Abstract

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. Conclusion: Hypertrophic cardiomyopathy manifested often in severe form in our patients, and the response to standard anti-failure therapy was unsatisfactory. The goals of therapy in HCM are symptoms control and prolongation of survival. In summary, randomized clinical trials would be quite valuable to help define optimal care of children with hypertrophic cardiomyopathy. Significant challenges exist, such as various medical combinations in therapy, surgical intervention and heart transplantation.

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