez A. Am J Emerg Med. 2016 Aug;34(8):1738.e1-4. doi: 10.1016/j.ajem.2015.12.081. No abstract available. PMID:26818154

Diagnosis of Brugada's syndrome after subarachnoid injection of prilocaine. Oliván B, Arbeláez A, de Miguel M, Pelavski A. Rev Esp Anestesiol Reanim. 2016 Oct;63(8):483-6. doi: 10.1016/j.redar.2015.11.010. English, Spanish. PMID:26778671

Brugada syndrome: clinical presentation and genotype-correlation with magnetic resonance imaging parameters. Rudic B, Schimpf R, Veltmann C, Doesch C, Tülümen E, Schoenberg SO, Borggrefe M, Papavassiliu T. Europace. 2016 Sep;18(9):1411-9. doi: 10.1093/europace/euv300. PMID:26511399 Right coronary cusp as a new window of ablation for pilsicainide-induced ventricular premature contractions in a patient with Brugada syndrome. Yamasaki H, Tada H, Sekiguchi Y, Aonuma K. Heart Vessels. 2016 Aug;31(8):1397-401. doi: 10.1007/s00380-015-0760-3. PMID:26510553

Expert cardiologists cannot distinguish between Brugada phenocopy and Brugada syndrome electrocardiogram patterns. Gottschalk BH, Anselm DD, Brugada J, Brugada P, Wilde AA, Chiale PA, Pérez-Riera AR, Elizari MV, De Luna AB, Krahn AD, Tan HL, Postema PG, Baranchuk A. Europace. 2016 Jul;18(7):1095-100. doi: 10.1093/europace/euv278. PMID:26498159

Not all ST-segment elevations are myocardial infarction: Hyperkalemia and Brugada phenocopy. Dendramis G, Petrina SM, Baranchuk A. Am J Emerg Med. 2016 Oct 29. pii: S0735-6757(16)30779-3. doi: 10.1016/j.ajem.2016.10.063. [Epub ahead of print] PMID:27839842

Sudden cardiac arrest due to a single sodium channel mutation producing a mixed phenotype of Brugada and Long QT3 syndromes. Lakshmanadoss U, Mertens A, Gallagher M, Kutinsky I, Williamson B. Indian Pacing Electrophysiol J. 2016 Mar-Apr;16(2):66-9. doi: 10.1016/j.ipej.2016.07.001. PMID:27676163 | PMCID:PMC5031864

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 20 Quarterly Review of Literature 3rd & 4th Quarter 2016

Mutation in S6 domain of HCN4 channel in patient with suspected Brugada syndrome modifies channel function. Biel S, Aquila M, Hertel B, Berthold A, Neumann T, DiFrancesco D, Moroni A, Thiel G, Kauferstein S. Pflugers Arch. 2016 Oct;468(10):1663-71. doi: 10.1007/s00424-016-1870-1. PMID:27553229 Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT)

Patient Specific Induced Pluripotent Stem Cell-Derived Cardiomyocytes for Drug Development and Screening In Catecholaminergic Polymorphic Ventricular Tachycardia. Jehuda Ronen B, Lili B. J Atr Fibrillation. 2016 Aug 31;9(2):1423. Review. PMID:27909533 | PMCID:PMC5129686

Management of Gene-Positive Catecholaminergic Polymorphic Ventricular Tachycardia: Are the Long Term Outcomes on Therapy Really So Poor? - Reply. Kawata H, Shimizu W. Circ J. 2016 Nov 25;80(12):2566. No abstract available. PMID:27840380

Management of Gene-Positive Catecholaminergic Polymorphic Ventricular Tachycardia: Are the Long Term Outcomes on Therapy Really So Poor? Roston TM, Sanatani S. Circ J. 2016 Nov 25;80(12):2565. No abstract available. PMID:27840378

Treatment of catecholaminergic polymorphic ventricular tachycardia in mice using novel RyR2-modifying drugs. Li N, Wang Q, Sibrian-Vazquez M, Klipp RC, Reynolds JO, Word TA, Scott L Jr, Salama G, Strongin RM, Abramson JJ, Wehrens XH. Int J Cardiol. 2016 Oct 29. pii: S0167-5273(16)33286-7. doi: 10.1016/j.ijcard.2016.10.078. [Epub ahead of print] PMID:27838126

Questioning flecainide's mechanism of action in the treatment of catecholaminergic polymorphic ventricular tachycardia. Williams AJ, Bannister ML, Thomas NL, Sikkel MB, Mukherjee S, Maxwell C, MacLeod KT, George CH. J Physiol. 2016 Nov 1;594(21):6431-6432. doi: 10.1113/JP272497. No abstract available. PMID:27800620 | PMCID:PMC5088245

Patient-Specific Human Induced Pluripotent Stem Cell Model Assessed with Electrical Pacing Validates S107 as a Potential Therapeutic Agent for Catecholaminergic Polymorphic Ventricular Tachycardia. Sasaki K, Makiyama T, Yoshida Y, Wuriyanghai Y, Kamakura T, Nishiuchi S, Hayano M, Harita T, Yamamoto Y, Kohjitani H, Hirose S, Chen J, Kawamura M, Ohno S, Itoh H, Takeuchi A, Matsuoka S, Miura M, Sumitomo N, Horie M, Yamanaka S, Kimura T. PLoS One. 2016 Oct 20;11(10):e0164795. doi: 10.1371/journal.pone.0164795. PMID:27764147 | PMCID:PMC5072719

Current topics in catecholaminergic polymorphic ventricular tachycardia. Sumitomo N. J Arrhythm. 2016 Oct;32(5):344-351. Review. PMID:27761157 | PMCID:PMC5063269

Catecholaminergic Polymorphic Ventricular Tachycardia. Napolitano C, Priori SG, Bloise R. 2004 Oct 14 [updated 2016 Oct 13]. In: Pagon RA, Adam MP, Ardinger HH, Wallace SE, Amemiya A, Bean LJH, Bird TD, Ledbetter N, Mefford HC, Smith RJH, Stephens K, editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2016. PMID:20301466

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 21 Quarterly Review of Literature 3rd & 4th Quarter 2016

Adeno-associated virus-mediated CASQ2 delivery rescues phenotypic alterations in a patient-specific model of recessive catecholaminergic polymorphic ventricular tachycardia. Lodola F, Morone D, Denegri M, Bongianino R, Nakahama H, Rutigliano L, Gosetti R, Rizzo G, Vollero A, Buonocore M, Napolitano C, Condorelli G, Priori SG, Di Pasquale E. Cell Death Dis. 2016 Oct 6;7(10):e2393. doi: 10.1038/cddis.2016.304. PMID:27711080

Catecholaminergic Polymorphic Ventricular Tachycardia as the Etiology of Emergency Medical Services-Reported Traumatic Arrest. Bailey J, Berkeley RP. Pediatr Emerg Care. 2016 Sep 23. [Epub ahead of print] PMID:27668923

A novel RYR2 loss-of-function mutation (I4855M) is associated with left ventricular non-compaction and atypical catecholaminergic polymorphic ventricular tachycardia. Roston TM, Guo W, Krahn AD, Wang R, Van Petegem F, Sanatani S, Chen SR, Lehman A. J Electrocardiol. 2016 Sep 8. pii: S0022-0736(16)30179-0. doi: 10.1016/j.jelectrocard.2016.09.006. [Epub ahead of print] PMID:27646203

Catecholaminergic polymorphic ventricular tachycardia associated with and : Case report and literature review. Brunetti ND, Pellegrino PL, D'Arienzo G, Sai R, Ziccardi L, Santoro F, Gaglione A, Di Biase M. J Electrocardiol. 2016 Jul 28. pii: S0022-0736(16)30102-9. doi: 10.1016/j.jelectrocard.2016.07.024. [Epub ahead of print] PMID:27539163

Integration of 60,000 exomes and ACMG guidelines question the role of Catecholaminergic Polymorphic Ventricular Tachycardia-associated variants. Paludan-Müller C, Ahlberg G, Ghouse J, Herfelt C, Svendsen JH, Haunsø S, Kanters JK, Olesen MS. Clin Genet. 2016 Aug 19. doi: 10.1111/cge.12847. [Epub ahead of print] PMID:27538377

Long-Term Prognosis of Catecholaminergic Polymorphic Ventricular Tachycardia Patients With Ryanodine Receptor (RYR2) Mutations. Yamazoe M, Furukawa T. Circ J. 2016 Aug 25;80(9):1892-4. doi: 10.1253/circj.CJ-16-0745. No abstract available. PMID:27523319

Novel CPVT-Associated Calmodulin Mutation in CALM3 (CALM3-A103V) Activates Arrhythmogenic Ca Waves and Sparks. Gomez-Hurtado N, Boczek NJ, Kryshtal DO, Johnson CN, Sun J, Nitu FR, Cornea RL, Chazin WJ, Calvert ML, Tester DJ, Ackerman MJ, Knollmann BC. Circ Arrhythm Electrophysiol. 2016 Aug;9(8). pii: e004161. doi: 10.1161/CIRCEP.116.004161. PMID:27516456 | PMCID:PMC4988333

A human pluripotent stem cell model of catecholaminergic polymorphic ventricular tachycardia recapitulates patient- specific drug responses. Preininger MK, Jha R, Maxwell JT, Wu Q, Singh M, Wang B, Dalal A, Mceachin ZT, Rossoll W, Hales CM, Fischbach PS, Wagner MB, Xu C. Dis Model Mech. 2016 Sep 1;9(9):927-39. doi: 10.1242/dmm.026823. PMID:27491078 | PMCID:PMC5047684

Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) Associated With Ryanodine Receptor (RyR2) Gene Mutations - Long-Term Prognosis After Initiation of Medical Treatment. Kawata H, Ohno S, Aiba T, Sakaguchi H, Miyazaki A, Sumitomo N, Kamakura T, Nakajima I, Inoue YY, Miyamoto K, Okamura H, Noda T, Kusano K, Kamakura S, Miyamoto Y, Shiraishi I, Horie M, Shimizu W. Circ J. 2016 Aug 25;80(9):1907-15. doi: 10.1253/circj.CJ-16-0250. PMID:27452199

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 22 Quarterly Review of Literature 3rd & 4th Quarter 2016

Clinical and genetic profile of catecholaminergic polymorphic ventricular tachycardia in Hong Kong Chinese children. Yu TC, Liu AP, Lun KS, Chung BH, Yung TC. Hong Kong Med J. 2016 Aug;22(4):314-9. doi: 10.12809/hkmj154653. PMID:27256466

Exercise training prevents ventricular tachycardia in CPVT1 due to reduced CaMKII-dependent arrhythmogenic Ca2+ release. Manotheepan R, Danielsen TK, Sadredini M, Anderson ME, Carlson CR, Lehnart SE, Sjaastad I, Stokke MK. Cardiovasc Res. 2016 Aug 1;111(3):295-306. doi: 10.1093/cvr/cvw095. PMID:27161030 | PMCID:PMC4957490

A novel heterozygous mutation in cardiac calsequestrin causes autosomal dominant catecholaminergic polymorphic ventricular tachycardia. Gray B, Bagnall RD, Lam L, Ingles J, Turner C, Haan E, Davis A, Yang PC, Clancy CE, Sy RW, Semsarian C. Heart Rhythm. 2016 Aug;13(8):1652-60. doi: 10.1016/j.hrthm.2016.05.004. PMID:27157848

Clinical Challenges in Catecholaminergic Polymorphic Ventricular Tachycardia. Imberti JF, Underwood K, Mazzanti A, Priori SG. Heart Lung Circ. 2016 Aug;25(8):777-83. doi: 10.1016/j.hlc.2016.01.012. Review. PMID:26948768

Early repolarization pattern: a marker of increased risk in patients with catecholaminergic polymorphic ventricular tachycardia. Tülümen E, Schulze-Bahr E, Zumhagen S, Stallmeyer B, Seebohm G, Beckmann BM, Kääb S, Rudic B, Liebe V, Wolpert C, Herrera-Siklody C, Veltmann C, Schimpf R, Borggrefe M. Europace. 2016 Oct;18(10):1587-1592. PMID:26705564

Slowed depolarization and irregular repolarization in catecholaminergic polymorphic ventricular tachycardia: a study from cellular Ca2+ transients and action potentials to clinical monophasic action potentials and . Paavola J, Väänänen H, Larsson K, Penttinen K, Toivonen L, Kontula K, Laine M, Aalto-Setälä K, Swan H, Viitasalo M. Europace. 2016 Oct;18(10):1599-1607. PMID:26705554 Channelopathies/ Arrhythmia

Catheter ablation for ventricular tachyarrhythmia in patients with channelopathies. Murakoshi N, Aonuma K. J Arrhythm. 2016 Oct;32(5):404-410. Review. PMID:27761165 | PMCID:PMC5063265

Atrial arrhythmias in inherited arrhythmogenic disorders. Hasdemir C. J Arrhythm. 2016 Oct;32(5):366-372. Review. PMID:27761160 | PMCID:PMC5063273

Left cardiac sympathetic denervation: An important treatment option for patients with hereditary ventricular arrhythmias. Cho Y. J Arrhythm. 2016 Oct;32(5):340-343. Review. PMID:27761156 | PMCID:PMC5063267

Ion channelopathies in functional GI disorders. Beyder A, Farrugia G. Am J Physiol Gastrointest Liver Physiol. 2016 Oct 1;311(4):G581-G586. doi: 10.1152/ajpgi.00237.2016. Review. PMID:27514480

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 23 Quarterly Review of Literature 3rd & 4th Quarter 2016

Challenges in Molecular Diagnostics of Channelopathies in the Next-Generation Sequencing Era: Less Is More? Novelli V, Gambelli P, Memmi M, Napolitano C. Front Cardiovasc Med. 2016 Sep 12;3:29. doi: 10.3389/fcvm.2016.00029. No abstract available. PMID:27672637 | PMCID:PMC5018689

Channels and channelopathies. Persson PB, Bondke Persson A. Acta Physiol (Oxf). 2016 Nov;218(3):149-151. doi: 10.1111/apha.12796. No abstract available. PMID:27589477

Atrial fibrillation in inherited cardiac channelopathies: From mechanisms to management. Enriquez A, Antzelevitch C, Bismah V, Baranchuk A. Heart Rhythm. 2016 Sep;13(9):1878-84. doi: 10.1016/j.hrthm.2016.06.008. PMID:27291509

