Staged Repair for a Patient with Infracardiac Total Anomalous Pulmonary Venous Connection Complicated by Hypoplastic Left Heart Complex / 日本心臓血管外科学会雑誌

ABSTRACT

Total anomalous pulmonary venous connection (TAPVC) is rarely associated with remarkably small left heart structures. In these types of cases, the hemodynamics resembles that of hypoplastic left heart syndrome, and the treatment strategy is controversial. We present the case of a 1-day-old girl with infracardiac TAPVC, small left heart structures (hypoplastic left heart complex), bilateral superior <i>vena cava</i>, and aberrant origin of the right subclavian artery. We performed a semi-emergent first-stage open palliation for repair of TAPVC, because of pulmonary venous obstruction. We concomitantly performed atrial septal defect (ASD) enlargement and bilateral pulmonary artery banding (BPAB). The postoperative course was uneventful and the left heart structures did not grow, so we performed the Norwood procedure and placed a right ventricle-pulmonary artery shunt with a 5.0 mm artificial graft. Subsequently, the left heart structures were not suitable for biventricular repair, so we chose univentricular repair. The patient underwent a bilateral bidirectional Glenn operation and Fontan completion at 6 and 23 months of age, respectively. TAPVC repair, BPAB, and ASD enlargement are reasonable surgical options for a patient with borderline small left heart structures and TAPVC, as they enable us to wait for growth in the left heart structures and to determine whether univentricular or biventricular repair is suitable.