Sumeet S Vaikunth1, Holly Bauser-Heaton2, George K Lui3, Lisa Wise-Faberowski4, Frandics P Chan5, Ritu Asija2, Frank L Hanley6, Doff B McElhinney7. 1. Department of Pediatrics, Lucile Packard Children's Hospital Stanford, Clinical and Translational Research Program, Stanford University School of Medicine, Palo Alto, California. Electronic address: sumeetv@stanford.edu. 2. Department of Pediatrics, Lucile Packard Children's Hospital Stanford, Clinical and Translational Research Program, Stanford University School of Medicine, Palo Alto, California. 3. Department of Pediatrics, Lucile Packard Children's Hospital Stanford, Clinical and Translational Research Program, Stanford University School of Medicine, Palo Alto, California; Department of Medicine, Lucile Packard Children's Hospital Stanford, Clinical and Translational Research Program, Stanford University School of Medicine, Palo Alto, California. 4. Department of Anesthesiology, Lucile Packard Children's Hospital Stanford, Clinical and Translational Research Program, Stanford University School of Medicine, Palo Alto, California. 5. Department of Radiology, Lucile Packard Children's Hospital Stanford, Clinical and Translational Research Program, Stanford University School of Medicine, Palo Alto, California. 6. Department of Cardiothoracic Surgery, Lucile Packard Children's Hospital Stanford, Clinical and Translational Research Program, Stanford University School of Medicine, Palo Alto, California. 7. Department of Pediatrics, Lucile Packard Children's Hospital Stanford, Clinical and Translational Research Program, Stanford University School of Medicine, Palo Alto, California; Department of Cardiothoracic Surgery, Lucile Packard Children's Hospital Stanford, Clinical and Translational Research Program, Stanford University School of Medicine, Palo Alto, California.
Abstract
BACKGROUND: Our programmatic approach to tetralogy of Fallot with major aortopulmonary collaterals emphasizes single-stage unifocalization with complete intracardiac repair during infancy. Little is known about suitability for complete repair in patients beyond infancy. We sought to analyze outcomes of our approach in older patients with previously untreated tetralogy of Fallot with major aortopulmonary collaterals. METHODS: Any patient with this lesion not treated before 2 years of age referred to our center from 2002 to 2017 met inclusion criteria. RESULTS: Of 33 patients, 32 were out-of-state (64% international) referrals, and 33% (n = 11) were older than 9 years, had polycythemia, or at least 1 high pressure collateral (>25 mm Hg). Complete repair was achieved in 94% (n = 31) of patients, 82% (n = 27) in one stage and 12% (n = 4) after unifocalization to a central shunt. The median right ventricular-to-aortic pressure ratio was 0.31 after the operation and 0.37 at follow-up. At a median of 4.8 years after repair, 9 patients (19%) underwent reintervention, including 5 conduit and 7 branch pulmonary artery interventions. Three patients also underwent aortic valve replacement. CONCLUSIONS: In this selected cohort of older patients with previously unoperated tetralogy of Fallot with major aortopulmonary collaterals, outcomes were comparable with infants undergoing treatment according to our approach. These findings support the notion that patients who are either born in low-resource settings or present to health care providers beyond infancy should be considered candidates and evaluated for complete repair.
BACKGROUND: Our programmatic approach to tetralogy of Fallot with major aortopulmonary collaterals emphasizes single-stage unifocalization with complete intracardiac repair during infancy. Little is known about suitability for complete repair in patients beyond infancy. We sought to analyze outcomes of our approach in older patients with previously untreated tetralogy of Fallot with major aortopulmonary collaterals. METHODS: Any patient with this lesion not treated before 2 years of age referred to our center from 2002 to 2017 met inclusion criteria. RESULTS: Of 33 patients, 32 were out-of-state (64% international) referrals, and 33% (n = 11) were older than 9 years, had polycythemia, or at least 1 high pressure collateral (>25 mm Hg). Complete repair was achieved in 94% (n = 31) of patients, 82% (n = 27) in one stage and 12% (n = 4) after unifocalization to a central shunt. The median right ventricular-to-aortic pressure ratio was 0.31 after the operation and 0.37 at follow-up. At a median of 4.8 years after repair, 9 patients (19%) underwent reintervention, including 5 conduit and 7 branch pulmonary artery interventions. Three patients also underwent aortic valve replacement. CONCLUSIONS: In this selected cohort of older patients with previously unoperated tetralogy of Fallot with major aortopulmonary collaterals, outcomes were comparable with infants undergoing treatment according to our approach. These findings support the notion that patients who are either born in low-resource settings or present to health care providers beyond infancy should be considered candidates and evaluated for complete repair.
Authors: Martin L Tomov; Alexander Cetnar; Katherine Do; Holly Bauser-Heaton; Vahid Serpooshan Journal: J Am Heart Assoc Date: 2019-12-10 Impact factor: 5.501