Hypoplastic left heart syndrome: morphological study of 21 autopsy cases

Hypoplastic left heart syndrome is a group of relatively rare congenital malformations associated with severe obstruction of the left outflow tract. Twenty-one cases of Clinical with the syndrome were found in the autopsy files of the Department of Pathomorphology, Medical University of Lublin in the years 1991-2000. Most children were males, delivered at term, with normal body weight. None of them was surgically treated nor survived longer than one month. The most common subtype of valvular malformations included in the syndrome was aortic stenosis with mitral stenosis. In 15 cases microscopic examination revealed: recent myocardial necrosis, interstitial fibrosis and focal calcification. In 6 cases the syndrome coexisted with the congenital malformations of other systems.