DOCSLIB.ORG

Outcome and Growth Potential of Left Heart Structures After Neonatal Intervention for Aortic Valve Stenosis

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Outcome and Growth Potential of Left Heart Structures After Neonatal Intervention for Aortic Valve Stenosis View metadata, citation and similar papers at core.ac.uk brought to you by CORE provided by Elsevier - Publisher Connector Journal of the American College of Cardiology Vol. 50, No. 25, 2007 © 2007 by the American College of Cardiology Foundation ISSN 0735-1097/07/$32.00 Published by Elsevier Inc. doi:10.1016/j.jacc.2007.07.082 Congenital Heart Disease Outcome and Growth Potential of Left Heart Structures After Neonatal Intervention for Aortic Valve Stenosis Ra K. Han, MD, FRCPC,* Rebecca C. Gurofsky, BSC,* Kyong-Jin Lee, MD, FRCPC,* Anne I. Dipchand, MD, FRCPC,* William G. Williams, MD, FRCSC,† Jeffrey F. Smallhorn, MD, FRCPC,* Brian W. McCrindle, MD, MPH, FRCPC* Toronto, Ontario, Canada Objectives The purpose of this study was to determine trends of growth of left heart structures after intervention for neona- tal aortic valve stenosis. Background The growth potential of left heart structures in neonatal aortic valve stenosis after relief of obstruction might influence risk for subsequent outcomes. Methods From 1994 to 2004, 53 patients underwent neonatal (Յ30 days old) balloon aortic valve dilation. Factors asso- ciated with time-related outcomes (death, reintervention, aortic valve replacement) and longitudinal changes in normalized left heart dimensions were sought. Results The median age at intervention was 3.5 days (range 1 to 30 days). During a median follow-up of 3.2 years rang- ing up to 10.9 years, there were 31 reinterventions on the aortic valve in 21 (40%) patients and 7 deaths (13%). The presence of moderate or severe left ventricular (LV) endocardial fibroelastosis was the only independent pre- dictor for time-related mortality (hazard ratio 22.1; p ϭ 0.004), and a smaller initial aortic valve annulus z-score was a significant independent predictor for aortic valve replacement (hazard ratio 0.63 per 1-U change; p ϭ 0.007). Aortic valve annulus, aortic sinus, and LV dimension z-scores significantly increased over time, whereas mitral valve z-scores remained below normal. The structure’s initial z-score and concomitant size of other left heart structures were significant independent factors associated with subsequent z-scores. Conclusions There is potential catch-up growth of the aortic valve and LV over time for neonates after intervention for aortic valve stenosis. However, the continued hypoplasia of the mitral valve warrants further consideration in the long- term management of these patients. (J Am Coll Cardiol 2007;50:2406–14) © 2007 by the American College of Cardiology Foundation Aortic valve stenosis in neonates is associated with varying deficits in functional status, and need for further interven- degrees of hypoplasia of left heart structures. Fetal echocar- tions. However, the pattern of growth in left heart structures diographic studies have demonstrated the progressive devel- after the relief of left-sided obstruction is unclear, even in opment of hypoplasia of left heart structures related to the absence of hypoplasia. A recent study demonstrated left-sided obstructive lesions (1,2). This hypoplasia adds to potential catch-up growth of the aortic valve and left increased morbidity and mortality for this patient popula- ventricle (LV) in those with smaller left heart structures (5). tion (3). In severe cases, the hypoplasia of left heart However, growth was not the focus of this study, the data structures associated with neonatal aortic valve stenosis provided were descriptive and limited to the aortic valve necessitates univentricular palliation or transplantation (4). annulus and left ventricular end-diastolic (LVED) dimen- In less severe cases, ongoing or progressive hypoplasia or sion, and multivariable longitudinal data analysis for trends growth failure might contribute to subsequent morbidity, and associated factors was not performed. Hence, we sought to determine the trends in longitudinal changes in left heart dimensions and aortic insufficiency and determine factors From the *Division of Cardiology, Department of Pediatrics, and the †Division of