Early Repolarisation Changes in ECG: Are they Benign or Malignant? Satish OS, Srivastav KS. J Assoc Physicians India. 2016 Jul;64(7):51-54. PMID:27759343

Nonsustained Ventricular Tachycardia in the Normal Heart: Risk Stratification and Management. Marine JE. Card Electrophysiol Clin. 2016 Sep;8(3):525-43. doi: 10.1016/j.ccep.2016.04.003. Review. PMID:27521087

Differences in the onset mode of ventricular tachyarrhythmia between patients with J wave in anterior leads versus inferolateral leads. Kamakura T, Wada M, Ishibashi K, Inoue YY, Miyamoto K, Okamura H, Nagase S, Noda T, Aiba T, Yasuda S, Shimizu W, Kamakura S, Kusano K. Heart Rhythm. 2016 Nov 23. pii: S1547-5271(16)31074-8. doi: 10.1016/j.hrthm.2016.11.027. [Epub ahead of print] PMID:27890733

Exaggerated Reactivity of Parasympathetic Nerves is Involved in Ventricular Fibrillation in J-Wave Syndrome. Shinohara T, Kondo H, Otsubo T, Fukui A, Yufu K, Nakagawa M, Takahashi N. J Cardiovasc Electrophysiol. 2016 Nov 25. doi: 10.1111/jce.13135. [Epub ahead of print] PMID:27885745

In Silico Evaluation of the Potential Antiarrhythmic Effect of Epigallocatechin-3-Gallate on Cardiac Channelopathies. Boukhabza M, El Hilaly J, Attiya N, El-Haidani A, Filali-Zegzouti Y, Mazouzi D, Amarouch MY. Comput Math Methods Med. 2016;2016:7861653. PMID:27882075 | PMCID:PMC5110949

Suppression-of-function mutations in the cardiac ryanodine receptor: Emerging evidence for a novel arrhythmia syndrome? Roston TM, Sanatani S, Chen SR. Heart Rhythm. 2016 Nov 3. pii: S1547-5271(16)31005-0. doi: 10.1016/j.hrthm.2016.11.004. [Epub ahead of print] No abstract available. PMID:27818320

Assessing arrhythmia risk in a 15-year-old boy whose father died suddenly. Dorian P, Krahn AD. CMAJ. 2016 Nov 1;188(16):1172-1174. No abstract available. PMID:27431299 | PMCID:PMC5088078

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 24 Quarterly Review of Literature 3rd & 4th Quarter 2016

Phenotypic Variability of ANK2 Mutations in Patients With Inherited Primary Arrhythmia Syndromes. Ichikawa M, Aiba T, Ohno S, Shigemizu D, Ozawa J, Sonoda K, Fukuyama M, Itoh H, Miyamoto Y, Tsunoda T, Makiyama T, Tanaka T, Shimizu W, Horie M. Circ J. 2016 Nov 25;80(12):2435-2442. PMID:27784853

J-Wave syndromes expert consensus conference report: Emerging concepts and gaps in knowledge. Antzelevitch C, Yan GX, Ackerman MJ, Borggrefe M, Corrado D, Guo J, Gussak I, Hasdemir C, Horie M, Huikuri H, Ma C, Morita H, Nam GB, Sacher F, Shimizu W, Viskin S, Wilde AA. J Arrhythm. 2016 Oct;32(5):315-339. No abstract available. PMID:27761155 | PMCID:PMC5063270

Medium-term outcomes of idiopathic ventricular fibrillation survivors and family screening: a multicentre experience. Honarbakhsh S, Srinivasan N, Kirkby C, Firman E, Tobin L, Finlay M, Hunter RJ, Murphy C, Lowe MD, Schilling RJ, Lambiase PD. Europace. 2016 Oct 12. pii: euw251. [Epub ahead of print] PMID:27738067

Enhanced Cytosolic Ca2+ Activation Underlies a Common Defect of Central Domain Cardiac Ryanodine Receptor Mutations Linked to Arrhythmias. Xiao Z, Guo W, Sun B, Hunt DJ, Wei J, Liu Y, Wang Y, Wang R, Jones PP, Back TG, Chen SR. J Biol Chem. 2016 Nov 18;291(47):24528-24537. PMID:27733687 | PMCID:PMC5114406

Long-Term Outcome of Patients Initially Diagnosed With Idiopathic Ventricular Fibrillation: A Descriptive Study. Visser M, van der Heijden JF, van der Smagt JJ, Doevendans PA, Wilde AA, Loh P, Hassink RJ. Circ Arrhythm Electrophysiol. 2016 Oct;9(10). pii: e004258. PMID:27733492

J Wave Syndromes: History and Current Controversies. Liu T, Zheng J, Yan GX. Korean Circ J. 2016 Sep;46(5):601-609. Review. PMID:27721848 | PMCID:PMC5054169

Risk of Ventricular Arrhythmias and Association with Ondansetron. Moeller JR, Gummin DD, Nelson TJ, Drendel AL, Shah BK, Berger S. J Pediatr. 2016 Dec;179:118-123.e1. doi: 10.1016/j.jpeds.2016.08.058. PMID:27665040

Electrocardiographic early repolarization characteristics and clinical presentations in the young: a benign finding or worrisome marker for arrhythmias. Safa R, Thomas R, Karpawich PP. Congenit Heart Dis. 2016 Sep 21. doi: 10.1111/chd.12410. [Epub ahead of print] PMID:27650315

12-lead electrocardiogram features of arrhythmic risk: A focus on early repolarization. Rizzo C, Monitillo F, Iacoviello M. World J Cardiol. 2016 Aug 26;8(8):447-55. doi: 10.4330/wjc.v8.i8.447. Review. PMID:27621772 | PMCID:PMC4997525

Reduced Sodium Current in the Lateral Ventricular Wall Induces Inferolateral J-Waves. Meijborg VM, Potse M, Conrath CE, Belterman CN, De Bakker JM, Coronel R. Front Physiol. 2016 Aug 26;7:365. doi: 10.3389/fphys.2016.00365. PMID:27616994 | PMCID:PMC5000556

Role of Adjuvant Renal Sympathetic Denervation in the Treatment of Ventricular Arrhythmias. Evranos B, Canpolat U, Kocyigit D, Coteli C, Yorgun H, Aytemir K. Am J Cardiol. 2016 Oct 15;118(8):1207-1210. doi: 10.1016/j.amjcard.2016.07.036. PMID:27600464

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 25 Quarterly Review of Literature 3rd & 4th Quarter 2016

The Role of the Ratio of J-Point Elevation Magnitude and R-Wave Amplitude on the Same ECG Lead in the Risk Stratification of Subjects With Early Repolarization Pattern. Chen XM, Ji CC, Cheng YJ, Liu LJ, Zhu WQ, Huang Y, Chen WY, Wu SH. Clin Cardiol. 2016 Sep 6. doi: 10.1002/clc.22587. [Epub ahead of print] PMID:27599368

NALCN channelopathies: Distinguishing gain-of-function and loss-of-function mutations. Bend EG, Si Y, Stevenson DA, Bayrak-Toydemir P, Newcomb TM, Jorgensen EM, Swoboda KJ. Neurology. 2016 Sep 13;87(11):1131-9. doi: 10.1212/WNL.0000000000003095. PMID:27558372 | PMCID:PMC5027803

Exercise-induced Ventricular Tachycardia/Ventricular Fibrillation in the Normal Heart: Risk Stratification and Management. Michowitz Y, Viskin S, Rosso R. Card Electrophysiol Clin. 2016 Sep;8(3):593-600. doi: 10.1016/j.ccep.2016.04.008. Review. PMID:27521092

Convulsive Syncope Induced by Ventricular Arrhythmia Masquerading as Epileptic Seizures: Case Report and Literature Review. Sabu J, Regeti K, Mallappallil M, Kassotis J, Islam H, Zafar S, Khan R, Ibrahim H, Kanta R, Sen S, Yousif A, Nai Q. J Clin Med Res. 2016 Aug;8(8):610-5. doi: 10.14740/jocmr2583w. PMID:27429683 | PMCID:PMC4931808

J-Wave syndromes expert consensus conference report: Emerging concepts and gaps in knowledge. Antzelevitch C, Yan GX, Ackerman MJ, Borggrefe M, Corrado D, Guo J, Gussak I, Hasdemir C, Horie M, Huikuri H, Ma C, Morita H, Nam GB, Sacher F, Shimizu W, Viskin S, Wilde AA. Heart Rhythm. 2016 Oct;13(10):e295-324. doi: 10.1016/j.hrthm.2016.05.024. Review. No abstract available. PMID:27423412 | PMCID:PMC5035208

Neuro-cardiac interaction in malignant ventricular arrhythmia and sudden cardiac death. Ng GA. Auton Neurosci. 2016 Aug;199:66-79. doi: 10.1016/j.autneu.2016.07.001. Review. PMID:27423297

J-Wave syndromes expert consensus conference report: Emerging concepts and gaps in knowledge: Endorsed by the Asia Pacific Heart Rhythm Society (APHRS), the European Heart Rhythm Association (EHRA), the Heart Rhythm Society (HRS), and the Latin American S Antzelevitch C, Yan GX, Ackerman MJ, Borggrefe M, Corrado D, Guo J, Gussak I, Hasdemir C, Horie M, Huikuri H, Ma C, Morita H, Nam GB, Sacher F, Shimizu W, Viskin S, Wilde AA. Europace. 2016 Jul 13. pii: euw235. [Epub ahead of print] No abstract available. PMID:27411360

Detection and successful treatment of emergent anti-SSA mediated fetal atrioventricular block. Cuneo BF, Ambrose SE, Tworetzky W. Am J Obstet Gynecol. 2016 Jul 9. pii: S0002-9378(16)30439-2. doi: 10.1016/j.ajog.2016.07.006. [Epub ahead of print] No abstract available. PMID:27403848

Further Insights in the Most Common SCN5A Mutation Causing Overlapping Phenotype of Long QT Syndrome, Brugada Syndrome, and Conduction Defect. Veltmann C, Barajas-Martinez H, Wolpert C, Borggrefe M, Schimpf R, Pfeiffer R, Cáceres G, Burashnikov E, Antzelevitch C, Hu D. J Am Heart Assoc. 2016 Jul 5;5(7). pii: e003379. doi: 10.1161/JAHA.116.003379. PMID:27381756 | PMCID:PMC5015375

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 26 Quarterly Review of Literature 3rd & 4th Quarter 2016

Cardiac sodium channel palmitoylation regulates channel availability and myocyte excitability with implications for arrhythmia generation. Pei Z, Xiao Y, Meng J, Hudmon A, Cummins TR. Nat Commun. 2016 Jun 23;7:12035. doi: 10.1038/ncomms12035. PMID:27337590 | PMCID:PMC4931030

Electrophysiological characterization of a large set of novel variants in the SCN5A-gene: identification of novel LQTS3 and BrS mutations. Ortiz-Bonnin B, Rinné S, Moss R, Streit AK, Scharf M, Richter K, Stöber A, Pfeufer A, Seemann G, Kääb S, Beckmann BM, Decher N. Pflugers Arch. 2016 Aug;468(8):1375-87. doi: 10.1007/s00424-016-1844-3. PMID:27287068

Challenging the unifying pathophysiological concept of J-wave syndromes. Wilde AA. Heart Rhythm. 2016 Aug;13(8):1729-30. doi: 10.1016/j.hrthm.2016.04.017. No abstract available. PMID:27132151

A patient with early repolarization syndrome and concurrent Brugada syndrome: Demonstration of a different pathophysiology? Visser M, van der Smagt JJ, Loh P, van der Heijden JF, Hassink RJ. Int J Cardiol. 2016 Nov 15;223:58-60. doi: 10.1016/j.ijcard.2016.08.072. No abstract available. PMID:27529593 Implantable Cardioverter Defibrillator (ICD)

Implementation of a Screening Program for Patients at Risk for Posttraumatic Stress Disorder. Roberts CR, Wofford JE, Hoy HM, Faddis MN. Clin Med Insights Cardiol. 2016 Aug 8;10:129-37. doi: 10.4137/CMC.S39957. PMID:27547041 | PMCID:PMC4978196

Performance of the subcutaneous implantable defibrillator in primary prevention patients with and without reduced ejection fraction versus secondary prevention patients. Boersma L, Barr CS, Burke MC, Leon AR, Theuns DA, Herre JM, Weiss R, Kremers MS, Neuzil P, Husby MP, Carter N, Stivland TM, Gold MR; EFFORTLESS and IDE Study Investigators.. Heart Rhythm. 2016 Nov 24. pii: S1547-5271(16)31070-0. doi: 10.1016/j.hrthm.2016.11.025. [Epub ahead of print] PMID:27890798

Posttraumatic stress and quality of life with the totally subcutaneous compared to conventional cardioverter-defibrillator systems. Köbe J, Hucklenbroich K, Geisendörfer N, Bettin M, Frommeyer G, Reinke F, Dechering D, Burgmer M, Eckardt L. Clin Res Cardiol. 2016 Nov 22. [Epub ahead of print] PMID:27878381

A propensity matched case-control study comparing efficacy, safety and costs of the subcutaneous vs. transvenous implantable cardioverter defibrillator. Honarbakhsh S, Providencia R, Srinivasan N, Ahsan S, Lowe M, Rowland E, Hunter RJ, Finlay M, Segal O, Earley MJ, Chow A, Schilling RJ, Lambiase PD. Int J Cardiol. 2016 Nov 7;228:280-285. doi: 10.1016/j.ijcard.2016.11.017. [Epub ahead of print] PMID:27865198

Should the Subcutaneous Implantable Defibrillator Be the First Choice for Primary Prevention of Sudden Cardiac Death? Knops RE, Brouwer TF. Rev Esp Cardiol (Engl Ed). 2016 Nov 9. pii: S1885-5857(16)30324-3. doi: 10.1016/j.rec.2016.09.030. [Epub ahead of print] English, Spanish. No abstract available. PMID:27838348

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 27 Quarterly Review of Literature 3rd & 4th Quarter 2016

