Importance of multidisciplinary management for pulmonary atresia, ventricular septal defect, major aorto-pulmonary collateral arteries and completely absent central pulmonary arteries

Background To review long-term clinical outcomes for pulmonary atresia, ventricular septal defect (PA/VSD), major aorto-pulmonary collateral arteries (MAPCAs) and completely absent central pulmonary arteries (cPAs). Methods Of all 120 surgically treated patients with PA/VSD, MAPCAs between 1981 and 2011, 15 patients (12.5%) with completely absent cPAs were enrolled. The median age at initial surgery was 3.8 ± 4.9 years old (range 0.1–17.6) and 3.5 (2–6). Since 2003, consecutive five patients have been treated by current treatment strategy consisting of staged midline approach with percutaneous trans-catheter angioplasties (PTA), where complete unifocalization and right ventricle (RV)—PA conduit placement was performed via median sternotomy at first, then which was followed by VSD closure with one-way fenestrated patch and conduit replacement, after PTA for unifocalized MAPCAs, reconstructed cPAs, and RV-PA conduit. For patients survived after definitive repair, PTA was aggressively repeated to maintain RV systolic pressure. Results Median follow-up period was 7.8 years (0.3–21.7). Actuarial survival rate at 20 years after the initial surgical intervention was 27.5%, and all five patients treated by current treatment strategy survived to date. Of all 10 survivors after the definitive repair, serial catheter examination revealed that right to left ventricular systolic pressure ratio was maintained from 0.59 ± 0.09 (0.45–0.73) at early after the definitive repair to 0.57 ± 0.14 (0.35–0.81) at latest follow-up. Conclusions Staged midline approach with PTA seemed feasible strategy to go on to definitive repair. Patency of MAPCAs and reconstructed cPAs were maintained by the aggressive PTA after the definitive repair.