ICD for Sudden Cardiac Death Prevention and New Pharmaceutical Treatment Options in Hypertrophic Obstructive Cardiomyopathy

Abstract

In humans, hypertrophic cardiomyopathy (HCM) is a heterogeneous cardiac illness typically caused by autosomal dominant sarcomeric gene mutations and characterized by reduced heart’s compliance, myofibrillar disarray, and fibrosis of the heart. Areas covered: Although HCM was formerly viewed as a malignant disease entity with few treatment choices, effective management strategies have emerged so that affected individuals may expect to have a normal lifespan without the need for pacing or another type of invasive intervention. Herein, these management strategies are discussed. There is no curative treatment for HCM that reverses or prevents hypertrophy and heart dysfunction. Drug-based therapies aim to alleviate its symptoms and slow disease progression. Mavacamten is a reversible cardiac myosin allosteric modulator with a potential therapeutic effect for obstructive HCM. Mavacamten markedly improved the health status of patients with symptomatic obstructive hypertrophic cardiomyopathy compared with a placebo. In patients with HOCM, the importance of an implantable cardioverter defibrillators (ICD) is to prevent sudden cardiac death (SCD). Approximately 25% of those with HCM suffer from atrial arrhythmias, and the condition is notoriously difficult to manage. Anti-arrhythmic drugs, such as sotalol, amiodarone, and disopyramide, are routinely prescribed. Radiofrequency ablations for atrial fibrillation in patients with HCM have become more common despite their limited effectiveness (about 70% recurrence).