2020 AHA/ACC guideline for the diagnosis and treatment of patients with hypertrophic cardiomyopathy: A report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines.

Published on Apr 26, 2021in The Journal of Thoracic and Cardiovascular Surgery4.451
· DOI :10.1016/J.JTCVS.2021.04.001
Steve R. Ommen78
Estimated H-index: 78
Seema Mital49
Estimated H-index: 49
+ 40 AuthorsY. Joseph Woo59
Estimated H-index: 59
#1Ethan J. Rowin (Tufts Medical Center)H-Index: 26
#2Aadhavi Sridharan (Tufts Medical Center)H-Index: 1
Last. Barry J. Maron (Tufts Medical Center)H-Index: 169
view all 8 authors...
ABSTRACT Highly reliable identification of adults with hypertrophic cardiomyopathy (HC) at risk for sudden death (SD) has been reported. A significant controversy remains, however, regarding the most reliable risk stratification methodology for children and adolescents with HC. The present study assesses the accuracy of SD prediction and prevention with prophylactic ICDs in young HC patients. The study group is comprised of 146 HC patients
6 CitationsSource
#1Anastasia MironH-Index: 2
#2Myriam Lafreniere-Roula (UHN: University Health Network)H-Index: 13
Last. Seema Mital (U of T: University of Toronto)H-Index: 49
view all 42 authors...
Background: Hypertrophic cardiomyopathy (HCM) is the leading cause of sudden cardiac death (SCD) in children and young adults. Our objective was to develop and validate a SCD risk prediction model ...
26 CitationsSource
#1Ethan J. Rowin (Tufts Medical Center)H-Index: 26
#2Barry J. Maron (Tufts Medical Center)H-Index: 169
Last. Martin S. Maron (Tufts Medical Center)H-Index: 70
view all 7 authors...
Abstract Background End-stage (ES) hypertrophic cardiomyopathy (HCM) has been considered a particularly grim and unfavorable disease complication, associated with substantial morbidity and mortality, frequently requiring heart transplant. Previous reports have included small numbers of patients with relatively short follow-up, predominantly in prior treatment eras. Objectives The purpose of this study was to re-evaluate clinical profile and prognosis for end-stage heart failure in a large HCM co...
19 CitationsSource
#1Carolyn Y. Ho (Brigham and Women's Hospital)H-Index: 50
Last. Stephen B. Heitner (OHSU: Oregon Health & Science University)H-Index: 15
view all 23 authors...
Abstract Background Patients with nonobstructive hypertrophic cardiomyopathy (nHCM) often experience a high burden of symptoms; however, there are no proven pharmacological therapies. By altering the contractile mechanics of the cardiomyocyte, myosin inhibitors have the potential to modify pathophysiology and improve symptoms associated with HCM. Objectives MAVERICK-HCM (Mavacamten in Adults With Symptomatic Non-Obstructive Hypertrophic Cardiomyopathy) explored the safety and efficacy of mavacam...
41 CitationsSource
#1Carolyn Y. Ho (Brigham and Women's Hospital)H-Index: 50
#2Iacopo OlivottoH-Index: 64
Last. Stephen B. Heitner (OHSU: Oregon Health & Science University)H-Index: 15
view all 10 authors...
BACKGROUND Obstructive hypertrophic cardiomyopathy (oHCM) is characterized by unexplained left ventricular (LV) hypertrophy associated with dynamic LV outflow tract obstruction. Current medical therapies are nonspecific and have limited efficacy in relieving symptoms. Mavacamten is a first-in-class targeted inhibitor of cardiac myosin, which has been shown to reduce LV outflow tract obstruction, improve exercise capacity, and relieve symptoms of oHCM in the PIONEER-HCM phase 2 study. METHODS EXP...
14 CitationsSource
#1Anupam MitraH-Index: 15
#2Raktim K. GhoshH-Index: 16
Last. Carl J. LavieH-Index: 121
view all 6 authors...
