(Circulation. 2000;102:600.)
© 2000 American Heart Association, Inc.
Editorial |
From the Section of Cardiology, Department of Medicine, Baylor College of Medicine, Houston, Tex.
Correspondence to Robert Roberts, MD, Section of Cardiology, Baylor College of Medicine, 6550 Fannin, MS SM677, Houston, TX 77030. E-mail rroberts@bcm.tmc.edu
Key Words: Editorials cardiomyopathy ablation genetics
Familial hypertrophic cardiomyopathy is a genetic disease with an autosomal-dominant inheritance.1 2 3 Patients with this illness are prone to sudden death, angina, syncope, and heart failure. A subset of patients with familial hypertrophic cardiomyopathy have left ventricular outflow tract obstruction (or hypertrophic obstructive cardiomyopathy [HOCM]) at rest or HOCM that can be induced with the Valsalva maneuver or with dobutamine, isoproterenol infusion, or amyl nitrite inhalation. Patients with significant obstruction have systolic anterior motion of the mitral valve associated with the outflow obstruction, which leads to mitral regurgitation. In association with the hemodynamic burden caused by left ventricular outflow tract obstruction, other abnormalities also contribute to the disabling symptoms. These include impaired left ventricular diastolic and systolic dysfunction, myocardial ischemia, and arrhythmias. In some variant patterns, the level of obstruction may be in the mid-left ventricular cavity rather than subaortic. In addition, midventricular obstruction may be associated with subaortic obstruction. With all these abnormalities of ventricular function, the disease is frequently disabling and progressive.
Numerous treatment options have been suggested for
HOCM.4 5 The primary aim of medical therapy is relief of
left ventricular outflow tract obstruction; this is often
done using medications such as ß-blockers, calcium channel blockers
(especially verapamil), and disopyramide.
Medical therapy benefits many patients and allows them to lead
acceptable, productive lives. Frequently, however, high doses of
medication produce side effects that resemble the symptoms of HOCM. In
the past, cardiac pacing has been suggested as a method of reducing the
outflow tract obstruction and improving symptomatology.6 7
However,
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