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Perspectives on the Overall Risks of Living With Hypertrophic Cardiomyopathy
Author(s) -
Barry J. Maron,
Martin S. Maron,
Ethan J. Rowin
Publication year - 2017
Publication title -
circulation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 7.795
H-Index - 607
eISSN - 1524-4539
pISSN - 0009-7322
DOI - 10.1161/circulationaha.117.027738
Subject(s) - hypertrophic cardiomyopathy , medicine , sudden cardiac death , cardiomyopathy , cardiology , intensive care medicine , heart failure
Hypertrophic cardiomyopathy (HCM) is the most common genetic heart disease, unique in many respects among cardiovascular conditions, including its extreme clinical heterogeneity. Through the years, HCM has acquired the unfortunate reputation as an unrelenting disease with a highly unfavorable and generally grim prognosis, and largely without effective treatment strategies or acceptable expectation for longevity and quality of life.1 Many patients with HCM, based on evaluation in general cardiology practices, have come to mistakenly believe that their diagnosis is among the least favorable that could confront them.This highly negative perception, created in part by early reports of cohorts impacted by patient referral bias, persists despite recent major advances in diagnosis and treatment, including advanced imaging, contemporary risk stratification, and implantable cardioverter-defibrillators for sudden death prevention2; surgical septal myectomy, alcohol ablation, and heart transplant to reverse progressive heart failure; prophylactic anticoagulation to prevent embolic stroke; and defibrillation and targeted temperature management for out-of-hospital cardiac arrest.1 …

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