DOCSLIB.ORG

Aortic Stenosis and Mitral Regurgitation Guidelines Overview

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Aortic Stenosis and Mitral Regurgitation Guidelines Overview Aortic Stenosis and Mitral Regurgitation Guidelines Overview Rick A. Nishimura, MD, MACC, FAHA, Co-Chair† Catherine M. Otto, MD, FACC, FAHA, Co-Chair† Robert O. Bonow, MD, MACC, FAHA† Carlos E. Ruiz, MD, PhD, FACC† Blase A. Carabello, MD, FACC*† Nikolaos J. Skubas, MD, FASE¶ John P. Erwin III, MD, FACC, FAHA‡ Paul Sorajja, MD, FACC, FAHA# Robert A. Guyton, MD, FACC*§ Thoralf M. Sundt III, MD* **†† Patrick T. O’Gara, MD, FACC, FAHA† James D. Thomas, MD, FASE, FACC, FAHA‡‡ 2014 ACC/AHA Valve Guidelines Core Concepts • Valve disease stages • Improved imaging and severity quantitation • Timing of intervention aligned with disease stages • Earlier intervention with trans-catheter options • Valve Disease Centers and Heart Valve Teams • Integrative approach to procedural risk assessment 2014 ACC/AHA Valvular Heart Disease (VHD) Guidelines Definitions of Disease Severity • Patient Symptoms due to valve dysfunction • Valve Leaflet anatomy and pathology • Flow Valve hemodynamics • Ventricle Hypertrophy, dilation, dysfunction AHA/ACC Valve Guidelines Valve Disease Stages Otto and Prendergast. NEJM 2014 2014 ACC/AHA Valve Guidelines Concept of Valve Disease Stages Stage Definition Description A At risk Patients with risk factors for the development of VHD B Progressive Patients with progressive VHD (mild-to-moderate severity and asymptomatic) C Asymptomatic Asymptomatic patients who have reached the criteria severe for severe VHD C1: Asymptomatic patients with severe VHD in whom the left or right ventricle remains compensated C2: Asymptomatic patients who have severe VHD, with decompensation of the left or right ventricle D Symptomatic Patients who have developed symptoms as a result of severe VHD Why Measure Aortic Stenosis Severity ? Ensure AS is the cause of symptoms AS? Aortic Stenosis Severity Is AS severe enough to be the cause of symptoms? Vmax 4.0 cm2 1 m/s Symptoms-- 2 m/s Not due to AS 2.0 cm2 3 m/s Symptoms-- Maybe due to AS? 4 m/s 1.0 cm2 Symptoms -- 5 m/s Likely due to AS 0.5 cm2 High sensitivity valued over high specificity AVA Aortic Stenosis Severity Optimal definition of severe stenosis ? Jet velocity Vmax Pressure gradient P = 4v2 The reference standard that Valvedefines “ Areasevere” aortic stenosis Continuityis prediction of clinical equation outcome. 2014 ACC/AHA Valvular Heart Disease (VHD) Guidelines Definitions of Disease Severity 2014 ACC/AHA Valvular Heart Disease (VHD) Guidelines Definitions of Disease Severity Multivariate predictors of symptom onset (normal flow): • Aortic velocity • Not AVA 2014 ACC/AHA Valvular Heart Disease (VHD) Guidelines Aortic Stenosis Disease Stages Stage Definition Valve anatomy and hemodynamics A At risk for AS Bicuspid valve, aortic sclerosis B Progressive AS Mild AS Vmax 2.0–2.9 m/s, Mean ΔP < 20 mm Hg Mod AS Vmax 3.0–3.9 m/s, Mean ΔP 20-39 mm Hg 2 (Typically AVA >1.0 cm ) C Asymptomatic Severe AS Vmax ≥ 4.0 m/s, Mean ΔP ≥40 mm Hg severe AS (Typically AVA ≤1.0 cm2) Very severe AS Vmax ≥ 5.0 m/s, Mean ΔP ≥60 mm Hg C1: Normal LV systolic C2: LV ejection fraction < 50% D Symptomatic D1: High gradient severe AS severe AS D2: Low gradient severe AS (low EF) D3: Low-flow low-gradient severe AS (normal EF) 2014 ACC/AHA Valvular Heart Disease (VHD) Guidelines Symptomatic Severe Aortic Stenosis ECHO Vmax ≥ 4 m/s Vmax < 4 m/s AVA ≤ 1 cm2 EF < 50% D1 High Gradient 2014 ACC/AHA Valvular Heart Disease (VHD) Guidelines Symptomatic Severe Aortic Stenosis ECHO Vmax ≥ 4 m/s Vmax < 4 m/s Vmax < 4 m/s AVA ≤ 1 cm2 AVA ≤ 1 cm2 EF < 50% EF ≥ 50% Low dose DSE Calcified valve Vmax ≥ 4 m/s with Normotensive AVA ≤ 1 cm2 AVAi ≤ 0.6 cm2/m2 at any flow rate SVi < 35 ml/m2 D1 High Gradient D2 LFLG Low EF D3 LFLG Normal EF 2014 ACC/AHA Valve Guidelines TIMING of Intervention for AS Aortic valve stenosis Symptoms due to AS No AS symptoms V ≥ 4 m/s V < 4 m/s max max Vmax ≥ 5 m/s + V ≥ 4 m/s Low surgical risk max EF < 50% EF < 50% YES NO ETT with BP or 2 2 ex. capacity DSE Vmax ≥ 4 m/s AVAi 0.6 cm /m at any flow rate and SVI <35 mL/m2 Rapid disease progression + low surgical risk AVR (I) AVR (I) AVR (IIa) AVR (IIa) AVR (IIa) AVR (IIa) AVR (IIb) 2014 ACC/AHA Valve Guidelines INTERVENTION FOR AORTIC STENOSIS TIMING of Intervention CHOICE of Intervention Valve Type Surgical vs Transcatheter 2014 ACC/AHA Valve Guidelines CHOICE of Intervention for AS Indication for AVR Heart Valve Team (I) Low-intermediate High Prohibitive surgical risk surgical risk surgical risk Bridge to SAVR or TAVR for severe Predicted post-TAVR symptoms survival > 1 yr BAV (IIb) YES NO Surgical TAVR TAVR (I) Palliative AVR (I) (IIa) Care Conceptual Framework Management of Aortic Stenosis SAVR TAVR BENEFIT RISK Years of life Procedural risk Quality of life Late complications Functional status Pain/discomfort Age 50 man 90 woman Comorbidities Otherwise Frail healthy Multiple comorbidities 2014 ACC/AHA Valve Guidelines Causes of Chronic Mitral regurgitation • Primary mitral valve disease • Myxomatous (MVP) • Rheumatic • Secondary (functional) regurgitation • Ischemic • Dilated cardiomyopathy Otto CM Textbook of Clinical Echocardiography, 5th Edition, 2013. 2014 ACC/AHA Valve Guidelines Stages of Chronic Primary Mitral Regurgitation Stage Anatomy Hemodynamics Left ventricle A At risk MVP No to trace MR Normal (asymptomatic) B Progressive Severe MVP Mild to moderate Normal LV volumes (asymptomatic) Rheumatic MR: Normal LV EF Vena contracta < 0.7 cm Mild LA, Normal PAP Endocarditis ERO < 0.4 cm2, RV< 60 ml, Angio 1- 2+ C Asymptomatic Severe MVP Severe MR C1: LV EF > 60% with LV +/- flail Vena contracta ≥ 0.7 cm ESD < 40 mm Severe MR ERO ≥ 0.4 cm2, RV ≥ 60 Severe ml, Angio 3-4+ C2: LV EF ≤60% or LV ESD rheumatic ≥40 mm D Symptomatic Endocarditis Severe MR LV dilation Severe MR Radiation Vena contracta ≥ 0.7 cm Pulmonary hypertension ERO ≥ 0.4 cm2, RV ≥ 60 Moderate to severe LA ml, Angio 3-4+ 2014 ACC/AHA Valve Guidelines Valve hemodynamics with severe primary MR • Central jet MR >40% LA or holosystolic eccentric jet MR • Vena contracta ≥0.7 cm • Regurgitant volume ≥60 mL • Regurgitant fraction ≥50% • ERO ≥0.40 cm2 • Angiographic grade 3–4+ 2014 ACC/AHA Valve Guidelines LV Response to Chronic Volume Overload LV Size and Systolic Function • Echo • MRI 2014 ACC/AHA Valve Guidelines: