Borderline hypoplastic left heart malformations: Norwood palliation or two-ventricle repair?

Background: Patients with hypoplastic left heart syndrome (HLHS) and associated malformations undergo Norwood palliation or potentially a two-ventricle repair. Methods: Since 8/99, 8 patients with typical HLHS and two with DILV underwent Norwood/Fontan palliation (group 1). Three other patients (group II) had two-ventricle repair. Anatomy was: aortic atresia, coarctation, unrestrictive VSD (patient 1), hypoplastic mitral and aortic valve, arch and LV, coarctation (patients 2 and 3). Surgical procedures were Norwood arch reconstruction with either Rastelli operation (patient 1) or ASD-closure (patients 2 and 3). Results: Operative mortality in group 1 was 1/8 (day 22; RV-failure). Two patients died before Glenn (sepsis, RV-failure). Six patients underwent Glenn procedure successfully. No patient died in group II. Echocardiography after 13 +/- 7.4 months showed mild homograft dysfunction (patient 1) and an LVOT-gradient of 20mmHg (patient 3). Clinical condition of all survivors in both groups is good. Conclusion: Some anatomical subsets of HLHS with borderline mitral valves and small left ventricles may undergo two ventricle repair despite severe LVOTO. Mortality and morbidity seem to be lower, but selection criteria are so far not defined.