associated with clinical outcomes and growth of left heart Cardiovascular Surgery, Department of Surgery, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada. structures for patients with neonatal balloon aortic valve Manuscript received July 5, 2007; accepted July 25, 2007. dilation. JACC Vol. 50, No. 25, 2007 Han et al. 2407 December 18/25, 2007:2406–14 Left Heart Growth in Aortic Valve Stenosis Methods z-scores over time and demo- Abbreviations graphic characteristics, echocar- and Acronyms Patient population. Patients with a diagnosis of aortic diography measurements at pre- Congenital Heart ؍ valve stenosis who underwent neonatal aortic valve dilation CHSS sentation, and at follow-up and at Յ30 days of age at the Hospital for Sick Children in Surgeons Society the degree of aortic and mitral confidence interval ؍ Toronto, Ontario, Canada, from January 1994 to December CI valve stenosis and insufficiency left ؍ were identified from the cardiac catheterization data- LV 2004 were initially tested in a stepwise base. All patients had situs solitus, levocardia, concordant ventricle/ventricular regression model (p Ͻ 0.25 to left ventricular ؍ atrioventricular and ventriculoarterial connections, a left LVED enter); backward selection was aortic arch, and normal venous connections. Patients with end-diastolic used to obtain a final multivari- parameter estimates ؍ initial univentricular palliation were excluded. Patients un- PE able model. Results from these derwent initial percutaneous transcatheter balloon dilation models are given as parameter of the aortic valve with previously described techniques (6). estimates (PE) with standard error that correspond to the Patient demographic characteristics, clinical status before average increase in outcome measure for each increase of 1 intervention and at last follow-up, procedural characteristics measurement unit (or otherwise indicated) in predictor and hemodynamic status, and outcomes were abstracted measurement. All data analyses were performed with SAS from patient records. The study was approved by our statistical software (version 9.1, SAS Institute Inc., Cary, institutional Research Ethics Board, and patient confiden- North Carolina). tiality was maintained. Anatomic measurements. Initial pre-procedural and follow-up post-procedural echocardiograms and echocar- Results diographic reports were reviewed. Measurements of left Patient characteristics. Between January 1994 and De- heart structures were performed offline with electronic cember 2004, 53 patients underwent neonatal balloon aortic calipers and included the standard published (4) morpho- valve dilation. Fetal diagnosis of isolated aortic valve steno- logic and functional characteristics. Measurements were sis was made in 6 (11%) patients. The majority (81%) of normalized for body surface area as z-scores on the basis of patients were male. There were 2 (4%) pre-term neonates published normative data (7). Published normative data was born at 34 and 35 weeks’ gestational age. used rather than institution-specific normative values to The median age at presentation was 2 days (range 0 to 30 facilitate comparison with different institutions. Degree of days). Median weight was 3.4 kg (range 2.0 to 4.5 kg), aortic valve insufficiency was assessed both qualitatively with median length was 50 cm (range 44 to 57 cm), and median a global assessment based on aortic insufficiency jet width body surface area was 0.22 m2 (range 0.16 to 0.26 m2). ratio, LV dilation and function, and diastolic flow reversal in Although 26 (49%) patients presented with a fetal diagnosis the descending aorta and quantitatively with aortic insuffi- or an asymptomatic murmur, 27 (51%) presented with ciency jet width to aortic valve annulus ratio in diastole symptoms of low cardiac output or heart failure. Before the measured offline by a single reviewer. The initial pre- initial intervention, prostaglandin E1 infusion was initiated procedural echocardiographic measurements were used to in 33 (62%) patients, 26 (49%) were mechanically ventilated determine the Congenital Heart Surgeons Society (CHSS) for symptoms or for hospital transfer, and 15 (28%) received score with the published regression equation for critical inotropic medications. aortic stenosis (4) and the Rhodes score (3). Anatomic