Outcome of patients with cyanotic congenital heart disease undergoing a second systemic-to-pulmonary artery shunt

Background. Multiple systemic-to-pulmonary artery shunts often represent a deviation from the initial management strategy for cyanotic congenital hear t disease. This study analysed the outcome in patients undergoing a second shunt. Methods. Between 1965 and 1998, 80 patients required a second shunt. Patien t age ranged from 11 days to 11.2 years at the initial shunt, and 4 months to 25.6 years at the second shunt. The interval between shunts ranged from 11 days to 19.6 years. Results. The patient survival rates at 1, 6, 12, 60 and 120 months followin g the second shunt were 95.0+/-2.4%, 92.5+/-3.0%, 87.4+/-3.7%, 82.4+/-4.5% and 74.8+/-5.8%, respectively. Although excess pulmonary now was not encoun tered after the second shunt, mode and incidence of morbidity were otherwis e similar to the first one. Between 9 months and 7.5 years after the second shunt, 24 patients underwent successful definitive repair. Multivariable a nalysis identified pulmonary atresia (p=0.027) and a short (<1 year) interv al between the two shunts (p=0.034) as the independent risk factors for lon g-term shunt failure. Single ventricle physiology (p=0.002) and a central a pproach for the second shunt (p=0.016) were independent risk factors for la ck of application, or failure of intracardiac definitive repair. Conclusions. A significant limitation in longevity and quality of life is c ommon in patients requiring a second shunt, especially those associated wit h pulmonary atresia, the single ventricle physiology heart, or ungraftably hypoplastic contralateral branch pulmonary artery to the first shunt. Since excess pulmonary blood flow leading to congenital heart failure and/or ven tricular diastolic dysfunction is unlikely, all patients who preclude defin itive repair due to decreased pulmonary blood flow even after the first shu nt should be shunted again.