Indications for Surgery Primary Mitral Regurgitation Severe MR Progressive MR (Stage C or D) (Stage B) Symptomatic Asymptomatic (Stage D) (Stage C) LVEF 30-60% or LVEF > 60% or New onset AF or LVEF > 30% LVESD ≥ 40 mm LVESD < 40 mm PASP > 50 mmHg (Stage C2) (Stage C1) (Stage C1) NO YES MV Surgery MV Surgery Periodic (IIb) (I) Monitoring 3D Anatomy Mitral Valve LA side LV side 3D Echo: Leaflet anatomy Prolapse Chordal rupture Amenable to: Valve repair? Systole Transcatheter procedure? Otto, Diastole Textbook of Clinical Echocardiography, 5th Ed. 2013 2014 ACC/AHA Valve Guidelines: Indications for Surgery Primary Mitral Regurgitation Severe MR Progressive MR (Stage C or D) (Stage B) Symptomatic Asymptomatic (Stage D) (Stage C) LVEF 30-60% or LVEF > 60% or New onset AF or LVEF > 30% LVESD ≥ 40 mm LVESD < 40 mm PASP > 50 mmHg (Stage C2) (Stage C1) (Stage C1) Likelihood of successful repair >95% and Expected mortality < 1% NO YES YES NO MV Surgery MV Surgery MV Repair Periodic (IIb) (I) (IIa) Monitoring Secondary Mitral Regurgitation Mechanisms and outcomes Coronary Disease • Acute ischemia • Regional LV dysfunction • Global LV dilation and dysfunction Cardiomyopathy (heart failure) • LV dilation and dysfunction • Leaflet tethering Liel-Cohen N, Guerrero JL, Otsuji Y, et al: Circulation 101[23]:2756-2763, 2000. 2014 ACC/AHA Valve Guidelines Stages of Chronic Secondary MR Stage Anatomy Hemodynamics/LV Symptoms A At risk CAD or No to trace MR--- Due to coronary (asymptomatic) Cardiomyopathy All have primary ischemia or heart failure myocardial disease B Progressive Regional LV Mild to moderate Symptoms may respond (asymptomatic) dysfx. MR: to Rx for coronary Annular dilation ERO < 0.2 cm2 ischemia or HF RV< 30 ml RF < 50% C Asymptomatic Regional or Severe MR Symptoms may respond global LV ERO ≥ 0.2 cm2 Severe MR to Rx for coronary dilation and RV ≥ 50 ml ischemia or HF dysfx. RF ≥ 50% Leaflet tethering D Symptomatic Annular dilation Severe MR HF symptoms persist 2 Severe MR ERO ≥ 0.2 cm after revascularization RV ≥ 50 ml and medical therapy RF ≥ 50% 2014 ACC/AHA Valve Guidelines Indications for Surgery Secondary Mitral Regurgitation CAD Rx HF Rx Consider CRT Symptomatic Asymptomatic Progressive Severe MR Severe MR MR (Stage D) (Stage C) (Stage B) MV Surgery Periodic Monitoring (IIb) ACC/AHA Valve Guidelines Balance between waiting and intervention Watchful Waiting Intervention Hemodynamic severity Mortality Valve anatomy Complications Progression rate Valve hemodynamics Age, comorbidities Valve durability Pt. preferences Thrombotic risk ACC/AHA Valve Guidelines Balance between waiting and intervention ACC/AHA Valve Guidelines Balance between waiting and intervention Challenges in Assessment and Management Aortic Stenosis • Diagnosis of low gradient severe AS • Intervention for asymptomatic “severe” AS • Choice of surgical vs. trans-catheter AVR • Benefit-risk balance in older adults with multiple comorbidities and frailty Challenges in Assessment and Management Mitral Regurgitation • Optimal (outcome based) definitions of MR severity • Centers of excellence for management of asymptomatic severe MR • Role of trans-catheter vs surgical approaches • Management of secondary MR .