data. Initial anatomic features on echocardio- Data analysis. Data are presented as frequencies, means gram before the initial intervention are listed in Table 1. with standard deviations (SDs), or median with minimum Decreased LV systolic function (ejection fraction Ͻ55%) and maximum, as appropriate. Time-related survival, time was noted in 25 (47%) patients. Table 2 lists the z-scores of to reintervention, and time to aortic valve replacement were left heart structures at presentation. The initial mitral valve modeled with nonparametric Kaplan-Meier estimates. Fac- dimensions, LV dimension and length, aortic valve annulus, tors associated with these outcomes were sought from aortic root at the sinus, and sinotubular junction were demographic characteristics, medical status at presentation, generally smaller than normal, whereas the ascending aorta and echocardiography measurements with Cox proportion- was larger than normal. The median CHSS score was 0 ate hazard regression. Variables were initially included in a (range Ϫ46 to 55). Only 28 (53%) patients had a negative stepwise model (p Ͻ 0.05 to enter), and those selected were CHSS score, which predicts a survival benefit
Load more

Recommended publications
  • Cardiovascular Magnetic Resonance Pocket Guide
    Series Editors Bernhard A. Herzog John P. Greenwood Sven Plein Cardiovascular Magnetic Resonance Congenital Heart Disease Pocket Guide Bernhard A. Herzog Ananth Kidambi George Ballard First Edition 2014 Congenital Pocket Guide Tetralogy / Foreword Pulmonary Atresia Standard Views TGA Difficult Scans Single Ventricle Sequential Ebstein Anomaly Segmental Analysis Coronary Artery Shunts Anomalies AV Disease / References Aortic Coarctation Terminology Series Editors Authors Bernhard A. Herzog Bernhard A. Herzog John P. Greenwood Ananth Kidambi Sven Plein George Ballard Foreword The role of cardiovascular magnetic resonance (CMR) in evaluating the adult population with congenital heart disease continues to expand. This pocket guide aims to provide a day-to-day companion for those new to congenital CMR and for those looking for a quick reference guide in routine practice. The booklet gives an overview of the most common abnormalities and interventions as well as post-operative complications. It also provides typical scan protocols, key issues and a guide for reporting for each topic. Bernhard A. Herzog Ananth Kidambi George Ballard The Cardiovascular Magnetic Resonance Pocket Guide represents the views of the ESC Working Group on Cardiovascular Magnetic Resonance and was arrived at after careful consideration of the available evidence at the time it was written. Health professionals are encouraged to take it fully into account when exercising their clinical judgment. This pocket guide does not, however, override the individual responsibility of health professionals to make appropriate decisions in the circumstances of the individual patients, in consultation with that patient and, where appropriate and necessary, the patient's guardian or carer. It is also the health professional's responsibility to verify the applicable rules and regulations applicable to drugs and devices at the time of prescription.
    [Show full text]
  • Congenital Cardiac Surgery ICD9 to ICD10 Crosswalks Page 1 of 4 8
    Congenital Cardiac Surgery ICD9 to ICD10 Crosswalks ICD-9 code ICD-9 Descriptor ICD-10 Code ICD-10 Descriptor 164.1 Malignant neoplasm of heart C38.0 Malignant neoplasm of heart 164.1 Malignant neoplasm of heart C45.2 Mesothelioma of pericardium 212.7 Benign neoplasm of heart D15.1 Benign neoplasm of heart 425.11 Hypertrophic obstructive cardiomyopathy I42.1 Obstructive hypertrophic cardiomyopathy 425.18 Other hypertrophic cardiomyopathy I42.2 Other hypertrophic cardiomyopathy 425.3 Endocardial fibroelastosis I42.4 Endocardial fibroelastosis 425.4 Other primary cardiomyopathies I42.0 Dilated cardiomyopathy 425.4 Other primary cardiomyopathies I42.5 Other restrictive cardiomyopathy 425.4 Other primary cardiomyopathies I42.8 Other cardiomyopathies 425.4 Other primary cardiomyopathies I42.9 Cardiomyopathy, unspecified 426.9 Conduction disorder, unspecified I45.9 Conduction disorder, unspecified 745.0 Common truncus Q20.0 Common arterial trunk 745.10 Complete transposition of great vessels Q20.3 Discordant ventriculoarterial connection 745.11 Double outlet right ventricle Q20.1 Double outlet right ventricle 745.12 Corrected transposition of great vessels Q20.5 Discordant atrioventricular connection 745.19 Other transposition of great vessels Q20.2 Double outlet left ventricle 745.19 Other transposition of great vessels Q20.3 Discordant ventriculoarterial connection 745.19 Other transposition of great vessels Q20.8 Other congenital malformations of cardiac chambers and connections 745.2 Tetralogy of fallot Q21.3 Tetralogy of Fallot 745.3 Common
