Assessment of ultrasonic imaging and pathological characteristics in fetuses with hypoplastic left heart syndrome

Abstract

Objective To detect pathologic and prenatal echocardiography features of the heart in fetuses with hypoplastic left heart syndrome (HLHS) and evaluate their right ventricular(RV) function during the fetal stages. Methods Twenty-five fetuses initially diagnosed with HLHS were included and second echocardiography detection was performed. Twelve necropsy heart specimens were analyzed for pathological changes. The results of second prenatal echocardiographic imaging were compared with pathological examination. Results Prenatal echocardiography displayed the typical characteristics of HLHS as a severe underdevelopment of the left ventricle(LV) in the form of mitral stenosis or atresia and/or form of aortic atresia or stenosis, with a decreased ratio of aortic diameter to pulmonary artery diameter (P 0.05). The results of pathological anatomy were in agreement with the results of echocardiography. A wide range of muscle fiber disorder and fibrosis were detected in the left ventricular myocardium and aortic wall. Conclusions In addition to severe anatomic abnormalities, the HLHS fetus also has clear histological abnormalities of the LV myocardium and aortic wall, as well as the potential of the RV dysfunction. Key words: Echocardiography; Fetus; Hypoplastic left heart syndrome