Repair of Untreated Older Patients With Tetralogy of Fallot With Major Aortopulmonary Collaterals. 2019

Sumeet S Vaikunth, and Holly Bauser-Heaton, and George K Lui, and Lisa Wise-Faberowski, and Frandics P Chan, and Ritu Asija, and Frank L Hanley, and Doff B McElhinney
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.

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. Any patient with this lesion not treated before 2 years of age referred to our center from 2002 to 2017 met inclusion criteria. 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. 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.

UI MeSH Term Description Entries
D007223 Infant A child between 1 and 23 months of age. Infants
D008297 Male Males
D011379 Prognosis A prediction of the probable outcome of a disease based on a individual's condition and the usual course of the disease as seen in similar situations. Prognostic Factor,Prognostic Factors,Factor, Prognostic,Factors, Prognostic,Prognoses
D011652 Pulmonary Circulation The circulation of the BLOOD through the LUNGS. Pulmonary Blood Flow,Respiratory Circulation,Circulation, Pulmonary,Circulation, Respiratory,Blood Flow, Pulmonary,Flow, Pulmonary Blood,Pulmonary Blood Flows
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D003097 Collateral Circulation Maintenance of blood flow to an organ despite obstruction of a principal vessel. Blood flow is maintained through small vessels. Blood Circulation, Collateral,Circulation, Collateral,Collateral Blood Circulation,Collateral Circulation, Blood,Blood Collateral Circulation,Circulation, Blood Collateral,Circulation, Collateral Blood,Collateral Blood Circulations,Collateral Circulations,Collateral Circulations, Blood
D005260 Female Females
D006348 Cardiac Surgical Procedures Surgery performed on the heart. Cardiac Surgical Procedure,Heart Surgical Procedure,Heart Surgical Procedures,Procedure, Cardiac Surgical,Procedure, Heart Surgical,Procedures, Cardiac Surgical,Procedures, Heart Surgical,Surgical Procedure, Cardiac,Surgical Procedure, Heart,Surgical Procedures, Cardiac,Surgical Procedures, Heart
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults

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