    [Show full text]
  • A Human Laterality Disorder Associated with a Homozygous WDR16 Deletion
    European Journal of Human Genetics (2015) 23, 1262–1265 & 2015 Macmillan Publishers Limited All rights reserved 1018-4813/15 www.nature.com/ejhg SHORT REPORT A human laterality disorder associated with a homozygous WDR16 deletion Asaf Ta-Shma*,1,3, Zeev Perles1,3, Barak Yaacov2, Marion Werner2, Ayala Frumkin2, Azaria JJT Rein1 and Orly Elpeleg2 The laterality in the embryo is determined by left-right asymmetric gene expression driven by the flow of extraembryonic fluid, which is maintained by the rotary movement of monocilia on the nodal cells. Defects manifest by abnormal formation and arrangement of visceral organs. The genetic etiology of defects not associated with primary ciliary dyskinesia is largely unknown. In this study, we investigated the cause of situs anomalies, including heterotaxy syndrome and situs inversus totalis, in a consanguineous family. Whole-exome analysis revealed a homozygous deleterious deletion in the WDR16 gene, which segregated with the phenotype. WDR16 protein was previously proposed to play a role in cilia-related signal transduction processes; the rat Wdr16 protein was shown to be confined to cilia-possessing tissues and severe hydrocephalus was observed in the wdr16 gene knockdown zebrafish. The phenotype associated with the homozygous deletion in our patients suggests a role for WDR16 in human laterality patterning. Exome analysis is a valuable tool for molecular investigation even in cases of large deletions. European Journal of Human Genetics (2015) 23, 1262–1265; doi:10.1038/ejhg.2014.265; published online 3 December 2014 INTRODUCTION and development, followed till 7 years, were normal. Her younger brother, Visceral asymmetry is determined through embryonic ciliary motion, patient II-4, was brought to medical attention at 7 weeks of age because of viral and normal function of the motile cilia plays a key role in the bronchiolitis.
    [Show full text]
  • Isolated Levocardia with Situs Inversus Without Cardiac Abnormality in Fetus: Prenatal Diagnosis and Management
    A L J O A T U N R I N R A E L P Case Report P L E R A Perinatal Journal 2020;28(1):48–51 I N N R A U T A L J O ©2020 Perinatal Medicine Foundation Isolated levocardia with situs inversus without cardiac abnormality in fetus: prenatal diagnosis and management Mucize Eriç ÖzdemirİD , Oya Demirci İD Department of Perinatology, Zeynep Kamil Maternity and Children’s Diseases Training and Research Hospital., Health Sciences University, Istanbul, Turkey Abstract Özet: Kardiyak anomalisi olmayan fetüste situs inversuslu izole levokardi: Prenatal tan› ve yönetim Objective: Isolated levocardia is a situs abnormality that the heart is Amaç: ‹zole levokardi, kalbin normal levo pozisyonunda oldu¤u in the normal levo position, but the abdominal viscera are in dextro fakat abdominal iç organlar›n dekstro pozisyonunda oldu¤u bir si- position. Most cases are accompanied by structural heart anomalies. tus anomalisidir. Ço¤u olguda yap›sal kalp anomalileri de efllik et- In this case, we aimed to present a fetus with isolated levocardia with- mektedir. Çal›flmam›zda, kardiyak anomalisi olmayan izole levo- out cardiac abnormality. kardili bir fetüsü sunmay› amaçlad›k. Case: The mother was referred to our clinic with a suspicion of fetal Olgu: Olgumuz, 22. gebelik haftas›nda fetal dekstrokardi flüphe- dextrocardia at 22 weeks of gestation. When detailed examination was siyle klini¤imize sevk edildi. Planlanan detayl› ultrason muayene- planned by ultrasonography isolated levocardia was detected in fetus. sinde, fetüste izole levokardi tespit edildi. Fetal ekokardiyografide There were no cardiac abnormalities in fetal echocardiography. Fetus hiçbir kardiyak anomali görülmedi.
