Results
The children, of whom 23 were male, were aged between 4 months and 9 years at first presentation, with a median of 2 years and 3 months. Cardiac failure, seen in almost half of them, was the most frequent presenting feature. In half of them reason for cardiac examination was systolic heart murmur. In admission, on the chest x-ray, the cardiothoracic ratio was increased, to a mean of 72 % in 5 infants and to 65 % in 37 older children. Measured by M mode and 2-D echocardiography hypertrophy of left ventricular wall was noted, where in 28 patients hypertrophy was asymmetric while 14 had concentric hypertrophy of left ventricle. Left ventricular ejection fraction was depressed in the 21 patients, all with signs of heart failure. Patients in cardiac failure received various combinations of diuretics, B-blockers, ACE inhibitors and aspirin. Surgical intervention was not performed to none of them, despite of clinical and echocardiographic indications. Death occurred in 8 patients, in 4 of them shortly after admission, 3 left Kosovo against medical advice and continued examination in Italy and Germany, and the remaining 32 were followed- up for a mean 42 months, with a range from 5 to 115 months. Recovery was noted in 14 patients but still requiring anti-failure medications. Slightly over two-fifths died. Of those with asymmetric form, 45 % died, in half of those presenting in infancy, and 89 % of those who presented with signs of cardiac failure.
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