Case of Hypoplastic Left Heart Syndrome with a Ruptured Sinus of Valsalva Aneurysm in a Fetus

Sinus of valsalva aneurysm (SVA) may be associated with congenital heart disease, and it is an extremely rare condition among children. Moreover, a ruptured SVA has not been observed in a fetus. Herein, we report a case of hypoplastic left heart syndrome (HLHS) with mitral atresia and aortic atresia and a ruptured SVA. HLHS with moderate tricuspid regurgitation was suspected on fetal echocardiography. However, transthoracic echocardiography after birth revealed a continuous flow shunting from the aortic root to the right atrium rather than tricuspid regurgitation. A ruptured SVA was suspected, and countercurrent aortography was performed for further evaluation. Results revealed an SVA of noncoronary cusp with a continuous flow to the right atrium. This finding indicated a ruptured SVA, which can occur even in a fetus. The shunt flow of a ruptured SVA into the right atrium can be misdiagnosed as tricuspid regurgitation on fetal echocardiography. Thus, countercurrent aortography may be a useful modality for diagnosing a ruptured SVA among neonates.