Hypertrophic Obstructive Cardiomyopathy.

Abstract

Hypertrophic cardiomyopathy (HCM) is caused by mutations in a number of genes. Its prevalence is 0.2% to 0.6%. This review is based on publications retrieved by a selective literature search and on the authors' clinical experi- ence. 70% of patients with HCM suffer from the obstructive type of the condition, clinically characterized by highly dynamic and variable manifestations in the form of dyspnea, angina pectoris, and stress-dependent presyncope and syn- cope. Younger patients are at particular risk of sudden cardiac death; thus, all patients need not only symptomatic treatment, but also risk assessment, which can be difficult in individual cases. Left ventricular obstruction, which usually causes symptoms, is treated medically at first, with either a beta- blocker or verapamil. If medical treatment fails, two invasive treatments are available, surgical myectomy and percu- taneous septum ablation. Both of these require a high level of expertise. If performed successfully, they lead to sustained gradient reduction and clinical improvement. Septum ablation is associated with low perioperative and peri-interventional mortality but necessitates permanent pacemaker implantation in 10-20% of patients. In the absence of evidence from randomized comparison trials, a suitable method of reducing the gradient should be determined by an HCM team in conjunction with each individual patient. Important criteria for decision-making include the anatomical findings and any accompanying illnesses.