Abstract Hypertrophic cardiomyopathy (HCM) is the most prevalent hereditary cardiac disease characterized by the presence of left ventricular and/or septal hypertrophy in the absence of other underlying cardiac disorders. Patients of HCM have a broad range of clinical presentation from being asymptomatic to severely ill condition requires hospitalization and urgent management. Broadly, HCM is classified in two variants: obstructive and nonobstructive. The mainstay of diagnosis is through echocar...
9 CitationsSource
#1Mark V. SherridH-Index: 33
#2Katherine RiedyH-Index: 4
Last. Harmony R. ReynoldsH-Index: 37
view all 16 authors...
Abstract Clinical spectrum of hypertrophic cardiomyopathy (HC) has been expanded to include patients with mild or no thickening of the left ventricle (LV), who nevertheless have outflow tract obstruction at rest or after exercise, due to systolic anterior motion (SAM) and ventricular septal contact, with mitral valve elongation and papillary muscles anomalies. Apical ballooning mimicking a takotsubo syndrome (TS) wall motion pattern can occur in HC with mild septal thickening when latent obstruc...
5 CitationsSource
#1Peter Marstrand (Brigham and Women's Hospital)H-Index: 2
#2Larry Han (Harvard University)H-Index: 16
Last. Carolyn Y. Ho (Brigham and Women's Hospital)H-Index: 50
view all 17 authors...
Background: The terminology "end-stage" has been used to describe hypertrophic cardiomyopathy (HCM) with left ventricular systolic dysfunction (herein referred to as HCM-LVSD), defined when left ventricular ejection fraction (LVEF) <50% is present. The prognosis of HCM-LVSD has reportedly been poor, but due to its relative rarity, natural history remains incompletely characterized. Methods: Data from eleven high-volume HCM specialty centers comprising the international Sarcomeric Human Cardiomyo...
26 CitationsSource
#1Antonio R. Polanco (Columbia University)H-Index: 3
#1Antonio R. Polanco (Columbia University)H-Index: 5
Last. Hiroo Takayama (Columbia University)H-Index: 58
view all 8 authors...
OBJECTIVE: Mitral regurgitation (MR) induced by systolic anterior motion in patients with hypertrophic cardiomyopathy (HCM) can frequently be abolished with a proficient septal myectomy (SM) without the need for mitral-valve replacement (MVR). ACC guidelines stress the importance of volume in improving outcomes after SM, but there is a lack of data measuring the impact of volume on the need for MVR during SM. This study was designed to assess the impact of institutional volume on MVR rates using...
6 CitationsSource
#1Carlo FumagalliH-Index: 8
#2Niccolò MauriziH-Index: 11
Last. Iacopo OlivottoH-Index: 64
view all 11 authors...
Importance Patients with hypertrophic cardiomyopathy (HCM) are prone to body weight increase and obesity. Whether this predisposes these individuals to long-term adverse outcomes is still unresolved. Objective To describe the association of body mass index (BMI, calculated as weight in kilograms divided by height in meters squared) with long-term outcomes in patients with HCM in terms of overall disease progression, heart failure symptoms, and arrhythmias. Design, Setting, and Participants In th...
23 CitationsSource
Cited By11
#1Diego PenelaH-Index: 18
#2Antonio SorgenteH-Index: 18
Last. Riccardo CappatoH-Index: 37
view all 3 authors...
Atrial fibrillation (AF) and hypertrophic cardiomyopathy (HCM) are two very common clinical entities, which often occur simultaneously, giving a hard time to both patients and cardiologists. Myocyte hypertrophy, myocyte disarray and interstitial fibrosis in the left atrium (LA) predisposes to atrial arrhythmias due to modifications of the substrate that promote re-entry. AF is usually poorly tolerated due to the shortening of the diastolic time with rapid heart rates and the lack of the atrial c...
Atrial fibrillation (AF) leads to remodeling characterized by changes in both size and shape of the left atrium (LA). Here we aimed to study the effect of hypertrophic cardiomyopathy (HCM) on the pattern of LA remodeling in AF-patients. HCM-patients (n = 23) undergoing AF ablation (2009-2012) were matched and compared with 125 Non-HCM patients from our prospective registry. Pre-procedural CT data were analyzed (EnSite Verismo, SJM, MN) to determine the maximal sagittal (anterior-posterior, AP), ...
