Operative treatment of hypertrophic obstructive cardiomyopathy and aortic valve disease in infants.

Abstract

The indications for operative intervention for symptomatic hypertrophic obstructive cardiomyopathy (HOCM) in infancy and childhood are not well defined because of the rarity of the lesion. The traditional surgical procedure consists of septal myectomy. In an attempt to further improve the outcome of HOCM associated with concentric left ventricular hypertrophy and aortic valve disease in infancy, we have combined resection of the left ventricular septum and free wall with a Ross-Konno procedure. Three infants (aged 3, 4, and 10 months) with HOCM (left ventricular aortic gradients of 75, 95, and 110 mmHg), associated concentric left ventricular hypertrophy, and valvar aortic stenosis (n = 1) or combined valvar aortic stenosis and regurgitation (n = 2) underwent extensive resection of fibroelastosis and subendocardial myocardium of the left ventricular septum and free wall in combination with a Ross-Konno operation. All three patients had marked systolic anterior motion of the mitral valve. The length of the incision into the ventricular septum was 1.8, 2.0, and 2.3 cm. In all three patients this procedure resulted in a marked reduction of width of the left ventricular septum (median 9 mm vs 14 mm preoperatively) and the left ventricular posterior free wall (median 8 mm vs 12 mm preoperatively) and an almost twofold increase of the left ventricular end-diastolic volume (median 13.5 cm3 vs 7.0 cm3 preoperatively). The neo-aortic valve functioned normally. Systolic anterior motion of the anterior leaflet of the mitral valve had completely resolved in two patients and had markedly regressed in the remaining patient. At follow-up of 15, 17, and 26 months, two patients had absence of a left ventricular outflow tract gradient and the third patient had a residual sub-valvar gradient of 15 mmHg. The reported procedure may be a valuable technique in severe forms of hypertrophic cardiomyopathy associated with aortic valve disease. The operation results in enlargement of the left ventricular stroke volume and improvement of the left ventricular diastolic function, restores aortic valve anatomy and function, and abolishes or decreases systolic anterior motion of the mitral valve.