Norwood Procedure for Hypoplastic Left Heart With Anomalous Coronary Artery From Pulmonary Trunk

Abstract

Undetected coronary anomalies at Norwood procedure are associated with poor prognosis due to inadequate myocardial protection. We report a case of anomalous origin of the right coronary artery from the main pulmonary artery trunk with hypoplastic left heart syndrome and aortic atresia. Although, during bilateral pulmonary artery banding as initial palliation, the proximity between the right coronary artery origin and the aortic root made a visual diagnosis difficult, it was diagnosed using computed tomography before the Norwood procedure. The Norwood procedure is safe in patients with coronary anomalies after accurate diagnosis to ensure intraoperative myocardial protection.

Figure 1

Preoperative evaluation of the coronary anomaly. (A) Three-dimensional computed tomography of the anomalous right coronary artery (RCA) arising from the right cusp of the pulmonary artery (PA). The left coronary artery (LCA) was continuous from the small ascending aorta (AAo). (B) Preoperative transthoracic echocardiography image of the RCA originating from the PA. (LPA, left pulmonary artery; RPA, right pulmonary artery.)

Figure 2

Surgical schema of cardioplegia administration during the Norwood procedure. Cardioplegia was first injected at the root of the pulmonary artery and the ascending aorta to infuse the left coronary artery and anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA). The second and subsequent infusions were administered at the root of the neoaorta. (BCA, brachiocephalic artery; des.Ao, descending aorta.)