Objective:Asplenia syndrome associated with total anomalous pulmonary venous connection (TAPVC) has remained a clinical challenge. Surgical relief of pulmonary venous obstruction (PVO) is a difficult clinical problem associated with significant risk of recurrent PVO, which makes difficult to achieve right heart bypass operation. Prevention of this problem remains elusive.
Methods: Between November 2007 and July 2008, 5 patients, including 3 neonates, with asplenia syndrome underwent sutureless pericardial repair of TAPVC. Age at operation ranged from 7 days to 2 years and body weight ranged from 1.9 to 9.5 kg. The sutureless repair was used in 3 naonates as an initial procedure and in 2 infants as a second procedure. Under deep hypothermic circulatory arrest or low flow cardiopulmonary bypass, pulmonary vein was incised as long as possible. Atrial wall was partially resected and anastomosed to the pericardial wall around the incised pulmonary vein.
Results: There were no early deaths. No patients showed recurrence of PVO. There was 1 late death. This patient died from congestive heart failure due to the progression of the common atrioventricular valve insufficiency. One patient underwent bilateral bidirectional Glenn and common atrioventricular valvuloplasty 12 months after the primary sutureless repair. Pulmonary venous confluence was confirmed to be left open at the time of Glenn surgery.
Conclusions: The sutureless technique may be useful not only for postrepair PVO but also for non-operated TAPVC in neonates with asplenia syndrome.