Spanish Implantable Cardioverter-defibrillator Registry. Twelfth Official Report of the Spanish Society of Electrophysiology and Arrhythmias Section (2015). Alzueta J, Fernández-Lozano I, Barrera A. Rev Esp Cardiol (Engl Ed). 2016 Dec;69(12):1168-1179. doi: 10.1016/j.rec.2016.08.013. English, Spanish. PMID:27818148

Long-Term Clinical Outcomes of Subcutaneous Versus Transvenous Implantable Defibrillator Therapy. Brouwer TF, Yilmaz D, Lindeboom R, Buiten MS, Olde Nordkamp LR, Schalij MJ, Wilde AA, van Erven L, Knops RE. J Am Coll Cardiol. 2016 Nov 8;68(19):2047-2055. doi: 10.1016/j.jacc.2016.08.044. PMID:27810043

Effect of Significant Weight Change on Inappropriate Implantable Cardioverter-Defibrillator Therapy. Daimee U, Biton Y, Aktas MK, Zannad F, Klein H, Szepietowska B, McNitt S, Polonsky B, Wang PJ, Zareba W, Moss AJ, Kutyifa V. Pacing Clin Electrophysiol. 2016 Nov 3. doi: 10.1111/pace.12973. [Epub ahead of print] PMID:27808410

Incidence of arrhythmias in a large cohort of patients with current implantable cardioverter-defibrillators in Spain: results from the UMBRELLA Registry. Fontenla A, Martínez-Ferrer JB, Alzueta J, Viñolas X, García-Alberola A, Brugada J, Peinado R, Sancho-Tello MJ, Cano A, Fernández-Lozano I. Europace. 2016 Nov;18(11):1726-1734. PMID:26705555

Concomitant Use of the Subcutaneous Implantable Cardioverter Defibrillator and a Permanent Pacemaker. Huang J, Patton KK, Prutkin JM. Pacing Clin Electrophysiol. 2016 Nov;39(11):1240-1245. doi: 10.1111/pace.12955. PMID:27734502

Implantable cardioverter defibrillator management: an update. Malagù M, Ferri A, Mancuso O, Trevisan F, Nardozza M, Bertini M. Future Cardiol. 2016 Nov;12(6):673-688. PMID:27762625 Short ECG segments predict defibrillation outcome using quantitative waveform measures. Coult J, Sherman L, Kwok H, Blackwood J, Kudenchuk PJ, Rea TD. Resuscitation. 2016 Dec;109:16-20. doi: 10.1016/j.resuscitation.2016.09.020. PMID:27702580

Midterm Results of Implantable Cardioverter Defibrillators in Children and Young Adults from a Single Center in Turkey. Aykan HH, Karagoz T, Gulgun M, Ertugrul I, Aypar E, Ozer S, Alehan D, Celiker A, Ozkutlu S. Pacing Clin Electrophysiol. 2016 Nov;39(11):1225-1239. doi: 10.1111/pace.12954. PMID:27620455

A qualitative study of decision-making about the implantation of cardioverter defibrillators and deactivation during end-of- life care. Standing H, Exley C, Flynn D, Hughes J, Joyce K, Lobban T, Lord S, Matlock D, McComb JM, Paes P, Thomson RG. Southampton (UK): NIHR Journals Library; 2016 Oct. PMID:27786432

Sex Differences in Inappropriate ICD Device Therapies: MADIT-II and MADIT-CRT. Tompkins CM, McNitt S, Polonsky B, Daubert JP, Wang PJ, Moss AJ, Zareba W, Kutyifa V. J Cardiovasc Electrophysiol. 2016 Oct 2. doi: 10.1111/jce.13102. [Epub ahead of print] PMID:27696593

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 28 Quarterly Review of Literature 3rd & 4th Quarter 2016

Comparative Evaluation of Four Risk Scores for Predicting Mortality in Patients With Implantable Cardioverter-defibrillator for Primary Prevention. Rodríguez-Mañero M, Abu Assi E, Sánchez-Gómez JM, Fernández-Armenta J, Díaz-Infante E, García-Bolao I, Benezet- Mazuecos J, Andrés Lahuerta A, Expósito-García V, Bertomeu-González V, Arce-León Á, Barrio-López MT, Peinado R, Martínez-Sande L, Arias MA. Rev Esp Cardiol (Engl Ed). 2016 Nov;69(11):1033-1041. doi: 10.1016/j.rec.2016.03.027. English, Spanish. PMID:27491594

Long-time "real-life" performance of the subcutaneous ICD in patients with electrical heart disease or idiopathic ventricular fibrillation. Frommeyer G, Dechering DG, Kochhäuser S, Bettin M, Köbe J, Eckardt L, Reinke F. J Interv Card Electrophysiol. 2016 Nov;47(2):185-188. PMID:27217031

Totally submuscular implantation of subcutaneous implantable cardioverter defibrillator: a safe and effective solution for obese or oversized patients. Droghetti A, Locatelli A, Casiraghi B, Malacrida M, Arupi M, Ragusa M. Clin Case Rep. 2016 Sep 17;4(10):1009-1011. PMID:27761257 | PMCID:PMC5054481

Erratum to '2015 HRS/EHRA/APHRS/SOLAECE expert consensus statement on optimal implantable cardioverter- defibrillator programming and testing' [Journal of Arrhythmia 32/1 (2016) 1-28]. Wilkoff BL, Fauchier L, Stiles MK, Morillo CA, Al-Khatib SM, Almendral J, Aguinaga L, Berger RD, Cuesta A, Daubert JP, Dubner S, Ellenbogen KA, Mark Estes NA 3rd, Fenelon G, Garcia FC, Gasparini M, Haines DE, Healey JS, Hurtwitz JL, Keegan R, Kolb C, Kuck KH, et al. J Arrhythm. 2016 Oct;32(5):441-442. PMID:27761170 | PMCID:PMC5063272

Canadian Registry of Implantable Electronic Device Outcomes: Surveillance of the Riata Lead Under Advisory. Parkash R, Thibault B, Mangat I, Coutu B, Bennett M, Healey J, Verma A, Philippon F, Sandhu R, Cameron D, Ayala- Paredes F, Sterns L, Essebag V, Kus T, Nery P, Stephenson E, Yee R, Exner D, Toal S, Birnie D, Wells G, Krahn A. Circ Arrhythm Electrophysiol. 2016 Oct;9(10). pii: e004282. PMID:27733493

Combined Subpectoral Implantation of Implantable Cardioverter-Defibrillator and Augmentation Mammoplasty in a Young Female Patient. Kim DJ, Uhm JS, Park JW, Youn JC, Lee DW, Koo BN, Lee MH. Korean Circ J. 2016 Sep;46(5):734-738. PMID:27721868 | PMCID:PMC5054189

Effect of psycho-educational interventions on quality of life in patients with implantable cardioverter defibrillators: a meta- analysis of randomized controlled trials. Kao CW, Chen MY, Chen TY, Lin PH. Health Qual Life Outcomes. 2016 Sep 30;14(1):138. PMID:27716219 | PMCID:PMC5045654

Outcomes of lead extraction without subsequent device reimplantation. Al-Hijji MA, Killu AM, Yousefian O, Hodge DO, Park JY, Hebsur S, El Sabbagh A, Pretorius VG, Ackerman MJ, Friedman PA, Birgersdotter-Green U, Cha YM. Europace. 2016 Oct 5. pii: euw184. [Epub ahead of print] PMID:27707785

Subcutaneous implantable cardioverter-defibrillator (S-ICD) for secondary prevention of sudden cardiac death. Kempa M, Budrejko S, Raczak G. Arch Med Sci. 2016 Oct 1;12(5):1179-1180. No abstract available. PMID:27695509 | PMCID:PMC5016596

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 29 Quarterly Review of Literature 3rd & 4th Quarter 2016

A Novel Method for Epicardial Defibrillator Lead Placement in Young Children: Coil Between the Great Arteries. Murthy R, Williams MR, Perry JC, Shepard S, DiBardino D. Ann Thorac Surg. 2016 Oct;102(4):e377-8. doi: 10.1016/j.athoracsur.2016.03.109. PMID:27645989

Clinical Experience With the Subcutaneous Implantable Cardioverter-Defibrillator in Adults With Congenital Heart Disease. Moore JP, Mondésert B, Lloyd MS, Cook SC, Zaidi AN, Pass RH, John AS, Fish FA, Shannon KM, Aboulhosn JA, Khairy P; Alliance for Adult Research in Congenital Cardiology (AARCC).. Circ Arrhythm Electrophysiol. 2016 Sep;9(9). pii: e004338. doi: 10.1161/CIRCEP.116.004338. PMID:27635073

Clinical Inferences of Cardiovascular Implantable Electronic Device Analysis at Autopsy. Sinha SK, Crain B, Flickinger K, Calkins H, Rickard J, Cheng A, Berger R, Tomaselli G, Marine JE. J Am Coll Cardiol. 2016 Sep 20;68(12):1255-64. doi: 10.1016/j.jacc.2016.06.052. PMID:27634115

ICD risk stratification studies - EU-CERT-ICD and the European perspective. Seegers J, Bergau L, Tichelbäcker T, Malik M, Zabel M. J Electrocardiol. 2016 Aug 26. pii: S0022-0736(16)30171-6. doi: 10.1016/j.jelectrocard.2016.08.011. [Epub ahead of print] PMID:27623399

Antiarrhythmic Drug Therapy to Avoid Implantable Cardioverter Defibrillator Shocks. Abboud J, R Ehrlich J. Arrhythm Electrophysiol Rev. 2016 Aug;5(2):117-21. doi: 10.15420/AER.2016.10.2. PMID:27617090 | PMCID:PMC5016598

Detection and discrimination of tachycardia in ICDs manufactured by St. Jude Medical. Zdarek J, Israel CW. Herzschrittmacherther Elektrophysiol. 2016 Sep;27(3):226-39. doi: 10.1007/s00399-016-0455-1. PMID:27605236

An analysis of patient-sharing physician networks and implantable cardioverter defibrillator therapy. Moen EL, Austin AM, Bynum JP, Skinner JS, O'Malley AJ. Health Serv Outcomes Res Methodol. 2016 Sep;16(3):132-153. PMID:27597812 | PMCID:PMC5010235

New horizon for infection prevention technology and implantable device. Kondo Y, Ueda M, Kobayashi Y, Schwab JO. J Arrhythm. 2016 Aug;32(4):297-302. doi: 10.1016/j.joa.2016.02.007. Review. PMID:27588153 | PMCID:PMC4996843

Indications and use of the wearable cardiac defibrillator. Sharma PS, Bordachar P, Ellenbogen KA. Eur Heart J. 2016 Aug 25. pii: ehw353. [Epub ahead of print] Review. PMID:27566663

Failed Maximal Defibrillation Threshold Testing in the Subcutaneous Implantable Cardioverter Defibrillator. Levine JD, Ellins C, Winn N, Kim R, Hsu SS, Catanzaro JN. Cardiology. 2016 Aug 23;136(1):29-32. [Epub ahead of print] PMID:27548370

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 30 Quarterly Review of Literature 3rd & 4th Quarter 2016

Psychological correlates, allostatic overload and clinical course in patients with implantable cardioverter defibrillator (ICD). Gostoli S, Bonomo M, Roncuzzi R, Biffi M, Boriani G, Rafanelli C. Int J Cardiol. 2016 Oct 1;220:360-4. doi: 10.1016/j.ijcard.2016.06.246. PMID:27390955

Pre-discharge defibrillation testing: Is it still justified? Kempa M, Królak T, Drelich Ł, Budrejko S, Daniłowicz-Szymanowicz L, Lewicka-Nowak E, Kozłowski D, Raczak G. Cardiol J. 2016 Jul 7. doi: 10.5603/CJ.a2016.0045. [Epub ahead of print] PMID:27387062

Long-term outcome of implantable cardioverter-defibrillator implantation in secondary prevention of sudden cardiac death. Boulé S, Sémichon M, Guédon-Moreau L, Drumez É, Kouakam C, Marquié C, Brigadeau F, Kacet S, Potelle C, Escande W, Souissi Z, Lacroix D, Duhamel A, Klug D. Arch Cardiovasc Dis. 2016 Oct;109(10):517-526. doi: 10.1016/j.acvd.2016.02.008. PMID:27342808

Use of the Wearable Cardioverter Defibrillator in High-Risk Populations. Lamichhane M, Safadi A, Surapaneni P, Salehi N, Thakur RK. Curr Cardiol Rep. 2016 Aug;18(8):78. doi: 10.1007/s11886-016-0746-5. Review. PMID:27319008

Impact of generator replacement on the risk of Fidelis lead fracture. Krahn AD, Bashir J, Birnie DH, Brown J, Spencer JH, Leander C, Estes NA. Heart Rhythm. 2016 Aug;13(8):1618-23. doi: 10.1016/j.hrthm.2016.05.001. PMID:27154229

Implantable cardioverter defibrillator therapy in young patients with and channelopathies: a single Italian centre experience. Migliore F, Silvano M, Zorzi A, Bertaglia E, Siciliano M, Leoni L, De Franceschi P, Iliceto S, Corrado D. J Cardiovasc Med (Hagerstown). 2016 Jul;17(7):485-93. doi: 10.2459/JCM.0000000000000395. PMID:27136702

Implantable Cardioverter Defibrillator Outcomes in Pediatric and Congenital Heart Disease: Time to System Revision. Dechert BE, Bradley DJ, Serwer GA, Dick Ii M, Lapage MJ. Pacing Clin Electrophysiol. 2016 Jul;39(7):703-8. doi: 10.1111/pace.12878. PMID:27119790

Recent developments in the subcutaneous ICD. Willcox ME, Prutkin JM, Bardy GH. Trends Cardiovasc Med. 2016 Aug;26(6):526-35. doi: 10.1016/j.tcm.2016.03.004. Review. PMID:27079889

Subcutaneous defibrillator implantation in pediatric patients. Ertuğrul İ, Karagöz T, Aykan H, Yıldırım I, Özer S, Karagöz H, Yılmaz M Anatol J Cardiol. 2016 Aug;16(8):630-634. doi: 10.5152/AnatolJCardiol.2015.6589. PMID:27004712