    [Show full text]
  • Factors Impacting Echocardiographic Imaging After the Fontan Procedure: a Report from the Pediatric Heart Network Fontan Cross- Sectional Study
    Children's Mercy Kansas City SHARE @ Children's Mercy Manuscripts, Articles, Book Chapters and Other Papers 10-1-2013 Factors impacting echocardiographic imaging after the Fontan procedure: a report from the pediatric heart network fontan cross- sectional study. Richard V. Williams Renee Margossian Minmin Lu Andrew M. Atz Timothy J. Bradley See next page for additional authors Follow this and additional works at: https://scholarlyexchange.childrensmercy.org/papers Part of the Cardiology Commons, Cardiovascular System Commons, Congenital, Hereditary, and Neonatal Diseases and Abnormalities Commons, Pediatrics Commons, and the Surgical Procedures, Operative Commons Recommended Citation Williams, R. V., Margossian, R., Lu, M., Atz, A. M., Bradley, T. J., Campbell, M. J., Colan, S. D., Gallagher, D., Lai, W. W., Pearson, G. D., Prakash, A., Shirali, G. S., Cohen, M. S., . Factors impacting echocardiographic imaging after the Fontan procedure: a report from the pediatric heart network fontan cross-sectional study. Echocardiography (Mount Kisco, N.Y.) 30, 1098-1106 (2013). This Article is brought to you for free and open access by SHARE @ Children's Mercy. It has been accepted for inclusion in Manuscripts, Articles, Book Chapters and Other Papers by an authorized administrator of SHARE @ Children's Mercy. For more information, please contact [email protected]. Creator(s) Richard V. Williams, Renee Margossian, Minmin Lu, Andrew M. Atz, Timothy J. Bradley, Michael Jay Campbell, Steven D. Colan, Dianne Gallagher, Wyman W. Lai, Gail D. Pearson, Ashwin Prakash, Girish S. Shirali, Meryl S. Cohen, and Pediatric Heart Network Investigators This article is available at SHARE @ Children's Mercy: https://scholarlyexchange.childrensmercy.org/papers/906 NIH Public Access Author Manuscript Echocardiography.
    [Show full text]
  • Pub 100-04 Medicare Claims Processing Centers for Medicare & Medicaid Services (CMS) Transmittal 3054 Date: August 29, 2014 Change Request 8803
    Department of Health & CMS Manual System Human Services (DHHS) Pub 100-04 Medicare Claims Processing Centers for Medicare & Medicaid Services (CMS) Transmittal 3054 Date: August 29, 2014 Change Request 8803 SUBJECT: Ventricular Assist Devices for Bridge-to-Transplant and Destination Therapy I. SUMMARY OF CHANGES: This Change Request (CR) is effective for claims with dates of service on and after October 30, 2013; contractors shall pay claims for Ventricular Assist Devices as destination therapy using the criteria in Pub. 100-03, part 1, section 20.9.1, and Pub. 100-04, Chapter 32, sec. 320. EFFECTIVE DATE: October 30, 2013 *Unless otherwise specified, the effective date is the date of service. IMPLEMENTATION DATE: September 30, 2014 Disclaimer for manual changes only: The revision date and transmittal number apply only to red italicized material. Any other material was previously published and remains unchanged. However, if this revision contains a table of contents, you will receive the new/revised information only, and not the entire table of contents. II. CHANGES IN MANUAL INSTRUCTIONS: (N/A if manual is not updated) R=REVISED, N=NEW, D=DELETED-Only One Per Row. R/N/D CHAPTER / SECTION / SUBSECTION / TITLE D 3/90.2.1/Artifiical Hearts and Related Devices R 32/Table of Contents N 32/320/Artificial Hearts and Related Devices N 32/320.1/Coding Requirements for Furnished Before May 1, 2008 N 32/320.2/Coding Requirements for Furnished After May 1, 2008 N 32/320.3/ Ventricular Assist Devices N 32/320.3.1/Postcardiotomy N 32/320.3.2/Bridge-To -Transplantation (BTT) N 32/320.3.3/Destination Therapy (DT) N 32/320.3.4/ Other N 32/320.4/ Replacement Accessories and Supplies for External Ventricular Assist Devices or Any Ventricular Assist Device (VAD) III.
