MY APPROACH to clinical management of hypertrophic cardiomyopathy

Abstract

1016/j.tcm.2014.06.006 sevier Inc. All rights reserved. n PracticeUpdate on June 2, 2014. Republished wi on the individual clinical scenario) may also be enough to consider a patient at risk and offer a primary-prevention ICD. In addition, I obtain a cardiac MRI in all eligible patients because high–spatial resolution imaging provides a very accurate measurement of maximal LV wall thickness, which may be underestimated by echocardiography in some patients. Furthermore, myocardial fibrosis identified by MRI is an emerging risk factor in HCM, and, in patients in whom the risk for sudden death remains ambiguous after assessment with the traditional risk factors, identification of extensive scarring (Z15% of the LV mass) can help resolve complex ICD decision-making. In patients with limiting heart failure symptoms, the presence of LV outflow tract obstruction must be determined so that optimal management strategies can be formulated. If obstruction is not present under resting conditions, then I nearly always pursue maneuvers to provoke for latent obstruction with exercise echocardiography as the preferred method. For patients with outflow obstruction at rest (or with provocation) and limiting heart failure symptoms, medical therapy should be initiated. Beta blockers are generally preferred and are dose-titrated to symptom benefit. Alternatively, calcium channel blockers can be tried, and, in some patients, disopyramide extended release (Norpace CR) can be added to a beta blocker, although this option is often limited by adverse effects. I only recommend invasive septal reduction therapy (myectomy or alcohol septal ablation) in patients with obstruction with drug-refractory advanced heart failure symptoms. If a patient has no evidence of outflow obstruction at rest or with provocation and heart failure symptoms, AV nodalblocking agents can be offered to improve diastolic dysfunction. If patients continue to experience progressive heart failure despite these drugs, then heart transplantation is the only long-term treatment option. If a patient is asymptomatic, there is no compelling role for drug therapy.