Sex difference in appropriate shocks but not mortality during long-term follow-up in patients with implantable cardioverter- defibrillators. Seegers J, Conen D, Jung K, Bergau L, Dorenkamp M, Lüthje L, Sohns C, Sossalla ST, Fischer TH, Hasenfuss G, Friede T, Zabel M. Europace. 2016 Aug;18(8):1194-202. doi: 10.1093/europace/euv361. PMID:26622054 | PMCID:PMC4974631

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 31 Quarterly Review of Literature 3rd & 4th Quarter 2016

Applicability of a risk score for prediction of the long-term benefit of the implantable cardioverter defibrillator in patients receiving cardiac resynchronization therapy. Barra S, Looi KL, Gajendragadkar PR, Khan FZ, Virdee M, Agarwal S. Europace. 2016 Aug;18(8):1187-93. doi: 10.1093/europace/euv352. PMID:26566940

Serum amine-based metabolites and their association with outcomes in primary prevention implantable cardioverter- defibrillator patients. Zhang Y, Blasco-Colmenares E, Harms AC, London B, Halder I, Singh M, Dudley SC, Gutmann R, Guallar E, Hankemeier T, Tomaselli GF, Cheng A. Europace. 2016 Sep;18(9):1383-90. doi: 10.1093/europace/euv342. PMID:26498162 | PMCID:PMC5006959 Sudden Cardiac Death (SCD)

Abnormal autonomic nervous system function in rural Thai men: A potential contributor to their high risk of sudden unexplained nocturnal death syndrome. Chanavirut R, Tong-Un T, Jirakulsomchok D, Wyss JM, Roysommuti S. Int J Cardiol. 2017 Jan 1;226:87-92. doi: 10.1016/j.ijcard.2016.10.001. PMID:27792993

Ventricular Fibrillation in a General Population - A National Database Study. Tseng WC, Wu MH, Chen HC, Kao FY, Huang SK. Circ J. 2016 Oct 25;80(11):2310-2316. PMID:27725494

Can anthropology improve our care of inherited cardiac arrhythmias? A modest proposal. Barlocco F, Olivotto I. Heart Rhythm. 2016 Dec;13(12):2395-2398. doi: 10.1016/j.hrthm.2016.08.042. No abstract available. PMID:27590433

Recent advances in genetic testing and counseling for inherited arrhythmias. Mizusawa Y. J Arrhythm. 2016 Oct;32(5):389-397. Review. PMID:27761163 | PMCID:PMC5063262

Molecular autopsy in victims of inherited arrhythmias. Semsarian C, Ingles J. J Arrhythm. 2016 Oct;32(5):359-365. Review. PMID:27761159 | PMCID:PMC5063264

Optogenetic defibrillation terminates ventricular arrhythmia in mouse hearts and human simulations. Bruegmann T, Boyle PM, Vogt CC, Karathanos TV, Arevalo HJ, Fleischmann BK, Trayanova NA, Sasse P. J Clin Invest. 2016 Oct 3;126(10):3894-3904. doi: 10.1172/JCI88950. PMID:27617859 | PMCID:PMC5096832

Molecular and Functional Characterization of Rare CACNA1C Variants in Sudden Unexplained Death in the Young. Sutphin BS, Boczek NJ, Barajas-Martínez H, Hu D, Ye D, Tester DJ, Antzelevitch C, Ackerman MJ. Congenit Heart Dis. 2016 Dec;11(6):683-692. doi: 10.1111/chd.12371. PMID:27218670

Electrocardiographic features of sudden unexpected death in epilepsy. Chyou JY, Friedman D, Cerrone M, Slater W, Guo Y, Taupin D, O'Rourke S, Priori SG, Devinsky O. Epilepsia. 2016 Jul;57(7):e135-9. doi: 10.1111/epi.13411. PMID:27215589

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 32 Quarterly Review of Literature 3rd & 4th Quarter 2016

Natural and Undetermined Sudden Death: Value of Post-Mortem Genetic Investigation. Sanchez O, Campuzano O, Fernández-Falgueras A, Sarquella-Brugada G, Cesar S, Mademont I, Mates J, Pérez-Serra A, Coll M, Pico F, Iglesias A, Tirón C, Allegue C, Carro E, Gallego MÁ, Ferrer-Costa C, Hospital A, Bardalet N, Borondo JC, Vingut A, Arbelo E, Brugada J, et al. PLoS One. 2016 Dec 8;11(12):e0167358. doi: 10.1371/journal.pone.0167358. PMID:27930701

Sudden cardiac death in South India: Incidence, risk factors and pathology. Srivatsa UN, Swaminathan K, Sithy Athiya Munavarah K, Amsterdam E, Shantaraman K. Indian Pacing Electrophysiol J. 2016 Jul - Aug;16(4):121-125. doi: 10.1016/j.ipej.2016.10.004. PMID:27924759

Fighting sudden cardiac death in India - Knowing your enemy is half the battle. Selvaraj R. Indian Pacing Electrophysiol J. 2016 Jul - Aug;16(4):120. doi: 10.1016/j.ipej.2016.11.001. No abstract available. PMID:27924758

Sudden cardiac death: a nationwide cohort study among the young. Risgaard B. Dan Med J. 2016 Dec;63(12). pii: B5321. PMID:27910804

Etiology of Sudden Cardiac Death in Athletes: Can Autopsy Clarify All the Issues? Angelini P. J Am Coll Cardiol. 2016 Dec 6;68(22):2495-2496. doi: 10.1016/j.jacc.2016.07.787. No abstract available. PMID:27908362

Polymorphisms in the GNAS Gene as Predictors of Ventricular Tachyarrhythmias and Sudden Cardiac Death: Results From the DISCOVERY Trial and Oregon Sudden Unexpected Death Study. Wieneke H, Svendsen JH, Lande J, Spencker S, Martinez JG, Strohmer B, Toivonen L, Le Marec H, Garcia-Fernandez FJ, Corrado D, Huertas-Vazquez A, Uy-Evanado A, Rusinaru C, Reinier K, Foldesi C, Hulak W, Chugh SS, Siffert W. J Am Heart Assoc. 2016 Nov 28;5(12). pii: e003905. PMID:27895044

Role of risk stratification and genetics in sudden cardiac death. Rai V, Agrawal DK. Can J Physiol Pharmacol. 2016 Oct 16:1-14. [Epub ahead of print] PMID:27875062

Population risk stratification for sudden cardiac death: Searching for the needle in the haystack? Viles-Gonzalez JF, Goldberger JJ. Heart Rhythm. 2016 Nov 8. pii: S1547-5271(16)31008-6. doi: 10.1016/j.hrthm.2016.11.007. [Epub ahead of print] No abstract available. PMID:27833008

Reply: How Often Does Athlete Sudden Cardiac Death Occur Outside the Context of Exertion? Finocchiaro G, Papadakis M, Robertus JL, Dhutia H, Steriotis AK, Tome M, Mellor G, Merghani A, Malhotra A, Behr E, Sharma S, Sheppard MN. J Am Coll Cardiol. 2016 Nov 8;68(19):2126. doi: 10.1016/j.jacc.2016.07.778. No abstract available. PMID:27810056

How Often Does Athlete Sudden Cardiac Death Occur Outside the Context of Exertion? Karam N, Pechmajou L, Marijon E, Jouven X. J Am Coll Cardiol. 2016 Nov 8;68(19):2125-2126. doi: 10.1016/j.jacc.2016.05.102. No abstract available. PMID:27810055

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 33 Quarterly Review of Literature 3rd & 4th Quarter 2016

Demographics and Epidemiology of Sudden Deaths in Young Competitive Athletes: From the United States National Registry. Maron BJ, Haas TS, Ahluwalia A, Murphy CJ, Garberich RF. Am J Med. 2016 Nov;129(11):1170-1177. doi: 10.1016/j.amjmed.2016.02.031. PMID:27039955

Incidence and Etiology of Sudden Cardiac Arrest and Death in High School Athletes in the United States. Harmon KG, Asif IM, Maleszewski JJ, Owens DS, Prutkin JM, Salerno JC, Zigman ML, Ellenbogen R, Rao AL, Ackerman MJ, Drezner JA. Mayo Clin Proc. 2016 Nov;91(11):1493-1502. doi: 10.1016/j.mayocp.2016.07.021. PMID:27692971

Sudden neurologic death masquerading as out-of-hospital sudden cardiac death. Kim AS, Moffatt E, Ursell PC, Devinsky O, Olgin J, Tseng ZH. Neurology. 2016 Oct 18;87(16):1669-1673. PMID:27638923 | PMCID:PMC5085076

Unmanned aerial vehicles (drones) in out-of-hospital-cardiac-arrest. Claesson A, Fredman D, Svensson L, Ringh M, Hollenberg J, Nordberg P, Rosenqvist M, Djarv T, Österberg S, Lennartsson J, Ban Y. Scand J Trauma Resusc Emerg Med. 2016 Oct 12;24(1):124. PMID:27729058 | PMCID:PMC5059909

Does Sudden Unexplained Nocturnal Death Syndrome Remain the Autopsy-Negative Disorder: A Gross, Microscopic, and Molecular Autopsy Investigation in Southern China. Zhang L, Tester DJ, Lang D, Chen Y, Zheng J, Gao R, Corliss RF, Tang S, Kyle JW, Liu C, Ackerman MJ, Makielski JC, Cheng J. Mayo Clin Proc. 2016 Nov;91(11):1503-1514. doi: 10.1016/j.mayocp.2016.06.031. PMID:27707468 | PMCID:PMC5097692

Traditional and novel electrocardiographic conduction and repolarization markers of sudden cardiac death. Tse G, Yan BP. Europace. 2016 Oct 4. pii: euw280. [Epub ahead of print] Review. PMID:27702850

Sudden death and cardiac arrest without phenotype: the utility of genetic testing. Wijeyeratne YD, Behr ER. Trends Cardiovasc Med. 2016 Aug 31. pii: S1050-1738(16)30134-7. doi: 10.1016/j.tcm.2016.08.010. [Epub ahead of print] Review. PMID:27692676

Sudden Cardiac Death in Athletes: Still Much to Learn. Sweeting J, Semsarian C. Cardiol Clin. 2016 Nov;34(4):531-541. doi: 10.1016/j.ccl.2016.06.003. Review. PMID:27692222

Sudden Cardiac Death in Children and Young Adults. Jiménez-Jáimez J, Jiménez-Fernández M, Tercedor L. N Engl J Med. 2016 Sep 29;375(13):1301-2. doi: 10.1056/NEJMc1609620#SA1. No abstract available. PMID:27682049

Sudden Cardiac Death in Children and Young Adults. [No authors listed] N Engl J Med. 2016 Sep 29;375(13):1301-2. doi: 10.1056/NEJMc1609620. No abstract available. PMID:27682048

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 34 Quarterly Review of Literature 3rd & 4th Quarter 2016

Sudden cardiac death: A reappraisal. Steinberg C, Laksman ZW, Krahn AD. Trends Cardiovasc Med. 2016 Nov;26(8):709-719. doi: 10.1016/j.tcm.2016.05.006. Review. PMID:27665048

Medico-legal perspectives on sudden cardiac death in young athletes. Oliva A, Grassi VM, Campuzano O, Brion M, Arena V, Partemi S, Coll M, Pascali VL, Brugada J, Carracedo A, Brugada R. Int J Legal Med. 2016 Sep 21. [Epub ahead of print] Review. PMID:27654714

Genetic investigation of 100 heart genes in sudden unexplained death victims in a forensic setting. Christiansen SL, Hertz CL, Ferrero-Miliani L, Dahl M, Weeke PE, LuCamp, Ottesen GL, Frank-Hansen R, Bundgaard H, Morling N. Eur J Hum Genet. 2016 Dec;24(12):1797-1802. doi: 10.1038/ejhg.2016.118. PMID:27650965

Cardiac Abnormalities in First-Degree Relatives of Unexplained Cardiac Arrest Victims: A Report From the Cardiac Arrest Survivors With Preserved Ejection Fraction Registry. Steinberg C, Padfield GJ, Champagne J, Sanatani S, Angaran P, Andrade JG, Roberts JD, Healey JS, Chauhan VS, Birnie DH, Janzen M, Gerull B, Klein GJ, Leather R, Simpson CS, Seifer C, Talajic M, Gardner M, Krahn AD. Circ Arrhythm Electrophysiol. 2016 Sep;9(9). pii: e004274. doi: 10.1161/CIRCEP.115.004274. PMID:27635072

Depression and Risk of Sudden Cardiac Death and Arrhythmias: A Meta-Analysis. Shi S, Liu T, Liang J, Hu D, Yang B. Psychosom Med. 2016 Sep 13. [Epub ahead of print] PMID:27627224

Anatomopathological changes of the cardiac conduction system in sudden cardiac death, particularly in infants: advances over the last 25 years. Ottaviani G, Buja LM. Cardiovasc Pathol. 2016 Nov - Dec;25(6):489-499. doi: 10.1016/j.carpath.2016.08.005. Review. PMID:27616614

Cardiorespiratory fitness and exercise-induced ST segment depression in assessing the risk of sudden cardiac death in men. Hagnäs MJ, Lakka TA, Kurl S, Rauramaa R, Mäkikallio TH, Savonen K, Laukkanen JA. Heart. 2016 Sep 7. pii: heartjnl-2015-309217. doi: 10.1136/heartjnl-2015-309217. [Epub ahead of print] PMID:27604814

Thyroid Function and Sudden Cardiac Death: A Prospective Population-Based Cohort Study. Chaker L, van den Berg ME, Niemeijer MN, Franco OH, Dehghan A, Hofman A, Rijnbeek PR, Deckers JW, Eijgelsheim M, Stricker BH, Peeters RP. Circulation. 2016 Sep 6;134(10):713-22. doi: 10.1161/CIRCULATIONAHA.115.020789. PMID:27601558

CARDIOSTIM-EHRA EUROPACE 2016 included recommendations to prevent sudden cardiac death in athletes. [No authors listed] Eur Heart J. 2016 Aug 21;37(32):2511. doi: 10.1093/eurheartj/ehw310. No abstract available. PMID:27587477

Nonischemic left ventricular scar and cardiac sudden death in the young. di Gioia CR, Giordano C, Cerbelli B, Pisano A, Perli E, De Dominicis E, Poscolieri B, Palmieri V, Ciallella C, Zeppilli P, d'Amati G. Hum Pathol. 2016 Dec;58:78-89. doi: 10.1016/j.humpath.2016.08.004. PMID:27569295

