Abstract 18443: Programmatic Approach to Management of Tetralogy of Fallot (TOF) With Major Aortopulmonary Collateral Arteries (MAPCAs)

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Introduction: TOF with MAPCAs is a complex and heterogeneous disease with varying degrees of severity. Since 1992, our surgical team has followed a programmatic approach to reach complete repair with the incorporation of all pulmonary vascular supply with low pulmonary artery and RV pressures. We describe and analyze our 15-year surgical experience with TOF and MAPCAs, during which time 458 patients were treated.

Methods: We performed a retrospective review of all patients undergoing surgical intervention for MAPCAs at LPCH since January of 2001. The study included only patients with TOF with pulmonary atresia or stenosis. Follow-up was conducted by a combination of medical and national death record review and contact with primary referring physicians.

Results: A total of 402 patients (87.7%) achieved complete unifocalization and repair. The median RV:AO pressure early after complete repair was 0.36 (0.31-0.43). Patients were followed for a median of 1.9 years (0.2 - 7.4 years) after the first LPCH surgery, with a total of 1798 patient-years of follow-up available. At time of follow up, 88% of all patients were alive. Survival over time was better for patients whose first LPCH surgery was complete repair (HR 0.46 [95% CI 0.26-0.81], p=0.007) than those who initially underwent palliation or revision. Patients with Alagille syndrome also had a higher risk of mortality than those without chromosomal anomalies (HR 3.9 [95% CI 1.6-9.5], p=0.003). On multivariable analysis, both initial surgery type and chromosomal abnormalities were associated with worse survival: unifocalization to shunt (HR 2.5 [95% CI 1.1-5.7], p=0.023), chromosome 22q11 deletion (2.8 [1.2-6.9], p=0.022), and Alagille syndrome or other chromosomal anomaly (6.2 [2.0-19], p=0.001). Early post-repair RV:AO pressure ratio was not associated with duration of postoperative hospitalization or ICU stay, but a RV:AO pressure ratio >0.35 was associated with worse survival over time.

Discussion: Using an approach that emphasizes early complete unifocalization and repair with incorporation of all lung segments and extensive lobar and segmental surgical reconstruction, we have achieved excellent results. Patients with chromosomal anomalies and a RV:AO ratio >0.35 experience worse outcomes.

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