日本小児循環器学会雑誌 Pediatric Cardiology and Cardiac Surgery

Objective: Between 2004 and 2011 in our institution, we performed the Norwood operation (NW) with bidirectional Glenn shunt (BDG) for patients with hypoplastic left heart syndrome (HLHS) at 3 months old following bilateral pulmonary artery banding (BPAB). Since 2012, we have been performsaing NW with a right ventricular-pulmonary shunt (RV–PA shunt) 1 month after BPAB in HLHS patients. The aim of this study was to evaluate the efficacy of a rapid two-stage NW operation.

Methods: Between January 2004 and December 2013, a total of 28 HLHS patients underwent NW. NW with BDG was performed in 22 patients (Group G) and NW with an RV–PA shunt was performed in 6 patients (Group S). Surgical results were compared between the groups.

Results: In Group G, 8 patients (36.3%) required pulmonary artery plasty and 20 patients (91.0%) underwent catheter intervention for pulmonary artery (PTPA). Postoperative complications comprised 3 in-hospital deaths, 2 BDG take-downs, and 1 cerebrovascular infarction. In Group S, no patients required either pulmonary artery plasty or PTPA, and BDG was performed at a median age of 7 months (range, 6–13 months). Postoperative pressure in the superior vena cava (SVC) was significantly lower in Group S.

Conclusions: NW with an RV–PA shunt 1 month after BPAB enabled avoidance of PTPA and maintenance of a low SVC pressure following BDG, and was thought to improve surgical outcomes.