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  • Title: Outcomes After Initial Unifocalization to a Shunt in Complex Tetralogy of Fallot With MAPCAs.
    Author: Bauser-Heaton H, Ma M, Wise-Faberowski L, Asija R, Shek J, Zhang Y, Peng LF, Sidell DR, Hanley FL, McElhinney DB.
    Journal: Ann Thorac Surg; 2019 Jun; 107(6):1807-1815. PubMed ID: 30772338.
    Abstract:
    BACKGROUND: Our approach to tetralogy of Fallot (TOF) with pulmonary atresia and major aortopulmonary collateral arteries (MAPCAs) emphasizes early single-stage unifocalization and intracardiac repair. However, a subset of patients with small native pulmonary arteries (PAs) and MAPCAs undergo unifocalization to a shunt rather than simultaneous intracardiac repair. METHODS: Patients with TOF/MAPCAs who underwent complete unifocalization to a systemic-to-PA shunt by a single surgeon were reviewed. The decision to perform simultaneous intracardiac repair was based on an intraoperative flow study or empirical assessment of PA and MAPCA size. RESULTS: From November 2001 to December 2017, 57 patients with TOF/MAPCAs underwent unifocalization to a shunt at a median age of 6.9 months. Genetic abnormalities were documented in 60% of patients, including a chromosome 22q11 deletion in 25 and Alagille syndrome in 8. Twenty patients (35%) had undergone prior surgery elsewhere (n = 16) and/or at our center (n = 7). During a median follow-up of 5.4 years, 9 patients had additional surgery to revise the PA reconstruction before complete repair, and 38 patients underwent complete repair. Five years after unifocalization, survival was 74% ± 6%. At follow-up, the median PA to aortic systolic pressure ratio was 0.36 and was greater than 0.50 in 2 patients. CONCLUSIONS: In patients with the smallest MAPCAs and PAs, single-stage unifocalization to a shunt followed by intracardiac repair yields an excellent outcome in most cases.
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