Load more

Recommended publications
  • Myocarditis and Cardiomyopathy
    CE: Tripti; HCO/330310; Total nos of Pages: 6; HCO 330310 REVIEW CURRENT OPINION Myocarditis and cardiomyopathy Jonathan Buggey and Chantal A. ElAmm Purpose of review The aim of this study is to summarize the literature describing the pathogenesis, diagnosis and management of cardiomyopathy related to myocarditis. Recent findings Myocarditis has a variety of causes and a heterogeneous clinical presentation with potentially life- threatening complications. About one-third of patients will develop a dilated cardiomyopathy and the pathogenesis is a multiphase, mutlicompartment process that involves immune activation, including innate immune system triggered proinflammatory cytokines and autoantibodies. In recent years, diagnosis has been aided by advancements in cardiac MRI, and in particular T1 and T2 mapping sequences. In certain clinical situations, endomyocardial biopsy (EMB) should be performed, with consideration of left ventricular sampling, for an accurate diagnosis that may aid treatment and prognostication. Summary Although overall myocarditis accounts for a minority of cardiomyopathy and heart failure presentations, the clinical presentation is variable and the pathophysiology of myocardial damage is unique. Cardiac MRI has significantly improved diagnostic abilities, but endomyocardial biopsy remains the gold standard. However, current treatment strategies are still focused on routine heart failure pharmacotherapies and supportive care or cardiac transplantation/mechanical support for those with end-stage heart failure. Keywords cardiac MRI, cardiomyopathy, endomyocardial biopsy, myocarditis INTRODUCTION prevalence seen in children and young adults aged Myocarditis refers to inflammation of the myocar- 20–30 years [1]. dium and may be caused by infectious agents, systemic diseases, drugs and toxins, with viral infec- CAUSE tions remaining the most common cause in the developed countries [1].
    [Show full text]
  • ICD-10-CM Documentation and Coding Best Practices
    ICD-10-CM Documentation and Coding Best Practices Cardiomyopathy Cardiomyopathy refers to diseases of the heart muscle, which can become enlarged, thick or rigid. In rare cases, cardiac muscle tissue can be replaced with scar tissue. As the condition worsens, the heart becomes weaker and less able to pump blood through the body or to maintain a normal electrical rhythm. Causes Risk factors that can increase the possibility of developing cardiomyopathy include: coronary artery disease, a history of heart attack (s), viral infections that cause heart inflammation, long-term hypertension or alcoholism, obesity, and diabetes to name a few. However, in most cases the exact cause is usually unknown (called primary or idiopathic cardiomyopathy). Symptoms Some patients will never have symptoms; others will not develop them until later in the disease. Symptoms can include: • Fatigue • Shortness of b reath or trouble breathing (dyspnea) • Dizziness, lightheadedness or fainting • Swelling in the ankles, feet, legs, abdomen and neck veins Treatment Treatment depends on the type of cardiomyopathy, the severity of symptoms, and the patient’s age and overall health. • Lifestyle changes can help manage condition(s) that may be causing cardiomyopathy. Recommendations include: o Consuming a heart healthy diet, engaging in physical activity, losing excess weight, giving up smoking, avoiding alcohol and illegal drugs, getting enough sleep, and reducing stress • Medicines may be p rescribed to: o Lower blood pressure (ACE inhibitors, angiotensin II receptor blo ckers, beta blockers, calcium channel blockers ) o Slow the heart rate (beta blockers, calcium channel blockers, digoxin) o Prevent arrhythmias (antiarrhythmics) o Remove excess fluid and sodium (diuretics) o Prevent blood clots (anticoagulants) • Alcohol septal ablation • Surgery o Septal myectomy – option for severe cases of obstructive hypertrophic cardiomyopathy Surgically implanted devices o .
    [Show full text]
  • Antithrombotic Therapy in Atrial Fibrillation Associated with Valvular Heart Disease
    Europace (2017) 0, 1–21 EHRA CONSENSUS DOCUMENT doi:10.1093/europace/eux240 Antithrombotic therapy in atrial fibrillation associated with valvular heart disease: a joint consensus document from the European Heart Rhythm Association (EHRA) and European Society of Cardiology Working Group on Thrombosis, endorsed by the ESC Working Group on Valvular Heart Disease, Cardiac Arrhythmia Society of Southern Africa (CASSA), Heart Rhythm Society (HRS), Asia Pacific Heart Rhythm Society (APHRS), South African Heart (SA Heart) Association and Sociedad Latinoamericana de Estimulacion Cardıaca y Electrofisiologıa (SOLEACE) Gregory Y. H. Lip1*, Jean Philippe Collet2, Raffaele de Caterina3, Laurent Fauchier4, Deirdre A. Lane5, Torben B. Larsen6, Francisco Marin7, Joao Morais8, Calambur Narasimhan9, Brian Olshansky10, Luc Pierard11, Tatjana Potpara12, Nizal Sarrafzadegan13, Karen Sliwa14, Gonzalo Varela15, Gemma Vilahur16, Thomas Weiss17, Giuseppe Boriani18 and Bianca Rocca19 Document Reviewers: Bulent Gorenek20 (Reviewer Coordinator), Irina Savelieva21, Christian Sticherling22, Gulmira Kudaiberdieva23, Tze-Fan Chao24, Francesco Violi25, Mohan Nair26, Leandro Zimerman27, Jonathan Piccini28, Robert Storey29, Sigrun Halvorsen30, Diana Gorog31, Andrea Rubboli32, Ashley Chin33 and Robert Scott-Millar34 * Corresponding author. Tel/fax: þ44 121 5075503. E-mail address: [email protected] Published on behalf of the European Society of Cardiology. All rights reserved. VC The Author 2017. For permissions, please email: [email protected]. 2 G.Y.H. Lip 1Institute of Cardiovascular Sciences, University of Birmingham and Aalborg Thrombosis Research Unit, Department of Clinical Medicine, Aalborg University, Denmark (Chair, representing EHRA); 2Sorbonne Universite´ Paris 6, ACTION Study Group, Institut De Cardiologie, Groupe Hoˆpital Pitie´-Salpetrie`re (APHP), INSERM UMRS 1166, Paris, France; 3Institute of Cardiology, ‘G.
