肥厚性心肌病變最新診斷及治療

Hypertrophy cardiomyopathy (HCM) is a genetic associated cardiac disease with a prevalence rate of 0.2% in the general population. Men are more common than women. Pathophysiologic presentations of this disorder include hyperdynamic systolic contraction, myocardial ischemia, arrhythmia, and heart failure. Most of HCM patients are asymptomatic, only present symptoms when the patients have high pressure gradient of the left ventricular outflow tract (LVOT), which was featured in hypertrophic obstructive cardiomyopathy. Current diagnostic modalities include echocardiography, magnetic resonance imaging and genetic test. Pharmacologic therapy, including beta-adrenergic-blocking agents, non-DHP calcium channel blockers and disopyramide is the first step to relieve symptoms. Mava-camten, an ATPase inhibitor can reduce LVOT obstruction, improve exercise capacity, and decrease New York Heart Association functional class in patients with obstructive hypertrophic cardiomyopathy in a phase 3 trial. Aficamten, another ATPase inhibitor, is on the way to prove its safety and efficacy. When patients have a poor response to medication, they can be treated by invasive procedures such as surgical septal myectomy or percutaneous transluminal septal myocardial ablation (PTSMA). Although there was an absence of evidence to prove which procedure is better, PTSMA has a higher rate of atrial-ventricular block and recurrence. The current guideline suggests patients having symptoms in pharmacological therapy or contraindicated to surgical septal myectomy, should consider PTSMA as the treatment.