    [Show full text]
  • Heterotaxy and Complex Structural Heart Defects in a Mutant Mouse Model of Primary Ciliary Dyskinesia
    Heterotaxy and complex structural heart defects in a mutant mouse model of primary ciliary dyskinesia Serena Y. Tan, … , Linda Leatherbury, Cecilia W. Lo J Clin Invest. 2007;117(12):3742-3752. https://doi.org/10.1172/JCI33284. Research Article Development Primary ciliary dyskinesia (PCD) is a genetically heterogeneous disorder associated with ciliary defects and situs inversus totalis, the complete mirror image reversal of internal organ situs (positioning). A variable incidence of heterotaxy, or irregular organ situs, also has been reported in PCD patients, but it is not known whether this is elicited by the PCD- causing genetic lesion. We studied a mouse model of PCD with a recessive mutation in Dnahc5, a dynein gene commonly mutated in PCD. Analysis of homozygous mutant embryos from 18 litters yielded 25% with normal organ situs, 35% with situs inversus totalis, and 40% with heterotaxy. Embryos with heterotaxy had complex structural heart defects that included discordant atrioventricular and ventricular outflow situs and atrial/pulmonary isomerisms. Variable combinations of a distinct set of cardiovascular anomalies were observed, including superior-inferior ventricles, great artery alignment defects, and interrupted inferior vena cava with azygos continuation. The surprisingly high incidence of heterotaxy led us to evaluate the diagnosis of PCD. PCD was confirmed by EM, which revealed missing outer dynein arms in the respiratory cilia. Ciliary dyskinesia was observed by videomicroscopy. These findings show that Dnahc5 is required for the specification of left-right asymmetry and suggest that the PCD-causing Dnahc5 mutation may also be associated with heterotaxy. Find the latest version: https://jci.me/33284/pdf Research article Heterotaxy and complex structural heart defects in a mutant mouse model of primary ciliary dyskinesia Serena Y.
    [Show full text]
  • Atrial Septal Stenting to Increase Interatrial Shunting in Cyanotic Congenital Heart Diseases: a Report of Two Cases
    422 Türk Kardiyol Dern Arş - Arch Turk Soc Cardiol 2011;39(5):422-426 doi: 10.5543/tkda.2011.01368 Atrial septal stenting to increase interatrial shunting in cyanotic congenital heart diseases: a report of two cases Siyanotik doğuştan kalp hastalıklarında interatriyal şantı artırmak amacıyla atriyal septuma stent uygulaması: İki olgu sunumu Yalım Yalçın, M.D., Cenap Zeybek, M.D.,§ İbrahim Özgür Önsel, M.D.,# Mehmet Salih Bilal, M.D.† Departments of Pediatric Cardiology, #Anesthesiology and Reanimation, and †Cardiovascular Surgery, Medicana International Hospital; §Department of Pediatric Cardiology, Şişli Florence Nightingale Hospital, İstanbul Summary – Aiming to increase mixing at the atrial level, Özet – Siyanotik doğuştan kalp hastalığı tanısıyla izle- atrial septal stenting was performed in two pediatric nen iki bebekte, atriyal düzeyde karışımı artırmak ama- cases with cyanotic congenital cardiac diseases. The cıyla atriyal septuma stent yerleştirme işlemi uygulandı. first case was a 3-month-old male infant with transpo- Birinci olgu, büyük arterlerin transpozisyonu tanısıyla sition of the great arteries. The second case was an izlenen üç aylık bir erkek bebekti. Diğer olgu, ameliyat 18-month-old male infant with increased central venous sonrası dönemde sağ ventrikül çıkım yolu tıkanıklığına pressure due to postoperative right ventricular outflow bağlı olarak santral venöz basınç yüksekliği gelişen 18 tract obstruction. Premounted bare stents of 8 mm in aylık bir erkek bebekti. Her iki olguda da 8 mm çapında, diameter were used in both cases. The length of the balona monte edilmiş çıplak stent kullanıldı. Stent uzun- stent was 20 mm in the first case and 30 mm in the lat- luğu ilk olguda 20 mm, ikinci olguda 30 mm idi.