    [Show full text]
  • The Pulmonary Manifestations of Left Heart Failure*
    The Pulmonary Manifestations of Left Heart Failure* Brian K. Gehlbach, MD; and Eugene Geppert, MD Determining whether a patient’s symptoms are the result of heart or lung disease requires an understanding of the influence of pulmonary venous hypertension on lung function. Herein, we describe the effects of acute and chronic elevations of pulmonary venous pressure on the mechanical and gas-exchanging properties of the lung. The mechanisms responsible for various symptoms of congestive heart failure are described, and the significance of sleep-disordered breathing in patients with heart disease is considered. While the initial clinical evaluation of patients with dyspnea is imprecise, measurement of B-type natriuretic peptide levels may prove useful in this setting. (CHEST 2004; 125:669–682) Key words: Cheyne-Stokes respiration; congestive heart failure; differential diagnosis; dyspnea; pulmonary edema; respiratory function tests; sleep apnea syndromes Abbreviations: CHF ϭ congestive heart failure; CSR-CSA ϭ Cheyne-Stokes respiration with central sleep apnea; CPAP ϭ continuous positive airway pressure; Dlco ϭ diffusing capacity of the lung for carbon monoxide; DM ϭ membrane conductance; FRC ϭ functional residual capacity; OSA ϭ obstructive sleep apnea; TLC ϭ total lung ϭ ˙ ˙ ϭ capacity; VC capillary volume; Ve/Vco2 ventilatory equivalent for carbon dioxide early 5 million Americans have congestive heart For a detailed review of the pathophysiology of N failure (CHF), with 400,000 new cases diag- high-pressure pulmonary edema, the reader is re- nosed each year.1 Unfortunately, despite the consid- ferred to several excellent recent reviews.2–4 erable progress that has been made in understanding the pathophysiology of pulmonary edema, the pul- monary complications of this condition continue to The Pathophysiology of Pulmonary challenge the bedside clinician.
    [Show full text]
  • Percutaneous Mitral Valve Therapies: State of the Art in 2020 LA ACP Annual Meeting
    Percutaneous Mitral Valve Therapies: State of the Art in 2020 LA ACP Annual Meeting Steven R Bailey MD MSCAI, FACC, FAHA,FACP Professor and Chair, Department of Medicine Malcolm Feist Chair of Interventional Cardiology LSU Health Shreveport Professor Emeritus, UH Health San Antonio [email protected] SRB March 2020 Disclosure Statement of Financial Interest Within the past 12 months, I or my spouse/partner have had a financial interest/arrangement or affiliation with the organization(s) listed below. Affiliation/Financial Relationship Company • Grant/Research Support • None • Consulting Fees/Honoraria • BSCI, Abbot DSMB • Intellectual Property Rights • UTHSCSA • Other Financial Benefit • CCI Editor In Chief SRB March 2020 The 30,000 Ft View Maria SRB March 2020 SRB March 2020 Mitral Stenosis • The most common etiology of MS is rheumatic fever, with a latency of approximately 10 to 20 years after the initial streptococcal infection. Symptoms usually appear in adulthood • Other etiologies are rare but include: congenital MS radiation exposure atrial myxoma mucopolysaccharidoses • MS secondary to calcific annular disease is increasingly seen in elderly patients, and in patients with advanced chronic kidney disease. SRB March 2020 Mitral Stenosis • Mitral stenosis most commonly results from rheumatic heart disease fusion of the valve leaflet cusps at the commissures thickening and shortening of the chordae calcium deposition within the valve leaflets • Characteristic “fish-mouth” or “hockey stick” appearance on the echocardiogram (depending on view) SRB March 2020 Mitral Stenosis: Natural History • The severity of symptoms depends primarily on the degree of stenosis. • Symptoms often go unrecognized by patient and physician until significant shortness of breath, hemoptysis, or atrial fibrillation develops.
