Management of Hypertrophic Cardiomyopathy

Background: Hypertrophic cardiomyopathy (HCM) is clinically defined as unexplained myocardial hypertrophy, and it is an autosomal dominant disease of the cardiac sarcomere. It is present in 1 in 500 in the general adult population, making it the most common genetic cardiovascular disease. The pathop...

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Autores principales:	Alan D. Enriquez, Martin E. Goldman
Formato:	article
Lenguaje:	EN
Publicado:	Ubiquity Press 2014
Materias:	alcohol septal ablation genetic screening hypertrophic cardiomyopathy implantable cardioverter-defibrillators sudden cardiac death surgical myectomy Infectious and parasitic diseases RC109-216 Public aspects of medicine RA1-1270
Acceso en línea:	https://doaj.org/article/cf3cb7ecb20e4a078b8f7c403c1ad179
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spelling	oai:doaj.org-article:cf3cb7ecb20e4a078b8f7c403c1ad1792021-12-02T05:15:58ZManagement of Hypertrophic Cardiomyopathy2214-999610.1016/j.aogh.2013.12.004https://doaj.org/article/cf3cb7ecb20e4a078b8f7c403c1ad1792014-04-01T00:00:00Zhttps://annalsofglobalhealth.org/articles/64https://doaj.org/toc/2214-9996Background: Hypertrophic cardiomyopathy (HCM) is clinically defined as unexplained myocardial hypertrophy, and it is an autosomal dominant disease of the cardiac sarcomere. It is present in 1 in 500 in the general adult population, making it the most common genetic cardiovascular disease. The pathophysiology of HCM is complex, leading to significant variability in clinical presentation. This, combined with the lack of randomized trials, makes the management of these patients difficult. Findings: The majority of patients with HCM are asymptomatic without a substantial reduction in survival. However, a considerable portion of patients will experience significant symptoms and HCM-related death, and effective therapies are available for these patients. Patients may have symptoms of heart failure from outflow tract obstruction and/or restrictive physiology. Medical therapy targeted at the underlying pathophysiology should be used, and surgical myectomy or alcohol septal ablation is available for those with refractory symptoms. While the overall risk of sudden cardiac death (SCD) is low in HCM patients, some are at elevated risk for and experience SCD, a devastating outcome in young patients. Risk stratification for SCD and treatment with implantable cardioverter-defibrillators is paramount. Many HCM patients will also develop atrial fibrillation, and this is often poorly tolerated. A rhythm control strategy with antiarrhythmic drugs or catheter ablation is often necessary, and anticoagulation should be administered to reduce the risk of thromboembolism. Finally, family members of patients with HCM should be regularly screened with electrocardiography and echocardiography. Conclusions: HCM is a complex disease with heterogeneous phenotypes and clinical manifestations. The management of HCM focuses on reducing symptoms of heart failure, preventing SCD, treating atrial fibrillation, and screening family members. Treatment should be tailored to the unique characteristics of each individual patient.Alan D. EnriquezMartin E. GoldmanUbiquity Pressarticlealcohol septal ablationgenetic screeninghypertrophic cardiomyopathyimplantable cardioverter-defibrillatorssudden cardiac deathsurgical myectomyInfectious and parasitic diseasesRC109-216Public aspects of medicineRA1-1270ENAnnals of Global Health, Vol 80, Iss 1, Pp 35-45 (2014)
institution	DOAJ
collection	DOAJ
language	EN
topic	alcohol septal ablation genetic screening hypertrophic cardiomyopathy implantable cardioverter-defibrillators sudden cardiac death surgical myectomy Infectious and parasitic diseases RC109-216 Public aspects of medicine RA1-1270
spellingShingle	alcohol septal ablation genetic screening hypertrophic cardiomyopathy implantable cardioverter-defibrillators sudden cardiac death surgical myectomy Infectious and parasitic diseases RC109-216 Public aspects of medicine RA1-1270 Alan D. Enriquez Martin E. Goldman Management of Hypertrophic Cardiomyopathy
description	Background: Hypertrophic cardiomyopathy (HCM) is clinically defined as unexplained myocardial hypertrophy, and it is an autosomal dominant disease of the cardiac sarcomere. It is present in 1 in 500 in the general adult population, making it the most common genetic cardiovascular disease. The pathophysiology of HCM is complex, leading to significant variability in clinical presentation. This, combined with the lack of randomized trials, makes the management of these patients difficult. Findings: The majority of patients with HCM are asymptomatic without a substantial reduction in survival. However, a considerable portion of patients will experience significant symptoms and HCM-related death, and effective therapies are available for these patients. Patients may have symptoms of heart failure from outflow tract obstruction and/or restrictive physiology. Medical therapy targeted at the underlying pathophysiology should be used, and surgical myectomy or alcohol septal ablation is available for those with refractory symptoms. While the overall risk of sudden cardiac death (SCD) is low in HCM patients, some are at elevated risk for and experience SCD, a devastating outcome in young patients. Risk stratification for SCD and treatment with implantable cardioverter-defibrillators is paramount. Many HCM patients will also develop atrial fibrillation, and this is often poorly tolerated. A rhythm control strategy with antiarrhythmic drugs or catheter ablation is often necessary, and anticoagulation should be administered to reduce the risk of thromboembolism. Finally, family members of patients with HCM should be regularly screened with electrocardiography and echocardiography. Conclusions: HCM is a complex disease with heterogeneous phenotypes and clinical manifestations. The management of HCM focuses on reducing symptoms of heart failure, preventing SCD, treating atrial fibrillation, and screening family members. Treatment should be tailored to the unique characteristics of each individual patient.
format	article
author	Alan D. Enriquez Martin E. Goldman
author_facet	Alan D. Enriquez Martin E. Goldman
author_sort	Alan D. Enriquez
title	Management of Hypertrophic Cardiomyopathy
title_short	Management of Hypertrophic Cardiomyopathy
title_full	Management of Hypertrophic Cardiomyopathy
title_fullStr	Management of Hypertrophic Cardiomyopathy
title_full_unstemmed	Management of Hypertrophic Cardiomyopathy
title_sort	management of hypertrophic cardiomyopathy
publisher	Ubiquity Press
publishDate	2014
url	https://doaj.org/article/cf3cb7ecb20e4a078b8f7c403c1ad179
work_keys_str_mv	AT alandenriquez managementofhypertrophiccardiomyopathy AT martinegoldman managementofhypertrophiccardiomyopathy
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Management of Hypertrophic Cardiomyopathy

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