    [Show full text]
  • Situs Inversus Totalis - a Case Report
    IOSR Journal of Applied Physics (IOSR-JAP) e-ISSN: 2278-4861. Volume 3, Issue 6 (May. - Jun. 2013), PP 12-16 www.iosrjournals.org Situs Inversus Totalis - A Case Report *Dr. G.Supriya,** Dr. S.Saritha, **Dr. Seema Madan *Assistant professor, ** Professor of Anatomy, GMC, Secunderabad Abstract: SITUS INVERSUS TOTALIS is a congenital positional anomaly characterized by transposition of abdominal viscera associated with a right sided heart (Dextrocardia) .It was Mathew Baillie who first described situs inversus totalis in early twentieth century. A transposed thoracic and abdominal organ is a mirror image of the normal anatomy when examined or visualized by tests such as x-ray filming. The term situs inversus is a short form of the Latin meaning inverted position of the internal organs. Generally individuals with situs inversus totalis are asymptomatic and have a normal life expectancy. Many people with situs inversus totalis are unaware of their unusual anatomy until they seek medical attention for an unrelated condition. The reversal of the organs may lead to some confusion as many signs and symptoms will be on the reverse side. A 27 years old male patient reported to the Department of Nephrology with the c/o left sided flank pain since 1 week. The Chest X-ray, Ultrasonography, CT scan and MRI were done and he was diagnosed as SITUS INVERSUS TOTALIS. The anatomic, pathologic, embryologic and etiology of complete Situs inversus and related abnormalities are presented in this case with special emphasis to genetic correlation. Key words: Situs inversus totalis, Dextrocardia, Congenital, Transposition, Thoracic and abdominal organs. I.
    [Show full text]
  • Transcatheter Device Closure of Atrial Septal Defect in Dextrocardia with Situs Inversus Totalis
    Case Report Nepalese Heart Journal 2019; Vol 16(1), 51-53 Transcatheter device closure of atrial septal defect in dextrocardia with situs inversus totalis Kiran Prasad Acharya1, Chandra Mani Adhikari1, Aarjan Khanal2, Sachin Dhungel1, Amrit Bogati1, Manish Shrestha3, Deewakar Sharma1 1 Department of Cardiology, Shahid Gangalal National Heart Centre, Kathmandu, Nepal 2 Department of Internal Medicine, Kathmandu Medical College, Kathmandu,Nepal 3 Department of Pediatric Cardiology, Shahid Gangalal National Heart Centre, Kathmandu, Nepal Corresponding Author: Chandra Mani Adhikari Department of Cardiology Shahid Gangalal National Heart Centre Kathmandu, Nepal Email: [email protected] Cite this article as: Acharya K P, Adhikari C M, Khanal A, et al. Transcatheter device closure of atrial septal defect in dextrocardia with situs inversus totalis. Nepalese Heart Journal 2019; Vol 16(1), 51-53 Received date: 17th February 2019 Accepted date: 16th April 2019 Abstract Only few cases of Device closure of atrial septal defect in dextrocardia with situs inversus totalis has been reported previously. We present a case of a 36 years old male, who had secundum type of atrial septal defect in dextrocardia with situs inversus totalis. ASD device closure was successfully done. However, we encountered few technical difficulties in this case which are discussed in this case review. Keywords: atrial septal defect; dextrocardia; transcatheter device closure, DOI: https://doi.org/10.3126/njh.v16i1.23901 Introduction There are only two case reported of closure of secundum An atrial septal defect (ASD) is an opening in the atrial ASD associated in patients with dextrocardia and situs inversus septum, excluding a patent foramen ovale.1 ASD is a common totalis.