    [Show full text]
  • How to Define Valvular Atrial Fibrillation?
    Archives of Cardiovascular Disease (2015) 108, 530—539 Available online at ScienceDirect www.sciencedirect.com REVIEW How to define valvular atrial fibrillation? Comment définir la fibrillation atriale valvulaire ? ∗ Laurent Fauchier , Raphael Philippart, Nicolas Clementy, Thierry Bourguignon, Denis Angoulvant, Fabrice Ivanes, Dominique Babuty, Anne Bernard Service de cardiologie, faculté de médecine, université Franc¸ois-Rabelais, CHU Trousseau, Tours, France Received 3 June 2015; accepted 8 June 2015 Available online 14 July 2015 KEYWORDS Summary Atrial fibrillation (AF) confers a substantial risk of stroke. Recent trials compar- Atrial fibrillation; ing vitamin K antagonists (VKAs) with non-vitamin K antagonist oral anticoagulants (NOACs) in Valve disease; AF were performed among patients with so-called ‘‘non-valvular’’ AF. The distinction between Stroke ‘‘valvular’’ and ‘‘non-valvular’’ AF remains a matter of debate. Currently, ‘‘valvular AF’’ refers to patients with mitral stenosis or artificial heart valves (and valve repair in North American guidelines only), and should be treated with VKAs. Valvular heart diseases, such as mitral regur- gitation, aortic stenosis (AS) and aortic insufficiency, do not result in conditions of low flow in the left atrium, and do not apparently increase the risk of thromboembolism brought by AF. Post-hoc analyses suggest that these conditions probably do not make the thromboembolic risk less responsive to NOACs compared with most forms of ‘‘non-valvular’’ AF. The pathogenesis of thrombosis is probably different for blood coming into contact with a mechanical prosthetic valve compared with what occurs in most other forms of AF. This may explain the results of the only trial performed with a NOAC in patients with a mechanical prosthetic valve (only a few of whom had AF), where warfarin was more effective and safer than dabigatran.
    [Show full text]
  • An Uncommon Clinical Presentation of Relapsing Dilated Cardiomyopathy with Identification of Sequence Variations in MYNPC3, KCNH2 and Mitochondrial Trna Cysteine M
    View metadata, citation and similar papers at core.ac.uk brought to you by CORE provided by George Washington University: Health Sciences Research Commons (HSRC) Himmelfarb Health Sciences Library, The George Washington University Health Sciences Research Commons Pediatrics Faculty Publications Pediatrics 6-2015 An uncommon clinical presentation of relapsing dilated cardiomyopathy with identification of sequence variations in MYNPC3, KCNH2 and mitochondrial tRNA cysteine M. J. Guillen Sacoto Kimberly A. Chapman George Washington University D. Heath M. B. Seprish Dina Zand George Washington University Follow this and additional works at: http://hsrc.himmelfarb.gwu.edu/smhs_peds_facpubs Part of the Pediatrics Commons Recommended Citation Guillen Sacoto, M.J., Chapman, K.A., Heath, D., Seprish, M.B., Zand, D.J. (2015). An uncommon clinical presentation of relapsing dilated cardiomyopathy with identification of sequence variations in MYNPC3, KCNH2 and mitochondrial tRNA cysteine. Molecular Genetics and Metabolism Reports, 3, 47-54. doi:10.1016/j.ymgmr.2015.03.007 This Journal Article is brought to you for free and open access by the Pediatrics at Health Sciences Research Commons. It has been accepted for inclusion in Pediatrics Faculty Publications by an authorized administrator of Health Sciences Research Commons. For more information, please contact [email protected]. Molecular Genetics and Metabolism Reports 3 (2015) 47–54 Contents lists available at ScienceDirect Molecular Genetics and Metabolism Reports journal homepage: http://www.journals.elsevier.com/molecular-genetics-and- metabolism-reports/ Case Report An uncommon clinical presentation of relapsing dilated cardiomyopathy with identification of sequence variations in MYNPC3, KCNH2 and mitochondrial tRNA cysteine Maria J. Guillen Sacoto a,1, Kimberly A.
