Pulmonary atresia/intact ventricular septum: influence of coronary anatomy on single-ventricle outcome.

BACKGROUND

We investigated the influence of coronary artery abnormalities on outcome in patients with pulmonary atresia/intact ventricular septum (PA-IVS) for planned single-ventricle palliation.

METHODS

Catheterization and medical records were reviewed in patients with PA-IVS for planned single-ventricle palliation at our institution between 2000 and 2012. Primary outcome was death or transplantation. Patients with confirmed or strong suspicion of stenosis in 2 or more main coronary arteries or coronary ostial atresia were defined as having right ventricle-dependent coronary circulation (RVDCC); those with stenosis of 1 main vessel or normal anatomy were defined as having non-RVDCC.

RESULTS

Of 58 patients with PA-IVS, 17 (30%) underwent single-ventricle palliation. Ten (59%) had RVDCC (3 with ostial atresia) and 7 (41%) had non-RVDCC. Median follow-up time was 8.2 years (0 months-11.3 years), with 1 patient in each group lost to follow-up. Five patients with RVDCC died, including the 3 patients with ostial atresia, and 1 underwent transplantation at 6 months of life. No deaths occurred after second-stage palliation. Three of the 4 surviving patients with RVDCC completed a Fontan operation, and 2 of these patients had evidence of cardiac ischemia on follow-up. No deaths occurred among patients with non-RVDCC. Kaplan-Meier analysis demonstrated significantly better survival in patients with non-RVDCC (100%) than in patients with RVDCC (40%) (p = 0.026).

CONCLUSIONS

In patients with PA-IVS undergoing single-ventricle palliation, RVDCC is associated with high early mortality, especially with coronary ostial atresia. There should be early consideration of transplantation in neonates with RVDCC. Patients with non-RVDCC undergoing single-ventricle palliation have excellent long-term outcomes, with no mortality seen in this series.