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 35 Quarterly Review of Literature 3rd & 4th Quarter 2016

Incidence of sudden cardiac death in professional cycling: Sudden cardiac death and exercise. Santos-Lozano A, Sanchis-Gomar F, Garatachea N, Arrarás-Flores Á, Pareja-Galeano H, Fiuza-Luces C, Joyner MJ, Lucia A. Int J Cardiol. 2016 Nov 15;223:222-223. doi: 10.1016/j.ijcard.2016.08.155. No abstract available. PMID:27541659

Sudden Cardiac Death Due to Deficiency of the Mitochondrial Inorganic Pyrophosphatase PPA2. Kennedy H, Haack TB, Hartill V, Mataković L, Baumgartner ER, Potter H, Mackay R, Alston CL, O'Sullivan S, McFarland R, Connolly G, Gannon C, King R, Mead S, Crozier I, Chan W, Florkowski CM, Sage M, Höfken T, Alhaddad B, Kremer LS, Kopajtich R, et al. Am J Hum Genet. 2016 Sep 1;99(3):674-82. doi: 10.1016/j.ajhg.2016.06.027. PMID:27523597 | PMCID:PMC5011043

Polymorphic Ventricular Tachycardia/Ventricular Fibrillation and Sudden Cardiac Death in the Normal Heart. Shah AJ, Hocini M, Denis A, Derval N, Sacher F, Jais P, Haissaguerre M. Card Electrophysiol Clin. 2016 Sep;8(3):581-91. doi: 10.1016/j.ccep.2016.04.007. Review. PMID:27521091

Clinical Presentation of Pediatric Patients at Risk for Sudden Cardiac Arrest. Dalal A, Czosek RJ, Kovach J, von Alvensleben JC, Valdes S, Etheridge SP, Ackerman MJ, Auld D, Huckaby J, McCracken C, Campbell R. J Pediatr. 2016 Oct;177:191-6. doi: 10.1016/j.jpeds.2016.06.088. PMID:27502104

Sudden cardiac death in epilepsy disappoints, but epileptologists keep faith. Scorza FA, Cavalheiro EA, Costa JC. Arq Neuropsiquiatr. 2016 Jul;74(7):570-3. doi: 10.1590/0004-282X20160086. PMID:27487377

Leaky RyR2 channels unleash a brainstem spreading depolarization mechanism of sudden cardiac death. Aiba I, Wehrens XH, Noebels JL. Proc Natl Acad Sci U S A. 2016 Aug 16;113(33):E4895-903. doi: 10.1073/pnas.1605216113. PMID:27482086 | PMCID:PMC4995941

Management of survivors of cardiac arrest - the importance of genetic investigation. Schwartz PJ, Dagradi F. Nat Rev Cardiol. 2016 Sep;13(9):560-6. doi: 10.1038/nrcardio.2016.104. Review. PMID:27383078

Editorial Commentary: Sudden cardiac death: We shall never surrender. Lemery R. Trends Cardiovasc Med. 2016 Nov;26(8):720-721. doi: 10.1016/j.tcm.2016.05.011. No abstract available. PMID:27378667

A Prospective Study of Sudden Cardiac Death among Children and Young Adults. Bagnall RD, Weintraub RG, Ingles J, Duflou J, Yeates L, Lam L, Davis AM, Thompson T, Connell V, Wallace J, Naylor C, Crawford J, Love DR, Hallam L, White J, Lawrence C, Lynch M, Morgan N, James P, du Sart D, Puranik R, Langlois N, et al. N Engl J Med. 2016 Jun 23;374(25):2441-52. doi: 10.1056/NEJMoa1510687. PMID:27332903

Is There a Role for Genetics in the Prevention of Sudden Cardiac Death? Faragli A, Underwood K, Priori SG, Mazzanti A. J Cardiovasc Electrophysiol. 2016 Sep;27(9):1124-32. doi: 10.1111/jce.13028. PMID:27279603

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 36 Quarterly Review of Literature 3rd & 4th Quarter 2016

Genetic variations involved in sudden cardiac death and their associations and interactions. Wei D, Tao L, Huang M. Heart Fail Rev. 2016 Jul;21(4):401-14. doi: 10.1007/s10741-016-9563-6. PMID:27241195

Post-mortem whole-exome sequencing (WES) with a focus on cardiac disease-associated genes in five young sudden unexplained death (SUD) cases. Neubauer J, Haas C, Bartsch C, Medeiros-Domingo A, Berger W. Int J Legal Med. 2016 Jul;130(4):1011-21. doi: 10.1007/s00414-016-1317-4. Erratum in: Int J Legal Med. 2016 Jul;130(4):1023. PMID:26846766

The CAHP (Cardiac Arrest Hospital Prognosis) score: a tool for risk stratification after out-of-hospital cardiac arrest. Maupain C, Bougouin W, Lamhaut L, Deye N, Diehl JL, Geri G, Perier MC, Beganton F, Marijon E, Jouven X, Cariou A, Dumas F. Eur Heart J. 2016 Nov 7;37(42):3222-3228. PMID:26497161

Detection of sudden death syndromes in New Zealand. Earle N, Crawford J, Gibson K, Love D, Hayes I, Neas K, Stiles M, Graham M, Donoghue T, Aitken A, Skinner J. N Z Med J. 2016 Nov 18;129(1445):67-74. PMID:27857240

Risk stratification for sudden cardiac death in North America - current perspectives. Buxton AE, Waks JW, Shen C, Chen PS. J Electrocardiol. 2016 Jul 29. pii: S0022-0736(16)30096-6. doi: 10.1016/j.jelectrocard.2016.07.018. [Epub ahead of print] PMID:27524476 Screening -Corrected QT and JT Intervals in Electrocardiograms in Physically Fit Students and Student Athletes. Misigoj-Durakovic M, Durakovic Z, Prskalo I. Ann Noninvasive Electrocardiol. 2016 Nov;21(6):595-603. doi: 10.1111/anec.12374. PMID:27194642

Interassociation Consensus Statement on Cardiovascular Care of College Student-Athletes. Hainline B, Drezner JA, Baggish A, Harmon KG, Emery MS, Myerburg RJ, Sanchez E, Molossi S, Parsons JT, Thompson PD. J Am Coll Cardiol. 2016 Jun 28;67(25):2981-95. doi: 10.1016/j.jacc.2016.03.527. Review. PMID:27090220

Coronary Artery Evaluation by Screening Echocardiogram in Intercollegiate Athletes. Hoyt WJ, Dean PN, Schneider DS, Conaway MR, Kramer CM, Battle RW. Med Sci Sports Exerc. 2016 Dec 6. [Epub ahead of print] PMID:27926615

Screening entire healthcare system ECG database: Association of deep terminal negativity of P wave in lead V1 and ECG referral with mortality. Junell A, Thomas J, Hawkins L, Sklenar J, Feldman T, Henrikson CA, Tereshchenko LG. Int J Cardiol. 2016 Nov 10;228:219-224. doi: 10.1016/j.ijcard.2016.11.128. [Epub ahead of print] PMID:27865189

Pre-participation cardiovascular evaluation for athletic participants to prevent sudden death: Position paper from the EHRA and the EACPR, branches of the ESC. Endorsed by APHRS, HRS, and SOLAECE. Mont L, Pelliccia A, Sharma S, Biffi A, Borjesson M, Brugada Terradellas J, Carré F, Guasch E, Heidbuchel H, La Gerche A, Lampert R, McKenna W, Papadakis M, Priori SG, Scanavacca M, Thompson P, Sticherling C, Viskin S, Wilson M, Corrado D; Reviewers., Lip GY, et al. Eur J Prev Cardiol. 2016 Nov 4. pii: 2047487316676042. [Epub ahead of print] No abstract available. PMID:27815537

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 37 Quarterly Review of Literature 3rd & 4th Quarter 2016

Why cardiovascular screening in young athletes can save lives: a critical review. Drezner JA, Harmon KG, Asif IM, Marek JC. Br J Sports Med. 2016 Nov;50(22):1376-1378. doi: 10.1136/bjsports-2016-096606. PMID:27418320

Early Screening for Cardiovascular Abnormalities With Preparticipation Echocardiography: Feasibility Study. Gleason CN, Kerkhof DL, Cilia EA, Lanyi MA, Finnoff J, Sugimoto D, Corrado GD. Clin J Sport Med. 2016 Oct 13. [Epub ahead of print] PMID:27755012

Infographic: AMSSM position statement on cardiovascular preparticipation screening in athletes: Current evidence, knowledge gaps, recommendations and future directions. Drezner JA, O'Connor FG, Harmon KG, Fields KB, Asplund CA, Asif IM, Price DE, Dimeff RJ, Bernhardt DT, Roberts WO. Br J Sports Med. 2016 Oct 4. pii: bjsports-2016-096946. doi: 10.1136/bjsports-2016-096946. [Epub ahead of print] No abstract available. PMID:27707743

Detecting Underlying in Young Competitive Athletes. McKinney J, Lithwick DJ, Morrison BN, Nazzari H, Luong M, Fordyce CB, Taunton J, Krahn AD, Heilbron B, Isserow S. Can J Cardiol. 2016 Jun 23. pii: S0828-282X(16)30170-2. doi: 10.1016/j.cjca.2016.06.007. [Epub ahead of print] PMID:27692657

Using the 12-Lead Electrocardiogram in the Care of Athletic Patients. Yeo TJ, Sharma S. Cardiol Clin. 2016 Nov;34(4):543-555. doi: 10.1016/j.ccl.2016.06.004. Review. PMID:27692223

AMSSM Position Statement on Cardiovascular Preparticipation Screening in Athletes: current evidence, knowledge gaps, recommendations and future directions. Drezner JA, O'Connor FG, Harmon KG, Fields KB, Asplund CA, Asif IM, Price DE, Dimeff RJ, Bernhardt DT, Roberts WO. Br J Sports Med. 2016 Sep 22. pii: bjsports-2016-096781. doi: 10.1136/bjsports-2016-096781. [Epub ahead of print] PMID:27660369

The Role of Genetic Testing in the Identification of Young Athletes with Inherited Primitive Cardiac Disorders at Risk of Exercise Sudden Death. Tiziano FD, Palmieri V, Genuardi M, Zeppilli P. Front Cardiovasc Med. 2016 Aug 26;3:28. doi: 10.3389/fcvm.2016.00028. Review. PMID:27617263 | PMCID:PMC5000131

AMSSM Position Statement on Cardiovascular Preparticipation Screening in Athletes: Current Evidence, Knowledge Gaps, Recommendations, and Future Directions. Drezner JA, OʼConnor FG, Harmon KG, Fields KB, Asplund CA, Asif IM, Price DE, Dimeff RJ, Bernhardt DT, Roberts WO. Clin J Sport Med. 2016 Sep;26(5):347-61. doi: 10.1097/JSM.0000000000000382. PMID:27598018

Routine ECG Screening of Young Athletes: Can This Strategy Ever Be Cost Effective? Lavie CJ, Harmon KG. J Am Coll Cardiol. 2016 Aug 16;68(7):712-4. doi: 10.1016/j.jacc.2016.06.017. No abstract available. PMID:27515330

Cost Implications of Using Different ECG Criteria for Screening Young Athletes in the United Kingdom. Dhutia H, Malhotra A, Gabus V, Merghani A, Finocchiaro G, Millar L, Narain R, Papadakis M, Naci H, Tome M, Sharma S. J Am Coll Cardiol. 2016 Aug 16;68(7):702-11. doi: 10.1016/j.jacc.2016.05.076. PMID:27515329

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 38 Quarterly Review of Literature 3rd & 4th Quarter 2016

Electrocardiographic Screening in National Collegiate Athletic Association Athletes. Drezner JA, Owens DS, Prutkin JM, Salerno JC, Harmon KG, Prosise S, Clark A, Asif IM. Am J Cardiol. 2016 Sep 1;118(5):754-9. doi: 10.1016/j.amjcard.2016.06.004. PMID:27496294

Optimizing QT Interval Measurement for the Preparticipation Screening of Young Athletes. Pickham D, Hsu D, Soofi M, Goldberg JM, Saini D, Hadley D, Perez M, Froelicher VF. Med Sci Sports Exerc. 2016 Sep;48(9):1745-50. doi: 10.1249/MSS.0000000000000962. PMID:27116644

Sports-related sudden cardiac death: How to prove an effect of preparticipation screening? Risgaard B, Tfelt-Hansen J, Winkel BG. Heart Rhythm. 2016 Jul;13(7):1560-2. doi: 10.1016/j.hrthm.2016.03.043. No abstract available. PMID:27016473

Sports-related sudden cardiac death in Switzerland classified by static and dynamic components of exercise. Gräni C, Chappex N, Fracasso T, Vital C, Kellerhals C, Schmied C, Saguner AM, Trachsel LD, Eser P, Michaud K, Wilhelm M. Eur J Prev Cardiol. 2016 Jul;23(11):1228-36. doi: 10.1177/2047487316632967. PMID:26915579

Positive predictive value of cardiovascular diagnoses in the Danish National Patient Registry: a validation study. Sundbøll J, Adelborg K, Munch T, Frøslev T, Sørensen HT, Bøtker HE, Schmidt M. BMJ Open. 2016 Nov 18;6(11):e012832. doi: 10.1136/bmjopen-2016-012832. PMID:27864249 | PMCID:PMC5129042 CPR

Implementing Cardiopulmonary Resuscitation Training Programs in High Schools: Iowa's Experience. Hoyme DB, Atkins DL. J Pediatr. 2016 Nov 14. pii: S0022-3476(16)31170-2. doi: 10.1016/j.jpeds.2016.10.037. [Epub ahead of print] PMID:27852456

Development of a video-based education and process change intervention to improve advance cardiopulmonary resuscitation decision-making. Waldron N, Johnson CE, Saul P, Waldron H, Chong JC, Hill AM, Hayes B. BMC Health Serv Res. 2016 Oct 6;16(1):555. PMID:27716183 | PMCID:PMC5053041

High School CPR/AED Training in Washington State. Salvatierra GG, Palazzo SJ, Emery A. Public Health Nurs. 2016 Oct 5. doi: 10.1111/phn.12293. [Epub ahead of print] PMID:27704621

