Prognosis in hypertrophic cardiomyopathy observed in a large clinic population

Overall annual cardiac mortality in hypertrophic cardiomyopathy (HC) has been reported to be between 2 and 4%, although these numbers are primarily from retrospective studies of patients referred to large research institutions. A clinic population of 113 patients with HC was prospectively studied to assess cardiac mortality in the overall group and in selected subgroups commonly thought to be at high risk for sudden death. The mean age at diagnosis was 37 ± 16 years. During follow-up, there were 11 cardiac and 2 noncardiac deaths. The annual cardiac mortality was 1% (95% confidence interval 0.2–1.8%). Because of the small number of deaths, relative risk for cardiac death was not significantly different in the presence of young age (≤30 years), family history of HC and sudden death, history of syncope or previous cardiac arrest, or both, ventricular tachycardia on 24-hour Holter monitoring, or septal myotomy/myectomy for refractory symptoms and outflow tract obstruction. It is concluded that HC has a relatively benign prognosis (1% annual cardiac mortality) that is 2 to 4 times less than that previously reported

Initial results of combined anterior mitral leaflet extension and myectomy in patients with obstructive hypertrophic cardiomyopathy

Objectives. The purpose of this study was to describe the clinical and functional results of combined anterior mitral leaflet extension and myectomy in patients with hypertrophic obstructive cardiomyopathy. Background. Septal myectomy is the most commonly performed surgical procedure in patients with hypertrophic cardiomyopathy and left ventricular outflow tract obstruction. Because of the role of the mitral valve in creating the outflow tract gradient, mitral valve replacement or plication is performed in selected cases in combination with myectomy, often with better hemodynamic results than those of myectomy alone. Mitral valve leaflet extension, in which a glutaraldehyde-preserved autologous pericardial patch is used to enlarge the mitral valve along its horizontal axis, is a novel surgical approach in patients with hypertrophic obstructive cardiomyopathy. Methods. Eight patients with hypertrophic obstructive cardiomyopathy were treated with mitral leaflet extension and myectomy. Preoperative and postoperative data (New York Heart Association functional class, number of drugs prescribed, width of the interventricular septum, severity of mitral valve regurgitation, severity of systolic anterior motion of the mitral valve and outflow tract gradient) were compared with those of 12 patients undergoing myectomy alone. Results. Preoperative evaluation demonstrated that mitral regurgitation and systolic anterior motion of the mitral valve were more severe in the group undergoing mitral valve extension (p < 0.001 and p < 0.05, respectively). There were no deaths associated with either surgical procedure. Two patients, both treated by myectomy alone, died during the follow-up period. Postoperatively, patients treated with mitral valve extension had less mitral regurgitation (p < 0.005), less residual systolic anterior motion (p < 0.01), greater improvement in functional class (p = 0.05) and greater reduction in the number of drugs (p < 0.005) and in septal thickness (p < 0.05). Conclusions. Mitral leaflet extension in combination with myectomy is a promising new surgical approach that may provide superior results to those of myectomy alone. Further studies are needed to determine the clinical value of this procedure