Repair of Partial Anomalous Pulmonary Venous Connection: A Rare Case Evaluated by Multi-Slice Computed Tomographic...

Partial anomalous pulmonary venous connection (PAPVC) is an uncommon congenital heart disease, which may be difficult to identify and often remains undiagnosed. Accurate diagnosis of major aortopulmonary collaterals and partial anomalous pulmonary venous drainage in patients with congenital heart disease is important but problematic. The goal of this publication is to present the diagnosis and surgical repair of this rare pathology in an eight-year-old boy. Atrial septal defect was found by echocardiography, but no anomalous pulmonary vein was found. However, multi-slice computed tomographic angiography (MSCTA) revealed that the isolated right superior pulmonary vein was replaced by right superior pulmonary vein 1 (RSPV1), right superior pulmonary vein 2 (RSPV2) and right superior pulmonary vein 3 (RSPV3), which connected to the superior vena cava (SVC), the orifice of SVC, and the left atrium, respectively. The patient underwent the repair of PAPVC with division of the SVC and re-implantation on the right atrial appendage to restore normal systemic venous drainage. Postoperative course was uneventful. In conclusion, PAPVC is a rare congenital cardiac pathology. MSCTA could contribute to an accurate anatomic and functional definition of this variant.