Video-Only Cardiopulmonary Resuscitation Education for High-Risk Families Before Hospital Discharge: A Multicenter Pragmatic Trial. Blewer AL, Putt ME, Becker LB, Riegel BJ, Li J, Leary M, Shea JA, Kirkpatrick JN, Berg RA, Nadkarni VM, Groeneveld PW, Abella BS; CHIP Study Group.. Circ Cardiovasc Qual Outcomes. 2016 Oct 4. pii: CIRCOUTCOMES.116.002493. [Epub ahead of print] PMID:27703033

The Effect of the Duration of Basic Life Support Training on the Learners' Cardiopulmonary and Automated External Defibrillator Skills. Lee JH, Cho Y, Kang KH, Cho GC, Song KJ, Lee CH. Biomed Res Int. 2016;2016:2420568. doi: 10.1155/2016/2420568. PMID:27529066 | PMCID:PMC4978818

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 39 Quarterly Review of Literature 3rd & 4th Quarter 2016

Survivors' quality of life after cardiopulmonary resuscitation: an integrative review of the literature. Haydon G, van der Riet P, Maguire J. Scand J Caring Sci. 2016 Jul 21. doi: 10.1111/scs.12323. [Epub ahead of print] Review. PMID:27440375

Compression-only CPR training in elementary schools and student attitude toward CPR. Kitamura T, Nishiyama C, Murakami Y, Yonezawa T, Nakai S, Hamanishi M, Marukawa S, Sakamoto T, Iwami T. Pediatr Int. 2016 Aug;58(8):698-704. doi: 10.1111/ped.12881. PMID:26663150 Automated External Defibrillator (AED)

Rise and Shock: Optimal Defibrillator Placement in a High-rise Building. Chan TC. Prehosp Emerg Care. 2016 Nov 18:1-6. [Epub ahead of print] PMID:27858504

Characteristics of automated external defibrillator coverage in Philadelphia, PA, based on land use and estimated risk. Chrisinger BW, Grossestreuer AV, Laguna MC, Griffis HM, Branas CC, Wiebe DJ, Merchant RM. Resuscitation. 2016 Dec;109:9-15. doi: 10.1016/j.resuscitation.2016.09.021. PMID:27702581

Assessment of knowledge and skills in using an Automated External Defibrillator (AED) by university students. A quasi- experimental research. Basanta Camiño S, Navarro Patón R, Freire Tellado M, Barcala Furelos R, Pavón Prieto MP, Fernández López M, Neira Pájaro MA. Med Intensiva. 2016 Oct 20. pii: S0210-5691(16)30174-7. doi: 10.1016/j.medin.2016.07.008. [Epub ahead of print] English, Spanish. PMID:27773493

Optimization of automated external defibrillator deployment outdoors: An evidence-based approach. Dahan B, Jabre P, Karam N, Misslin R, Bories MC, Tafflet M, Bougouin W, Jost D, Beganton F, Beal G, Pelloux P, Marijon E, Jouven X. Resuscitation. 2016 Nov;108:68-74. doi: 10.1016/j.resuscitation.2016.09.010. PMID:27670358

National experience with long-term use of the wearable cardioverter defibrillator in patients with cardiomyopathy. Lamichhane M, Gardiner JC, Bianco NR, Szymkiewicz SJ, Thakur RK. J Interv Card Electrophysiol. 2016 Oct 17. [Epub ahead of print] PMID:27752809

The wearable cardioverter-defibrillator: current technology and evolving indications. Reek S, Burri H, Roberts PR, Perings C, Epstein AE, Klein HU. Europace. 2016 Oct 4. pii: euw180. [Epub ahead of print] Review. PMID:27702851

Quality of basic life support education and automated external defibrillator setting in schools in Ishikawa, Japan. Takamura A, Ito S, Maruyama K, Ryo Y, Saito M, Fujimura S, Ishiura Y, Hori A. Pediatr Int. 2016 Sep 2. doi: 10.1111/ped.13155. [Epub ahead of print] PMID:27589486

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 40 Quarterly Review of Literature 3rd & 4th Quarter 2016

Installation of multiple automated external defibrillators to prevent sudden death in school-aged children. Higaki T, Chisaka T, Moritani T, Ohta M, Takata H, Yamauchi T, Yamaguchi Y, Konishi K, Yamamoto E, Ochi F, Eguchi M, Eguchi-Ishimae M, Mitani Y, Ishii E. Pediatr Int. 2016 Aug 26. doi: 10.1111/ped.13143. [Epub ahead of print] PMID:27562900

Overcoming Spatial and Temporal Barriers to Public Access Defibrillators Via Optimization. Sun CL, Demirtas D, Brooks SC, Morrison LJ, Chan TC. J Am Coll Cardiol. 2016 Aug 23;68(8):836-45. doi: 10.1016/j.jacc.2016.03.609. PMID:27539176 | PMCID:PMC4992180

Experience With the Wearable Cardioverter-Defibrillator in Patients at High Risk for Sudden Cardiac Death. Wäßnig NK, Günther M, Quick S, Pfluecke C, Rottstädt F, Szymkiewicz SJ, Ringquist S, Strasser RH, Speiser U. Circulation. 2016 Aug 30;134(9):635-43. doi: 10.1161/CIRCULATIONAHA.115.019124. PMID:27458236 | PMCID:PMC4998124

Public-access AED pad application and outcomes for out-of-hospital cardiac arrests in Osaka, Japan. Kiyohara K, Kitamura T, Sakai T, Nishiyama C, Nishiuchi T, Hayashi Y, Sakamoto T, Marukawa S, Iwami T. Resuscitation. 2016 Sep;106:70-5. doi: 10.1016/j.resuscitation.2016.06.025. PMID:27373223

Automated external defibrillation skills by naive schoolchildren. Jorge-Soto C, Abelairas-Gómez C, Barcala-Furelos R, Garrido-Viñas A, Navarro-Patón R, Muiño-Piñeiro M, Díaz-Pereira MP, Rodríguez-Núñez A. Resuscitation. 2016 Sep;106:37-41. doi: 10.1016/j.resuscitation.2016.06.007. PMID:27353288

The wearable cardioverter defibrillator as a bridge to implantable defibrillator post myocardial infarction: what do we know? Barsheshet A, Vamvouris T, Goldenberg I. Expert Rev Med Devices. 2016 Jul;13(7):627-32. doi: 10.1080/17434440.2016.1200969. PMID:27333818

Improving Defibrillation Efficiency in Area Schools. Thomas VC, Shen JJ, Stanley R, Dahlke J, McPartlin S, Row L. Congenit Heart Dis. 2016 Jul;11(4):359-64. doi: 10.1111/chd.12375. PMID:27237434

Innovative cardiopulmonary resuscitation and automated external defibrillator programs in schools: Results from the Student Program for Olympic Resuscitation Training in Schools (SPORTS) study. Vetter VL, Haley DM, Dugan NP, Iyer VR, Shults J. Resuscitation. 2016 Jul;104:46-52. doi: 10.1016/j.resuscitation.2016.04.010. PMID:27131845

Prevention of sudden cardiac death by the wearable cardioverter defibrillator in a young patient with cardiac sarcoidosis. Andrié R, Gaertner FC, Skowasch D. Thorax. 2016 Dec 5. pii: thoraxjnl-2016-208834. doi: 10.1136/thoraxjnl-2016-208834. [Epub ahead of print] No abstract available. PMID:27920335

The wearable cardioverter-defibrillator in a real-world clinical setting: experience in 102 consecutive patients. Erath JW, Vamos M, Sirat AS, Hohnloser SH. Clin Res Cardiol. 2016 Nov 25. [Epub ahead of print] PMID:27888304

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 41 Quarterly Review of Literature 3rd & 4th Quarter 2016

Hypertrophic Cardiomyopathy (HCM)

Exercise-induced quantitative microvolt T-wave alternans in hypertrophic cardiomyopathy. de Oliveira Antunes M, Samesima N, Pereira Filho HG, Matsumoto AY, Verrier RL, Pastore CA, Arteaga-Fernández E, Mady C. J Electrocardiol. 2016 Oct 28. pii: S0022-0736(16)30280-1. doi: 10.1016/j.jelectrocard.2016.10.010. [Epub ahead of print] PMID:27916321

Safety profile and utility of treadmill exercise in patients with high-gradient hypertrophic cardiomyopathy. Sorensen LL, Liang HY, Pinheiro A, Hilser A, Dimaano V, Olsen NT, Hansen TF, Sogaard P, Nowbar A, Pisanello C, Pozios I, Phillip S, Zhou X, Abraham R, Abraham TP. Am Heart J. 2016 Oct 20;184:47-54. doi: 10.1016/j.ahj.2016.10.010. [Epub ahead of print] PMID:27892886

Genetic testing in cardiomyopathies: an update on indications and benefits. Aleksova N, Rutberg J, Green M, Haddad H. Curr Opin Cardiol. 2016 Nov 18. [Epub ahead of print] PMID:27861187

Recent Advances in the Molecular Genetics of Familial Hypertrophic Cardiomyopathy in South Asian Descendants. Kraker J, Viswanathan SK, Knöll R, Sadayappan S. Front Physiol. 2016 Oct 28;7:499. Review. PMID:27840609 | PMCID:PMC5083855

ECG-derived spatial QRS-T angle is strongly associated with hypertrophic cardiomyopathy. Cortez D, Schlegel TT, Ackerman MJ, Bos JM. J Electrocardiol. 2016 Oct 14. pii: S0022-0736(16)30271-0. doi: 10.1016/j.jelectrocard.2016.10.001. [Epub ahead of print] PMID:27839835

Grey zones in cardiomyopathies: defining boundaries between genetic and iatrogenic disease. Quarta G, Papadakis M, Donna PD, Maurizi N, Iacovoni A, Gavazzi A, Senni M, Olivotto I. Nat Rev Cardiol. 2016 Nov 10. doi: 10.1038/nrcardio.2016.175. [Epub ahead of print] Review. PMID:27830773

Relationship between aetiology and left ventricular systolic dysfunction in hypertrophic cardiomyopathy. Rosmini S, Biagini E, O'Mahony C, Bulluck H, Ruozi N, Lopes LR, Guttmann O, Reant P, Quarta CC, Pantazis A, Tome- Esteban M, Mckenna WJ, Rapezzi C, Elliott PM. Heart. 2016 Oct 24. pii: heartjnl-2016-310138. doi: 10.1136/heartjnl-2016-310138. [Epub ahead of print] PMID:27798053

Exercise in patients with hypertrophic cardiomyopathy: A review of current evidence, national guideline recommendations and a proposal for a new direction to fitness. Hindieh W, Adler A, Weissler-Snir A, Fourey D, Harris S, Rakowski H. J Sci Med Sport. 2016 Sep 20. pii: S1440-2440(16)30207-9. doi: 10.1016/j.jsams.2016.09.007. [Epub ahead of print] Review. PMID:27707537

Exercise Prescription for the Athlete with Cardiomyopathy. Saberi S, Day SM. Cardiol Clin. 2016 Nov;34(4):591-601. doi: 10.1016/j.ccl.2016.06.008. Review. PMID:27692227

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 42 Quarterly Review of Literature 3rd & 4th Quarter 2016

Update on hypertrophic cardiomyopathy and a guide to the guidelines. Sen-Chowdhry S, Jacoby D, Moon JC, McKenna WJ. Nat Rev Cardiol. 2016 Nov;13(11):651-675. doi: 10.1038/nrcardio.2016.140. Review. PMID:27681577

Approaching the facts between genetic mutation and clinical practice of hypertrophic cardiomyopathy: A case report with RAF1 770C>T mutant. Wang X, Zhou K, Hua Y, Li Y. Medicine (Baltimore). 2016 Sep;95(37):e4815. doi: 10.1097/MD.0000000000004815. PMID:27631234

Histological and Histometric Characterization of Myocardial Fibrosis in End-Stage Hypertrophic Cardiomyopathy: A Clinical-Pathological Study of 30 Explanted Hearts. Galati G, Leone O, Pasquale F, Olivotto I, Biagini E, Grigioni F, Pilato E, Lorenzini M, Corti B, Foà A, Agostini V, Cecchi F, Rapezzi C. Circ Heart Fail. 2016 Sep;9(9). pii: e003090. doi: 10.1161/CIRCHEARTFAILURE.116.003090. PMID:27618852

Comparison of Long-Term Outcome between Apical and Asymmetric Septal Hypertrophic Cardiomyopathy. An S, Fan C, Yan L, Cai C, Yang Y, Zhai S, Zhao S, Liu Y, Duan F, Wang Z, Li Y. Cardiology. 2016 Sep 6;136(2):108-114. [Epub ahead of print] PMID:27595481

Usefulness of 14-Day Holter for Detection of Nonsustained Ventricular Tachycardia in Patients With Hypertrophic Cardiomyopathy. Weissler-Snir A, Chan RH, Adler A, Care M, Chauhan V, Gollob MH, Ziv-Baran T, Fourey D, Hindieh W, Rakowski H, Spears DA. Am J Cardiol. 2016 Oct 15;118(8):1258-1263. doi: 10.1016/j.amjcard.2016.07.043. PMID:27567133

Analysis of SCN5A Gene Variants in East Slovak Patients with Cardiomyopathy. Priganc M, Zigová M, Boroňová I, Bernasovská J, Dojčáková D, Szabadosová V, Mydlárová Blaščáková M, Tóthová I, Kmec J, Bernasovský I. J Clin Lab Anal. 2016 Aug 24. doi: 10.1002/jcla.22037. [Epub ahead of print] PMID:27554632

Apical hypertrophic cardiomyopathy: Present status. Jan MF, Todaro MC, Oreto L, Tajik AJ. Int J Cardiol. 2016 Nov 1;222:745-59. doi: 10.1016/j.ijcard.2016.07.154. Review. PMID:27521551

Early gadolinium enhancement in hypertrophic cardiomyopathy: a potential premature marker of myocardial damage. Pozo E, Viliani D, Aguirre N, Agudo-Quilez P, Olivera MJ, Caballero P, Jiménez-Borreguero LJ, Alfonso F. Int J Cardiovasc Imaging. 2016 Nov;32(11):1635-1643. PMID:27503551

