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

Background: Bilateral pulmonary artery banding for patients with Hypoplastic Left Heart Syndrome (HLHS) prevails as the first palliation in Japan, however, concerns remain about the long-term outcomes. Primary Norwood operation, nevertheless, has advantages such as fewer inter-stage palliations. This study’s goal is to review the mid-term results of primary Norwood operations up to the Fontan operations and to analyze risk factors.

Methods: Sixteen consecutive patients with HLHS (male/female: 6/10) were included from October 2004 to May 2014. Of those, fifteen patients underwent the primary Norwood operations. Survival was assessed by the Kaplan–Meier method and compared between groups using the Mantel–Cox log-rank test. A Cox regression model was used for risk factor analysis.

Results: Estimated survival rate was 69％ at one year and 56％ at three years after the Norwood operations. Prior to Fontan operations, palliations were performed in 2.5±0.8 times. Hazard ratio for death of genetic disorder/malformation and heterotaxy was 14.03 (95％CI, 1.17–167.8, p＝0.037) and 18.66 (95％CI, 1.43–244.4, p＝0.026), respectively. Nine patients (9/16＝56％) achieved Fontan operations. All survivors after the Fontan operations except one patient who suffered from protein-losing enteropathy, are in NYHA class 1.

Conclusion: The strategy of primary Norwood operation for patients with HLHS was feasible up to the Fontan operation with fewer inter-stage palliations, except for genetic disorder and heterotaxy. More investigations are required to justify the operational strategy for genetic disorder and heterotaxy patients.