Outcomes After Initial Unifocalization to a Shunt in Complex Tetralogy of Fallot With MAPCAs

Ann Thorac Surg. 2019 Jun;107(6):1807-1815. doi: 10.1016/j.athoracsur.2019.01.030. Epub 2019 Feb 14.


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.

MeSH terms

  • Abnormalities, Multiple / surgery*
  • Adolescent
  • Aorta, Thoracic / abnormalities*
  • Aorta, Thoracic / surgery*
  • Cardiac Surgical Procedures / methods
  • Child
  • Child, Preschool
  • Collateral Circulation
  • Female
  • Humans
  • Infant
  • Infant, Newborn
  • Male
  • Pulmonary Artery / abnormalities*
  • Pulmonary Artery / surgery*
  • Retrospective Studies
  • Tetralogy of Fallot / complications
  • Tetralogy of Fallot / surgery*
  • Treatment Outcome
  • Vascular Surgical Procedures / methods