Management of Heart Failure in Hypertrophic Cardiomyopathy

The following are key points to remember on the clinical spectrum and management of heart failure (HF) in hypertrophic cardiomyopathy (HCM):

1. HCM is the most common genetic heart disease, diverse in phenotypic expression and natural history, characterized by well recognized largely treatable complications, including highly visible risk for sudden death.
2. Demographics and epidemiology of HCM have evolved, with the pendulum swinging toward greater recognition of functional disability due to exertional dyspnea (and fatigue), traditionally regarded as a form of HF. HF, characterized by excessive exertional dyspnea, is now a common complication of HCM.
3. There are a number of important differences in the prevalence, clinical profile, pathophysiology, management, and outcome of HCM patients with HF compared to the larger population of non-HCM patients with conventional congestive HF.
4. HCM-related HF is most commonly due to dynamic mechanical impedance to left ventricular (LV) outflow produced by mitral valve systolic anterior motion, leading to high intracavity pressures.
5. HF in HCM is associated with diverse and complex pathophysiology with a substantially more favorable prognosis than conventional non-HCM HF, and highly amenable to effective treatment options in the vast majority of patients.
6. Traditional pharmacologic strategies to control or reduce exertional symptoms in HCM have been largely empiric, based on retrospective observational studies. Drug treatment has consisted primarily of beta-blockers and calcium antagonists (predominantly verapamil), which improve LV filling, but do not significantly reduce outflow gradients at rest.
7. Surgical septal myectomy with low operative mortality (<1%) produces HF reversal and symptom relief in 90-95% of patients, while also conveying a survival benefit.
8. In accordance with American College of Cardiology/American Heart Association guidelines, alcohol ablation is considered a therapeutic alternative to myectomy in selected patients, particularly those of advanced age or with comorbidities.
9. Exercise echocardiography has assumed an important role in the evaluation of HCM patients, by identifying candidates for septal reduction therapy with refractory HF when outflow gradients are present only with physiologic exercise, distinguishing highly symptomatic nonobstructive patients as heart transplant candidates, and predicting future development of progressive HF.
10. Treatment for advanced HF has acquired an increasingly prominent profile within the HCM disease spectrum, leading to transplant as the only definitive treatment option in a small minority of nonobstructive patients.

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