The neonate with a functional single ventricle (FSV) and aortic arch obstruction or interruption other than hypoplastic left heart syndrome (HLHS) poses a uniquely difficult challenge because of the stimulus for myocardial hypertrophy and failure from impedance to systemic outflow and pulmonary vascular disease from pulmonary overcirculation.
Among the patients with FSV and excessive pulmonary blood flow, who underwent palliative surgery since 1990, 36 neonates had aortic coarctation (n=32) or interruption (n=4). Patients with HLHS were excluded. The median age at operation was 7.5 days (range 2 to 84 days) and the median weight was 3.3 kg (range 2.7 to 5 kg). 28 children have undergone a pulmonary artery banding (PAB) and aortic coarctation or interruption repair (78%), and 8 children (22%;8/36) underwent a modified Norwood procedure.
There were four early deaths and two late deaths after the initial surgical palliation. The overall survival at 10 years was 78%. Follow-up has ranged from 6 months to 13 years (average 5.2 years). Of the 32 survivors, 19 have undergone a bidirectional cavopulmonary anastomosis and 20 a Fontan procedure with no deaths. Twelve infants have required Damus-Kaye-Stansel procedure (DKS) and three infants have required bulboventricular foramen (BVF) enlargement for subaortic obstruction (two early deaths). In all survivors patients who underwent DKS or BVF enlargement (42%;15/36) regular sinus rhythm was maintained postoperatively.
Our experience suggests that this high-risk subgroup of neonates with FSV and aortic arch obstruction or interruption is safely managed by initial palliation procedures (PAB with repair of aortic obstruction or modified Norwood procedure). This strategy, careful surveillance, and early relief of subaortic stenosis can maintain acceptable anatomy and hemodynamics for later bidirectional Glenn and Fontan procedures.
Our experience suggests that this high-risk subgroup of neonates with FSV and aortic arch obstruction or interruption is safely managed by initial palliation procedures (PAB with repair of aortic obstruction or modified Norwood procedure).
Mark Ruzmetov, None.