    [Show full text]
  • Currentstateofknowledgeonaetiol
    European Heart Journal (2013) 34, 2636–2648 ESC REPORT doi:10.1093/eurheartj/eht210 Current state of knowledge on aetiology, diagnosis, management, and therapy of myocarditis: a position statement of the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases Downloaded from Alida L. P. Caforio1†*, Sabine Pankuweit2†, Eloisa Arbustini3, Cristina Basso4, Juan Gimeno-Blanes5,StephanB.Felix6,MichaelFu7,TiinaHelio¨ 8, Stephane Heymans9, http://eurheartj.oxfordjournals.org/ Roland Jahns10,KarinKlingel11, Ales Linhart12, Bernhard Maisch2, William McKenna13, Jens Mogensen14, Yigal M. Pinto15,ArsenRistic16, Heinz-Peter Schultheiss17, Hubert Seggewiss18, Luigi Tavazzi19,GaetanoThiene4,AliYilmaz20, Philippe Charron21,andPerryM.Elliott13 1Division of Cardiology, Department of Cardiological Thoracic and Vascular Sciences, University of Padua, Padova, Italy; 2Universita¨tsklinikum Gießen und Marburg GmbH, Standort Marburg, Klinik fu¨r Kardiologie, Marburg, Germany; 3Academic Hospital IRCCS Foundation Policlinico, San Matteo, Pavia, Italy; 4Cardiovascular Pathology, Department of Cardiological Thoracic and Vascular Sciences, University of Padua, Padova, Italy; 5Servicio de Cardiologia, Hospital U. Virgen de Arrixaca Ctra. Murcia-Cartagena s/n, El Palmar, Spain; 6Medizinische Klinik B, University of Greifswald, Greifswald, Germany; 7Department of Medicine, Heart Failure Unit, Sahlgrenska Hospital, University of Go¨teborg, Go¨teborg, Sweden; 8Division of Cardiology, Helsinki University Central Hospital, Heart & Lung Centre,
    [Show full text]
  • Echo in Asymptomatic Mitral and Aortic Regurgitation
    2017 ASE Florida | Orlando, FL October 9, 2017 | 10:40 – 11:00 PM | 20 min | Grand Harbor Ballroom South Echo in Asymptomatic Mitral and Aortic Regurgitation Muhamed Sarić MD, PhD, MPA Director of Noninvasive Cardiology | Echo Lab Associate Professor of Medicine Disclosures Speakers Bureau (Philips, Medtronic) Advisory Board (Siemens) Regurgitation Axioms ▪Typically, regurgitation is NOT symptomatic unless severe ▪The opposite is not true: Severe regurgitation may be asymptomatic ▪ Chronic regurgitation leads to chamber dilatation on either side of the regurgitant valve Regurgitation Discovery ▪ Regurgitation as a anatomic entity was recognized in the 17th century ▪ Regurgitation was first clinically diagnosed by auscultation in the 19th century, well before the advent of echocardiography First Use of Regurgitation Term in English 1683 W. Charleton Three Anat. Lect. i. 18 Those [valves] that are placed in the inlet and outlet of the left Ventricle, to obviate the regurgitation of the bloud into the arteria venosa, and out of the aorta into the left Ventricle. Walter Charleton (1619 – 1707) English Physician Heart Murmur OXFORD ENGLISH DICTIONARY DEFINITION ▪ Any of various auscultatory sounds ▪ Adventitious sounds of cardiac or vascular origin [that is, separate from standard heart sounds: S1, S2, S3, S4] ▪ Sometimes of no significance ▪ But sometimes caused by valvular lesions of the heart or other diseases of the Στῆθος : Stēthos = chest circulatory system René Laënnec Stethoscope (1781 – 1826) (‘Chest examiner’) French Physician Hollow wooden cylinder Inventor of stethoscope in 1816 Laënnec Performing Auscultation Painted by Robert Alan Thom (1915 – 1979), American illustrator Commissioned by Parke, Davis & Co. 1816 1832 René Laënnec, James Hope French physician British physician Invents MONAURAL stethoscope separates MS from MR murmur 1852 1862 George Cammann Austin Flint Sr.