    [Show full text]
  • Norepinephrine Induced Pulmonary Congestion in Patients with Aortic Valve Regurgitation
    NOREPINEPHRINE INDUCED PULMONARY CONGESTION IN PATIENTS WITH AORTIC VALVE REGURGITATION Timothy J. Regan, … , Kenan Binak, Harper K. Hellems J Clin Invest. 1959;38(9):1564-1571. https://doi.org/10.1172/JCI103935. Research Article Find the latest version: https://jci.me/103935/pdf NOREPINEPHRINE INDUCED PULMONARY CONGESTION IN PATIENTS WITH AORTIC VALVE REGURGITATION * By TIMOTHY J. REGAN, VALENTINO DEFAZIO, KENAN BINAKt AND HARPER K. HELLEMS (From the Cardiovascular Research Laboratories, Departments of Medicine, Wayne State Uni- versity College of Medicine, and City of Detroit Receiving Hospital, Detroit, Mich.) (Submitted for publication March 3, 1959; accepted May 14, 1959) The regulation of venous blood flow into the METHODS heart and the effects of blood volume redistribution All subjects were studied in the fasting state approxi- upon cardiac function have not been amply defined. mately two hours after receiving 0.1 Gm. of pentobarbital While enhanced venomotor tone induced by nor- sodium. A double lumen catheter was used to measure epinephrine has been found to increase the volume pressures simultaneously from the pulmonary "capillary" and pulmonary artery. The "capillary" position was as- of a central reservoir in the dog (1), such a vol- sumed to be present when the catheter could not be ad- ume shift has not been demonstrated in man, ex- vanced farther in the lung bed, the tip did not move with cept inferentially by the finding of a decrease in the cardiac cycle and a compatible pressure pattern was the limb venous volume (2). obtained. A specimen of blood saturated with oxygen This type of alteration might be expected to af- was secured from the wedged catheter tip whenever this particularly was possible.
    [Show full text]
  • Scoliosis Convexity and Organ Anatomy Are Related
    Eur Spine J DOI 10.1007/s00586-017-4970-5 ORIGINAL ARTICLE Scoliosis convexity and organ anatomy are related 1 2 3 2 Tom P. C. Schlo¨sser • Tom Semple • Siobha´n B. Carr • Simon Padley • 4 1,2,3,4 1 Michael R. Loebinger • Claire Hogg • Rene´ M. Castelein Received: 24 August 2016 / Revised: 1 January 2017 / Accepted: 22 January 2017 Ó The Author(s) 2017. This article is published with open access at Springerlink.com Abstract Methods Chest radiographs of PCD patients were sys- Purpose Primary ciliary dyskinesia (PCD) is a respiratory tematically screened for existence of significant lateral syndrome in which ‘random’ organ orientation can occur; spinal deviation using the Cobb angle. Positive values with approximately 46% of patients developing situs represented right-sided convexity. Curve convexity and inversus totalis at organogenesis. The aim of this study was Cobb angles were compared between PCD patients with to explore the relationship between organ anatomy and situs inversus and normal anatomy. curve convexity by studying the prevalence and convexity Results A total of 198 PCD patients were screened. The of idiopathic scoliosis in PCD patients with and without prevalence of scoliosis (Cobb [10°) and significant spinal situs inversus. asymmetry (Cobb 5–10°) was 8 and 23%, respectively. Curve convexity and Cobb angle were significantly dif- ferent within both groups between situs inversus patients & Rene´ M. Castelein and patients with normal anatomy (P B 0.009). Moreover, [email protected] curve convexity correlated significantly with organ orien- Tom P. C. Schlo¨sser tation (P \ 0.001; / = 0.882): In 16 PCD patients with [email protected] scoliosis (8 situs inversus and 8 normal anatomy), except Tom Semple for one case, matching of curve convexity and orientation [email protected] of organ anatomy was observed: convexity of the curve Siobha´n B.
    [Show full text]