Value of Genetic Testing for the Prediction of Long-Term Outcome in Patients With Hypertrophic Cardiomyopathy. van Velzen HG, Vriesendorp PA, Oldenburg RA, van Slegtenhorst MA, van der Velden J, Schinkel AF, Michels M. Am J Cardiol. 2016 Sep 15;118(6):881-7. doi: 10.1016/j.amjcard.2016.06.038. PMID:27476098

Hypertrophic cardiomyopathy: New approaches and a time to reappraise older approaches. Takayama H, Chung WK, Maurer MS, Ginns JN. J Thorac Cardiovasc Surg. 2016 Oct;152(4):983-8. doi: 10.1016/j.jtcvs.2016.06.034. No abstract available. PMID:27474178

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 43 Quarterly Review of Literature 3rd & 4th Quarter 2016

Identification of LAMP2 Mutations in Early-Onset Danon Disease With Hypertrophic Cardiomyopathy by Targeted Next- Generation Sequencing. Fu L, Luo S, Cai S, Hong W, Guo Y, Wu J, Liu T, Zhao C, Li F, Huang H, Huang M, Wang J. Am J Cardiol. 2016 Sep 15;118(6):888-94. doi: 10.1016/j.amjcard.2016.06.037. PMID:27460667

Current perspectives in hypertrophic cardiomyopathy with the focus on patients in the Finnish population: a review. Kuusisto J, Sipola P, Jääskeläinen P, Naukkarinen A. Ann Med. 2016 Nov;48(7):496-508. PMID:27460395

Prognostic Value of LGE-CMR in HCM: A Meta-Analysis. Weng Z, Yao J, Chan RH, He J, Yang X, Zhou Y, He Y. JACC Cardiovasc Imaging. 2016 Jul 19. pii: S1936-878X(16)30406-5. doi: 10.1016/j.jcmg.2016.02.031. [Epub ahead of print] PMID:27450876

Mitochondrial long noncoding RNAs as blood based biomarkers for cardiac remodeling in patients with hypertrophic cardiomyopathy. Kitow J, Derda AA, Beermann J, Kumarswarmy R, Pfanne A, Fendrich J, Lorenzen JM, Xiao K, Bavendiek U, Bauersachs J, Thum T. Am J Physiol Heart Circ Physiol. 2016 Sep 1;311(3):H707-12. doi: 10.1152/ajpheart.00194.2016. PMID:27422984

Syncope in the young athlete: Assessment of prognosis in subjects with hypertrophic cardiomyopathy. Magalhães-Ribeiro C, Freitas J. Rev Port Cardiol. 2016 Jul-Aug;35(7-8):433-40. doi: 10.1016/j.repc.2016.04.007. English, Portuguese. PMID:27372256

Prevention of sudden death in hypertrophic cardiomyopathy: bridging the gaps in knowledge. Weissler-Snir A, Adler A, Williams L, Gruner C, Rakowski H. Eur Heart J. 2016 Jul 1. pii: ehw268. [Epub ahead of print] Review. PMID:27371714

Pregnancy in Hypertrophic Cardiomyopathy with Severe Left Ventricular Outflow Tract Obstruction. Shin J, Kim M, Lee J, Kim S, Kim M, Hwang H, Moon J, Shin MS, Chung WJ. J Cardiovasc Ultrasound. 2016 Jun;24(2):158-62. doi: 10.4250/jcu.2016.24.2.158. PMID:27358709 | PMCID:PMC4925394

Hypertrophic Cardiomyopathy: New Evidence Since the 2011 American Cardiology of Cardiology Foundation and American Heart Association Guideline. Fraiche A, Wang A. Curr Cardiol Rep. 2016 Jul;18(8):70. doi: 10.1007/s11886-016-0751-8. Review. PMID:27294414

Usefulness of Electrocardiographic Patterns at Presentation to Predict Long-term Risk of Cardiac Death in Patients With Hypertrophic Cardiomyopathy. Biagini E, Pazzi C, Olivotto I, Musumeci B, Limongelli G, Boriani G, Pacileo G, Mastromarino V, Bacchi Reggiani ML, Lorenzini M, Lai F, Berardini A, Mingardi F, Rosmini S, Resciniti E, Borghi C, Autore C, Cecchi F, Rapezzi C. Am J Cardiol. 2016 Aug 1;118(3):432-9. doi: 10.1016/j.amjcard.2016.05.023. PMID:27289293

Left ventricular mechanical dispersion is associated with nonsustained ventricular tachycardia in hypertrophic cardiomyopathy. Jalanko M, Tarkiainen M, Sipola P, Jääskeläinen P, Lauerma K, Laine M, Nieminen MS, Laakso M, Heliö T, Kuusisto J. Ann Med. 2016 Sep;48(6):417-427. PMID:27249556

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 44 Quarterly Review of Literature 3rd & 4th Quarter 2016

Clinical utility of natriuretic peptides and troponins in hypertrophic cardiomyopathy. Kehl DW, Buttan A, Siegel RJ, Rader F. Int J Cardiol. 2016 Sep 1;218:252-8. doi: 10.1016/j.ijcard.2016.05.031. Review. PMID:27236124

Validation of the 2014 European Society of Cardiology Sudden Cardiac Death Risk Prediction Model in Hypertrophic Cardiomyopathy in a Reference Center in South America. Fernández A, Quiroga A, Ochoa JP, Mysuta M, Casabé JH, Biagetti M, Guevara E, Favaloro LE, Fava AM, Galizio N. Am J Cardiol. 2016 Jul 1;118(1):121-6. doi: 10.1016/j.amjcard.2016.04.021. PMID:27189816

Appropriate Implantable Defibrillator Therapy in Adults With Hypertrophic Cardiomyopathy. Thavikulwat AC, Tomson TT, Knight BP, Bonow RO, Choudhury L. J Cardiovasc Electrophysiol. 2016 Aug;27(8):953-60. doi: 10.1111/jce.13005. PMID:27138377

Pharmacological treatment of hypertrophic cardiomyopathy: current practice and novel perspectives. Ammirati E, Contri R, Coppini R, Cecchi F, Frigerio M, Olivotto I. Eur J Heart Fail. 2016 Sep;18(9):1106-18. doi: 10.1002/ejhf.541. Review. PMID:27109894

Rapid Device-Detected Nonsustained Ventricular Tachycardia in the Risk Stratification of Hypertrophic Cardiomyopathy. Viswanathan K, Suszko AM, DAS M, Jackson N, Gollob M, Cameron D, Spears D, Woo A, Rakowski H, Khurana M, Chauhan VS. Pacing Clin Electrophysiol. 2016 Jul;39(7):642-51. doi: 10.1111/pace.12861. PMID:27027856

Serum Matrix Metalloproteinases as Quantitative Biomarkers for Myocardial Fibrosis and Sudden Cardiac Death Risk Stratification in Patients With Hypertrophic Cardiomyopathy. Münch J, Avanesov M, Bannas P, Säring D, Krämer E, Mearini G, Carrier L, Suling A, Lund G, Patten M. J Card Fail. 2016 Oct;22(10):845-50. doi: 10.1016/j.cardfail.2016.03.010. PMID:27018569

Effect of alcohol dosage on long-term outcomes after alcohol septal ablation in patients with hypertrophic cardiomyopathy. Liebregts M, Vriesendorp PA, Steggerda RC, Schinkel AF, Balt JC, Ten Cate FJ, Michels M, Ten Berg JM. Catheter Cardiovasc Interv. 2016 Nov 15;88(6):945-952. doi: 10.1002/ccd.26448. PMID:26946355

A meta analysis of current status of alcohol septal ablation and surgical myectomy for obstructive hypertrophic cardiomyopathy. Singh K, Qutub M, Carson K, Hibbert B, Glover C. Catheter Cardiovasc Interv. 2016 Jul;88(1):107-15. doi: 10.1002/ccd.26293. PMID:26526299

Impact of exercise-induced mitral regurgitation on hypertrophic cardiomyopathy outcomes. Feneon D, Schnell F, Galli E, Bernard A, Mabo P, Daubert JC, Leclercq C, Carre F, Donal E. Eur Heart J Cardiovasc Imaging. 2016 Oct;17(10):1110-7. doi: 10.1093/ehjci/jev242. PMID:26476399

The value of cardiac magnetic resonance and distribution of late gadolinium enhancement for risk stratification of sudden cardiac death in patients with hypertrophic cardiomyopathy. Klopotowski M, Kukula K, Malek LA, Spiewak M, Polanska-Skrzypczyk M, Jamiolkowski J, Dabrowski M, Baranowski R, Klisiewicz A, Kusmierczyk M, Jasinska A, Jarmus E, Kruk M, Ruzyllo W, Witkowski A, Chojnowska L. J Cardiol. 2016 Jul;68(1):49-56. doi: 10.1016/j.jjcc.2015.07.020. PMID:26363820

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 45 Quarterly Review of Literature 3rd & 4th Quarter 2016

The role of mutations in the SCN5A gene in cardiomyopathies. Zaklyazminskaya E, Dzemeshkevich S. Biochim Biophys Acta. 2016 Jul;1863(7 Pt B):1799-805. doi: 10.1016/j.bbamcr.2016.02.014. Review. PMID:26916278

Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/ARVC)

Truncating FLNC Mutations Are Associated With High-Risk Dilated and Arrhythmogenic Cardiomyopathies. Ortiz-Genga MF, Cuenca S, Dal Ferro M, Zorio E, Salgado-Aranda R, Climent V, Padrón-Barthe L, Duro-Aguado I, Jiménez-Jáimez J, Hidalgo-Olivares VM, García-Campo E, Lanzillo C, Suárez-Mier MP, Yonath H, Marcos-Alonso S, Ochoa JP, Santomé JL, García-Giustiniani D, Rodríguez-Garrido JL, Domínguez F, Merlo M, Palomino J, et al. J Am Coll Cardiol. 2016 Dec 6;68(22):2440-2451. doi: 10.1016/j.jacc.2016.09.927. PMID:27908349

Should all patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy undergo epicardial catheter ablation? Müssigbrodt A, Efimova E, Knopp H, Bertagnolli L, Dagres N, Richter S, Husser D, Bollmann A, Hindricks G, Arya A. J Interv Card Electrophysiol. 2016 Nov 30. [Epub ahead of print] PMID:27900527

A unique association of arrhythmogenic right ventricular dysplasia and acute , as assessed by cardiac MRI: a case report. Ponsiglione A, Puglia M, Morisco C, Barbuto L, Rapacciuolo A, Santoro M, Spinelli L, Trimarco B, Cuocolo A, Imbriaco M. BMC Cardiovasc Disord. 2016 Nov 21;16(1):230. PMID:27871237 | PMCID:PMC5117697

The Arrhythmogenic Calmodulin Mutation D129G Dysregulates Cell Growth, Calmodulin-dependent Kinase II Activity and Cardiac Function in Zebrafish. Berchtold MW, Zacharias T, Kulej K, Wang K, Torggler R, Jespersen T, Chen JN, Larsen MR, La Cour JM. J Biol Chem. 2016 Nov 4. pii: jbc.M116.758680. [Epub ahead of print] PMID:27815504

Arrhythmogenic Right Ventricular Dysplasia in Neuromuscular Disorders. Finsterer J, Stöllberger C. Clin Med Insights Cardiol. 2016 Oct 19;10:173-180. Review. PMID:27790050 | PMCID:PMC5072460

The Application of Ambulatory Electrocardiographically-Based T-Wave Alternans in Patients with Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy. Chung FP, Lin YJ, Chong E, Chang SL, Lo LW, Hu YF, Tuan TC, Chao TF, Liao JN, Chen SA. Can J Cardiol. 2016 Nov;32(11):1355.e15-1355.e22. doi: 10.1016/j.cjca.2016.01.035. PMID:27062236

Current Concepts on Diagnosis and Prognosis of Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia. Castaños Gutiérrez SL, Kamel IR, Zimmerman SL. J Thorac Imaging. 2016 Nov;31(6):324-335. PMID:26429589

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 46 Quarterly Review of Literature 3rd & 4th Quarter 2016

Arrhythmogenic Cardiomyopathy: Electrical and Structural Phenotypes. Akdis D, Brunckhorst C, Duru F, Saguner AM. Arrhythm Electrophysiol Rev. 2016 Aug;5(2):90-101. doi: 10.15420/AER.2016.4.3. PMID:27617087 | PMCID:PMC5013177

Utility of speckle tracking echocardiography imaging in patients with asymptomatic and symptomatic arrhythmogenic right ventricular cardiomyopathy. Alizade E, Yesin M, Tabakci MM, Avci A, Bulut M, Acar G, Şimşek Z, Izci S, Barutçu S, Pala S. Echocardiography. 2016 Nov;33(11):1683-1688. doi: 10.1111/echo.13338. PMID:27599902

Clinical characterisation and risk stratification of patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy ≥50 years of age. van der Pols MJ, Mast TP, Loh P, van der Heijden JF, Cramer MJ, Hauer RN, Te Riele AS. Neth Heart J. 2016 Dec;24(12):740-747. PMID:27580740 | PMCID:PMC5120007

Influence of Genotype on Structural Atrial Abnormalities and Atrial Fibrillation or Flutter in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy. Bourfiss M, Te Riele AS, Mast TP, Cramer MJ, VAN DER Heijden JF, VAN Veen TA, Loh P, Dooijes D, Hauer RN, Velthuis BK. J Cardiovasc Electrophysiol. 2016 Dec;27(12):1420-1428. doi: 10.1111/jce.13094. PMID:27572111

Atrioventricular Conduction Disorder as a First Manifestation of Arrhythmogenic Right Ventricular Dysplasia. Bruña V, Díez-Villanueva P, Martínez-Sellés M, Datino T, Fernández-Avilés F. Rev Esp Cardiol (Engl Ed). 2016 Dec;69(12):1222-1224. doi: 10.1016/j.rec.2016.05.028. English, Spanish. No abstract available. PMID:27514624

The Canadian Arrhythmogenic Right Ventricular Cardiomyopathy Registry: Rationale, Design, and Preliminary Recruitment. Krahn AD, Healey JS, Gerull B, Angaran P, Chakrabarti S, Sanatani S, Arbour L, Laksman ZW, Carroll SL, Seifer C, Green M, Roberts JD, Talajic M, Hamilton R, Gardner M. Can J Cardiol. 2016 Dec;32(12):1396-1401. doi: 10.1016/j.cjca.2016.04.004. PMID:27474350

