Aortopathy in an Adult With Tricuspid Atresia and Left Ventricular Non-Compaction After Fontan Procedure

Abstract

an association with tricuspid atresia (TA), however, and of successful Fontan procedure for LVNC and TA, are scarce. 2 In contrast, a new concept of "aortopathy" in which aortic dilation, aneurysms, dissections, and/or aortic A lthough left ventricular non-compaction (LVNC) was originally described as an isolated cardiomyopathy, LVNC has been observed in association with several congenital heart diseases (CHD). IMAGES IN CARDIOVASCULAR MEDICINE

Aortic root replacement in a patient with unoperated tricuspid atresia

“…6 Aortic root dilatation, decreased aortic elasticity, and secondary aortic regurgitation impair systemic ventricular function. Although some cases about surgical interventions for aortic valve regurgitation with a Fontan circulation were reported, 7 to our knowledge, our case was the first report describing a surgical procedure for aortic valve regurgitation with unoperated univentricular heart. Since it was difficult to estimate what degree the pulmonary blood flow would be relevant to heart failure in this patient, concomitant pulmonary artery banding was planned when recovery of cardiac function was inadequate.…”