Hypoplastic left heart syndrome with parchment left ventricle: A rare perinatal autopsy case report

Hypoplastic left heart syndrome (HLHS) refers to the abnormal development of the left-sided cardiac structures, resulting in obstruction to blood fl ow from the left ventricular outfl ow tract. In addition, the syndrome includes underdevelopment of the left ventricle, aorta, and aortic arch, as well as mitral atresia or stenosis. The syndrome can be diagnosed by fetal echocardiography between 18 and 22 weeks of gestation. This is a fetal autopsy case report of HLHS, which was diagnosed on ultrasonography and medically terminated at 20 weeks of gestation. The autopsy and histopathological fi ndings are discussed in detail. Some important aspects of epidemiology, prenatal diagnosis, and current literature on HLHS are highlighted.