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Abstract

All had severe right ventricular hypoplasia. Ten of the SVP pts (59%) were diagnosed with RVDCC (3 with ostial atresia), and 7 (41%) with non-RVDCC (5 with normal coronary anatomy and 2 with stenosis of one coronary artery). Sinusoids were present in all RVDCC and in 4 out of 7 non-RVDCC pts. Median follow up time was 8.2 years (0 months to 11.3 years). One patient in each group was lost to follow up. Five RVDCC pts died and 1 underwent transplant due to ischemia in the first 6 months of life. Four of the deaths, including the 3 pts with ostial atresia, occurred before discharge from first hospitalization. No deaths occurred after second stage palliation. Three of the 4 surviving RVDCC pts having undergone Fontan, although 2 demonstrated signs of cardiac ischemia (one by PET scan and another by ischemic cardiac arrest during diagnostic catheterization). No deaths or ischemic events occurred in non-RVDCC pts, 5 of whom have undergone completion of Fontan. Kaplan-Meier analysis demonstrated significantly better survival in non-RVDCC vs RVDCC pts (p=0.013). Conclusions: In pts with PA/IVS undergoing SVP, RVDCC is associated with high early in-hospital mortality. There should be early evaluation for cardiac transplant in pts with RVDCC. Mechanical circulatory support, as a bridge to transplant, should be considered in pts with signs of ischemia and heart failure. Non-RVDCC pts, including those with stenosis of a single coronary artery, have excellent long-term outcome in this series.