Total pericardial agenesis mimicking arrhythmogenic right ventricular dysplasia. Pedrotti P, Peritore A, Cereda A, Giannattasio C, Imazio M. J Cardiovasc Med (Hagerstown). 2016 Jul 27. [Epub ahead of print] PMID:27467548

LMNA-Mediated Arrhythmogenic Right Ventricular Cardiomyopathy and Charcot-Marie-Tooth Type 2B1: A Patient- Discovered Unifying Diagnosis. Liang JJ, Grogan M, Ackerman MJ. J Cardiovasc Electrophysiol. 2016 Jul;27(7):868-71. doi: 10.1111/jce.12984. PMID:27405450

Arrhythmogenic right ventricular dysplasia/cardiomyopathy: arrhythmogenesis in the apparently normal heart? Hauer RN. Europace. 2016 Jul;18(7):953-4. doi: 10.1093/europace/euv316. No abstract available. PMID:27358359

Long-term right ventricular implantable cardioverter-defibrillator lead performance in arrhythmogenic right ventricular cardiomyopathy. Herman AR, Gardner M, Steinberg C, Yeung-Lai-Wah JA, Healey JS, Leong-Sit P, Krahn AD, Chakrabarti S. Heart Rhythm. 2016 Oct;13(10):1964-70. doi: 10.1016/j.hrthm.2016.06.021. PMID:27321245

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 47 Quarterly Review of Literature 3rd & 4th Quarter 2016

Minimally invasive epicardial implantable cardioverter-defibrillator placement for infants and children: An effective alternative to the transvenous approach. Schneider AE, Burkhart HM, Ackerman MJ, Dearani JA, Wackel P, Cannon BC. Heart Rhythm. 2016 Sep;13(9):1905-12. doi: 10.1016/j.hrthm.2016.06.024. PMID:27321244

Right Ventricular Imaging and Computer Simulation for Electromechanical Substrate Characterization in Arrhythmogenic Right Ventricular Cardiomyopathy. Mast TP, Teske AJ, Walmsley J, van der Heijden JF, van Es R, Prinzen FW, Delhaas T, van Veen TA, Loh P, Doevendans PA, Cramer MJ, Lumens J. J Am Coll Cardiol. 2016 Nov 15;68(20):2185-2197. doi: 10.1016/j.jacc.2016.08.061. PMID:27855808

Management of arrhythmogenic right ventricular cardiomyopathy. Silvano M, Mastella G, Zorzi A, Migliore F, Pilichou K, Bauce B, Rigato I, Perazzolo Marra M, Iliceto S, Thiene G, Basso C, Corrado D. Minerva Med. 2016 Aug;107(4):194-216. PMID:27186923

The ventricular ectopic QRS interval for diagnosis and risk stratification in arrhythmogenic right ventricular dysplasia/cardiomyopathy: Is this the answer? Calkins H. Heart Rhythm. 2016 Jul;13(7):1513-4. doi: 10.1016/j.hrthm.2016.04.004. No abstract available. PMID:27063103

The ventricular ectopic QRS interval (VEQSI): Diagnosis of arrhythmogenic right ventricular cardiomyopathy in patients with incomplete disease expression. Bastiaenen R, Pantazis A, Gonna H, Chis-Ster I, Castelletti S, Batchvarov VN, Domenichini G, Coccolo F, Boriani G, McKenna WJ, Behr ER, Gallagher MM. Heart Rhythm. 2016 Jul;13(7):1504-12. doi: 10.1016/j.hrthm.2016.03.039. PMID:27016477

Fibrofatty Changes: Incidence at Cardiac MR Imaging in Patients with Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy. Rastegar N, Te Riele AS, James CA, Bhonsale A, Murray B, Tichnell C, Calkins H, Tandri H, Bluemke DA, Kamel IR, Zimmerman SL. Radiology. 2016 Aug;280(2):405-12. doi: 10.1148/radiol.2016150988. PMID:26967143 | PMCID:PMC4976459

Novel electrocardiographic criteria for the diagnosis of arrhythmogenic right ventricular cardiomyopathy. Batchvarov VN, Bastiaenen R, Postema PG, Clark EN, Macfarlane PW, Wilde AA, Behr ER. Europace. 2016 Sep;18(9):1420-6. doi: 10.1093/europace/euv379. PMID:26622053

Arrhythmogenic right ventricular cardiomyopathy, clinical manifestations, and diagnosis. Haugaa KH, Haland TF, Leren IS, Saberniak J, Edvardsen T. Europace. 2016 Jul;18(7):965-72. doi: 10.1093/europace/euv340. Review. PMID:26498164

Phenotypic expression is a prerequisite for malignant arrhythmic events and sudden cardiac death in arrhythmogenic right ventricular cardiomyopathy. Zorzi A, Rigato I, Pilichou K, Perazzolo Marra M, Migliore F, Mazzotti E, Gregori D, Thiene G, Daliento L, Iliceto S, Rampazzo A, Basso C, Bauce B, Corrado D. Europace. 2016 Jul;18(7):1086-94. doi: 10.1093/europace/euv205. PMID:26138720

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 48 Quarterly Review of Literature 3rd & 4th Quarter 2016

Influence Of Novel Electrocardiographic Features Of Provocable Brugada ECG In Arrhythmogenic Cardiomyopathy And Its Exclusion By Lead AVR. Peters S. J Atr Fibrillation. 2016 Jun 30;9(1):1411. Review. PMID:27909514 | PMCID:PMC5089508 Timothy Syndrome (TS)

Genetic Syndromes, Maternal Diseases and Antenatal Factors Associated with Autism Spectrum Disorders (ASD). Ornoy A, Weinstein-Fudim L, Ergaz Z. Front Neurosci. 2016 Jul 6;10:316. doi: 10.3389/fnins.2016.00316. Review. PMID:27458336 | PMCID:PMC4933715

Novel long QT syndrome-associated missense mutation, L762F, in CACNA1C-encoded L-type calcium channel imparts a slower inactivation tau and increased sustained and window current. Landstrom AP, Boczek NJ, Ye D, Miyake CY, De la Uz CM, Allen HD, Ackerman MJ, Kim JJ. Int J Cardiol. 2016 Oct 1;220:290-8. doi: 10.1016/j.ijcard.2016.06.081. PMID:27390944

Incomplete Timothy syndrome secondary to a mosaic mutation of the CACNA1C gene diagnosed using next-generation sequencing. Baurand A, Falcon-Eicher S, Laurent G, Villain E, Bonnet C, Thauvin-Robinet C, Jacquot C, Eicher JC, Gourraud JB, Schmitt S, Bézieau S, Giraud M, Dumont S, Kuentz P, Probst V, Burguet A, Kyndt F, Faivre L. Am J Med Genet A. 2016 Nov 21. doi: 10.1002/ajmg.a.38045. [Epub ahead of print] PMID:27868338

A case of Timothy syndrome with adrenal medullary dystrophy. Kawaida M, Abe T, Nakanishi T, Miyahara Y, Yamagishi H, Sakamoto M, Yamada T. Pathol Int. 2016 Oct;66(10):587-592. doi: 10.1111/pin.12456. PMID:27593853 Short QT (SQT)

Ranolazine and Vernakalant Prevent Ventricular Arrhythmias in an Experimental Whole-Heart Model of Short QT Syndrome. Frommeyer G, Ellermann C, Dechering DG, Kochhäuser S, Bögeholz N, Güner F, Leitz P, Pott C, Eckardt L. J Cardiovasc Electrophysiol. 2016 Oct;27(10):1214-1219. doi: 10.1111/jce.13029. PMID:27283775

Bystander Cardio Pulmonary Resuscitation Saves Life in a Patient with Short QT Syndrome. Sharma PK, Awasthy N. Indian Pediatr. 2016 Oct 8;53(10):933-934. No abstract available. PMID:27771688 Considering Ranolazine as a Potential Treatment for K(+) Channel Linked Short QT Syndrome. Hancox JC. J Cardiovasc Electrophysiol. 2016 Oct;27(10):E5. doi: 10.1111/jce.13055. No abstract available. PMID:27477827

Considering Ranolazine as a Potential Treatment for K(+) Channel Linked Short QT Syndrome. Frommeyer G, Ellermann C, Eckardt L. J Cardiovasc Electrophysiol. 2016 Oct;27(10):E6. doi: 10.1111/jce.13054. No abstract available. PMID:27477620

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 49 Quarterly Review of Literature 3rd & 4th Quarter 2016

Epilepsy

The pathophysiology of cardiac dysfunction in epilepsy. Ravindran K, Powell KL, Todaro M, O'Brien TJ. Epilepsy Res. 2016 Nov;127:19-29. doi: 10.1016/j.eplepsyres.2016.08.007. Review. PMID:27544485

Seizures and Epilepsies due to Channelopathies and Neurotransmitter Receptor Dysfunction: A Parallel between Genetic and Immune Aspects. Lascano AM, Korff CM, Picard F. Mol Syndromol. 2016 Sep;7(4):197-209. Review. PMID:27781030 | PMCID:PMC5073503

Studying the pathophysiologic connection between cardiovascular and nervous systems using stem cells. Coskun V, Lombardo DM. J Neurosci Res. 2016 Dec;94(12):1499-1510. doi: 10.1002/jnr.23924. Review. PMID:27629698 Exercise

Characterizing the spectrum of right ventricular remodelling in response to chronic training. Sitges M, Merino B, Butakoff C, de la Garza MS, Paré C, Montserrat S, Vidal B, Azqueta M, Sarquella G, Gutierrez JA, Canal R, Brugada J, Bijnens BH. Int J Cardiovasc Imaging. 2016 Nov 15. [Epub ahead of print] PMID:27848162

Exercise and β-blocker therapy recommendations for inherited arrhythmogenic conditions. Christian S, Somerville M, Taylor S, Atallah J. Cardiol Young. 2016 Aug;26(6):1123-9. doi: 10.1017/S1047951115001894. PMID:26395163 Genetics & Genetic Testing

Enhancing Literacy in Cardiovascular Genetics: A Scientific Statement From the American Heart Association. Mital S, Musunuru K, Garg V, Russell MW, Lanfear DE, Gupta RM, Hickey KT, Ackerman MJ, Perez MV, Roden DM, Woo D, Fox CS, Ware S; American Heart Association Council on Functional Genomics and Translational Biology; Council on Cardiovascular Disease in the Young; Council on Cardiovascular and Nursing; Stroke Council; Council on Lifestyle and Cardiometabolic Health; and Council on Quality of Care and Outcomes Research.. Circ Cardiovasc Genet. 2016 Oct;9(5):448-467. PMID:27672144

Role of Genetic Testing in Patients with Ventricular Arrhythmias in Apparently Normal Hearts. Hofman N, Wilde AA. Card Electrophysiol Clin. 2016 Sep;8(3):515-23. doi: 10.1016/j.ccep.2016.04.002. Review. PMID:27521086

Clinical Genetic Testing for the Cardiomyopathies and Arrhythmias: A Systematic Framework for Establishing Clinical Validity and Addressing Genotypic and Phenotypic Heterogeneity. Garcia J, Tahiliani J, Johnson NM, Aguilar S, Beltran D, Daly A, Decker E, Haverfield E, Herrera B, Murillo L, Nykamp K, Topper S. Front Cardiovasc Med. 2016 Jun 27;3:20. doi: 10.3389/fcvm.2016.00020. PMID:27446933 | PMCID:PMC4921949

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 50 Quarterly Review of Literature 3rd & 4th Quarter 2016

Syncope

Canadian Cardiovascular Society and Canadian Pediatric Cardiology Association Position Statement on the Approach to Syncope in the Pediatric Patient. Sanatani S, Chau V, Fournier A, Dixon A, Blondin R, Sheldon RS. Can J Cardiol. 2016 Oct 3. pii: S0828-282X(16)30978-3. doi: 10.1016/j.cjca.2016.09.006. [Epub ahead of print] PMID:27838109 Miscellaneous

Keeping time in the chest. Triedman JK. Heart Rhythm. 2016 Nov 16. pii: S1547-5271(16)31007-4. doi: 10.1016/j.hrthm.2016.11.006. [Epub ahead of print] No abstract available. PMID:27865898

Cardiac Emergency Response Planning for Schools: A Policy Statement. Rose K, Martin Goble M, Berger S, Courson R, Fosse G, Gillary R, Halowich J, Indik JH, Konig M, Lopez-Anderson M, Murphy MK, Newman MM, Ranous J, Sasson C, Taras H, Thompson A. NASN Sch Nurse. 2016 Sep;31(5):263-70. doi: 10.1177/1942602X16655839. PMID:27486226

Clinical neurocardiology defining the value of neuroscience-based cardiovascular therapeutics. Shivkumar K, Ajijola OA, Anand I, Armour JA, Chen PS, Esler M, De Ferrari GM, Fishbein MC, Goldberger JJ, Harper RM, Joyner MJ, Khalsa SS, Kumar R, Lane R, Mahajan A, Po S, Schwartz PJ, Somers VK, Valderrabano M, Vaseghi M, Zipes DP. J Physiol. 2016 Jul 15;594(14):3911-54. doi: 10.1113/JP271870. PMID:27114333 | PMCID:PMC4945719

What Is the Arrhythmic Substrate in Viral Myocarditis? Insights from Clinical and Animal Studies. Tse G, Yeo JM, Chan YW, Lai ET, Yan BP. Front Physiol. 2016 Jul 21;7:308. doi: 10.3389/fphys.2016.00308. Review. PMID:27493633 | PMCID:PMC4954848

Pediatric & Congenital Electrophysiology Society: building an international paediatric electrophysiology organisation. Cohen M, Sanatani S, Stephenson E, Skinner J, Drago F, Davis A, Janousek J, Rosenthal E, Collins KK, Triedman J. Cardiol Young. 2016 Aug;26(6):1039-43. doi: 10.1017/S1047951115002553. PMID:27075202

The sympathetic innervation of the heart: Important new insights. Coote JH, Chauhan RA. Auton Neurosci. 2016 Aug;199:17-23. doi: 10.1016/j.autneu.2016.08.014. Review. PMID:27568995

SADS Quarterly Research Literature Review 2016 – Q3&4. Page 51