#1Praveen Kerala Varma (AIMS: Amrita Institute of Medical Sciences and Research Centre)H-Index: 10
#2Kirun Gopal (AIMS: Amrita Institute of Medical Sciences and Research Centre)H-Index: 3
Last. Sudheer Babu Vanga (AIMS: Amrita Institute of Medical Sciences and Research Centre)H-Index: 1
view all 4 authors...
Abnormalities of the mitral valve apparatus are a phenotypical expression of hypertrophic cardiomyopathy and can contribute to systolic anterior motion and left ventricular outflow tract obstruction. In patients with significant abnormalities of the mitral apparatus, adjunct procedures like plication of the anterior mitral leaflet and/or release of papillary muscles may be required to obtain sufficient relief of obstruction. Very rarely, an elongated posterior mitral leaflet is the main culprit ...
#1Stephanie NguyenH-Index: 5
#2David Blitzer (CUMC: Columbia University Medical Center)H-Index: 2
Last. Hiroo TakayamaH-Index: 1
view all 4 authors...
Hypertrophic cardiomyopathy (HCM) is an inherited cardiac condition characterized by left ventricular muscular hypertrophy in the absence of other cardiac, systemic, or metabolic conditions like hypertension, aortic stenosis, amyloidosis, glycogen storage diseases, or lysosomal storage diseases. The diagnosis is established by non-invasive imaging modalities such as two-dimensional echocardiography (ECHO) or cardiovascular magnetic resonance (CMR), or cardiac computed tomography (CT). The maxima...
#1Sara Bombace (Humanitas University)
Last. Lorenzo MontiH-Index: 3
view all 4 authors...
Hypertrophic cardiomyopathy (HCM) is a genetic cardiac disease that presents with cardiac hypertrophy. HCM phenocopies are clinical conditions that are phenotypically undistinguishable from HCM, but with a different underlying etiology. Cardiac tumors are rare entities that can sometimes mimic HCM in their echocardiographic appearance, thus representing an example of HCM phenocopy. At present, only case reports of tumoral HCM phenocopies can be found in literature. In this systematic review, we ...
#1Erwan Donal (French Institute of Health and Medical Research)H-Index: 68
#2Vasileios Panis (French Institute of Health and Medical Research)
Last. Wojciech Kosmala (Wrocław Medical University)H-Index: 21
view all 3 authors...
In recent years, there has been an increasing use of stress echocardiography in the assessment of non-ischaemic cardiac disease. Exercise stress echocardiography (ESE) provides a unique ability for simultaneous assessment of both cardiac performance and exercise-related non-invasive haemodynamic changes, which can help guide treatment and inform about the prognosis of patients.1 The recommendations of European Association for Cardiovascular Imaging and American Society of Echocardiography have t...
#1Ilaria My (Humanitas University)H-Index: 8
#1Ilaria MyH-Index: 1
Last. Elisa Di Pasquale (National Research Council)H-Index: 17
view all 2 authors...
Genetic cardiomyopathies represent a wide spectrum of inherited diseases and constitute an important cause of morbidity and mortality among young people, which can manifest with heart failure, arrhythmias, and/or sudden cardiac death. Multiple underlying genetic variants and molecular pathways have been discovered in recent years; however, assessing the pathogenicity of new variants often needs in-depth characterization in order to ascertain a causal role in the disease. The application of human...
#1Alessia ArgiròH-Index: 1
#2Mattia ZampieriH-Index: 3
Last. Iacopo OlivottoH-Index: 64
view all 12 authors...
Hypertrophic cardiomyopathy (HCM) is a common myocardial disease characterized by otherwise unexplained left ventricular hypertrophy. The main cause of disabling symptoms in patients with HCM is left ventricular outflow tract (LVOT) obstruction. This phenomenon is multifactorial, determined both by anatomical and functional abnormalities: myocardial hypercontractility is believed to represent one of its major determinants. The anatomical anomalies are targeted by surgical interventions, whereas ...
2 CitationsSource