    [Show full text]
  • Avalus™ Pericardial Aortic Surgical Valve System
    FACT SHEET Avalus™ Pericardial Aortic Surgical Valve System Aortic stenosis is a common heart problem caused by a narrowing of the heart’s aortic valve due to excessive calcium deposited on the valve leaflets. When the valve narrows, it does not open or close properly, making the heart work harder to pump blood throughout the body. Eventually, this causes the heart to weaken and function poorly, which may lead to heart failure and increased risk for sudden cardiac death. Disease The standard treatment for patients with aortic valve disease is surgical aortic valve Overview: replacement (SAVR). During this procedure, a surgeon will make an incision in the sternum to open the chest and expose the heart. The diseased native valve is then Aortic removed and a new artificial valve is inserted. Once in place, the device is sewn into Stenosis the aorta and takes over the original valve’s function to enable oxygen-rich blood to flow efficiently out of the heart. For patients that are unable to undergo surgical aortic valve replacement, or prefer a minimally-invasive therapy option, an alternative procedure to treat severe aortic stenosis is called transcatheter aortic valve replacement (TAVR). The Avalus Pericardial Aortic Surgical Valve System is a next- generation aortic surgical valve from Medtronic, offering advanced design concepts and unique features for the millions of patients with severe aortic stenosis who are candidates for open- heart surgery. The Avalus Surgical Valve The Avalus valve, made of bovine tissue, is also the only stented surgical aortic valve on the market that is MRI-safe (without restrictions) enabling patients with severe aortic stenosis who have the Avalus valve to undergo screening procedures for potential co-morbidities.
    [Show full text]
  • Heart Failure
    FACT SHEET FOR PATIENTS AND FAMILIES Heart Failure What is it? Enlarged heart Heart failure is a condition in which your heart can’t pump enough blood to meet your body’s needs. Usually, this is because your heart muscle is too weak to “squeeze” out enough blood with each beat. But heart failure can also happen when your heart gets stiff “Normal” heart and can’t fill up with enough blood between each beat. Heart failure is found most often in older people, but it can happen to anyone at any age. It’s a serious condition — and also quite common. Many people with heart failure continue to have a full and active life for many years after their diagnosis. What are the symptoms? Symptoms of heart failure vary based on the type of With heart failure, initial damage weakens the heart failure you have. Common symptoms include: heart muscle. This makes your heart beat faster, and the muscle stretches or thickens. Over time, • Shortness of breath the heart muscle begins to wear out. • Cough • Feeling very tired and weak • Atherosclerosis (coronary artery disease). • Weight gain (from fluid buildup) Atherosclerosis is when the arteries that supply your • Swollen ankles, feet, belly, lower back, and fingers heart with blood become narrowed by fatty plaque • Puffiness or swelling around the eyes buildup. This restricts the amount of oxygen your • Trouble concentrating or remembering heart gets and weakens the muscle. It can also cause a heart attack, which can damage your heart even more. The main cause of heart failure (heart muscle damage and weakness) cannot be cured, but symptoms can be • High blood pressure (hypertension).
    [Show full text]
  • Heart Disease and Diseases of the Circulatory System in Westchester
    Westchester County 2016.01 Department of Health KEEP HEALTHY @wchealthdept AND Community Health Assessment Data Update GET #keephealthy THE STATS Heart Disease and Diseases of the Circulatory System in Westchester In this issue: Heart disease as a Heart disease is the number one cause of death in Westchester County. leading cause of death in Westchester county In 2012, heart disease accounted for 2,113 deaths or 31% of all deaths Deaths due to heart disease across different in the county. Adding in 490 deaths due to stroke and other diseases population and risk of the circulatory system, total deaths from circulatory disease are groups 60% higher than the next leading cause of death - cancer. Hospitalizations due to cardiovascular disease- related conditions, Selected Causes of Death in Westchester County, 2012 including diseases of the heart Emergency room visits 2% 3% 7% due to cardiovascular disease-related 3% conditions Selected risk factors 4% that contribute to Heart Disease, cardiovascular disease 4% in Westchester county 31% 5% 9% Cerebrovascular Jiali Li, Ph.D. Director of Disease (Stroke), Research & Evaluation Neoplasms 5% Planning & Evaluation (Cancer), 24% Other Circulatory, Renee Recchia, MPH 3% Acting Deputy Commissioner of Administration Heart Disease Cerebrovascular Disease (Stroke) Project Staff: Other Circulatory Neoplasms (Cancer) Bonnie Lam, MPH Respiratory Diseases External Causes (e.g. accidents) Medical Data Analyst Communicable Diseases Nervous System Diseases Milagros Venuti, MPA Digestive System Diseases
    [Show full text]