A Case of Hypoplastic Left Heart Syndrome Diagnosed by Fetal Echocardiography

Fetal echocardiograpy can now be used to detect congenital heart disease before birth, and increased experience with the four-chamber view has led a greater number of fetuses referred to pediatric cardiologists for confirmation or exclusion of a cardiac lesion. Hypoplastic Ieft heart syndrome includes hypoplasia of the ascending aorta, aortic valve atresia or severe stenosis, a small left ventricle, and mitral atresia or stenosis. Without surgical intervention, the condition is fatal and around 95% of newborns die in the first month of life. A 23-week old fetus was referred for echocardiography with a suspicion of congenital heart disease and was diagnosed to have hypoplastic left heart disease (atretic aortic valve, small left ventricle, hypoplastic ascending aorta, mild hypoplasia of the mitral valve) and a large trabecular ventricular septal defect, Termination was undertaken at 24 weeks gestation and postmortem examination confirmed the echocardiographic findings. The details of the fetal echocardiographic and postmortem macroscopic findings are presented in this report. Since long-term results of staged palliation remain disappointing, families tend to opt for the challenging decision of terminatin the pregnancy.