Holly Bauser-Heaton1, Alejandro Borquez1, Brian Han1, Michael Ladd1, Ritu Asija1, Laura Downey1, Andrew Koth1, Claudia A Algaze1, Lisa Wise-Faberowski1, Stanton B Perry1, Andrew Shin1, Lynn F Peng1, Frank L Hanley1, Doff B McElhinney2. 1. From the Departments of Pediatrics (H.B.-H., A.B., B.H., M.L., R.A., A.K., C.A.A., S.B.P., A.S., L.F.P., D.B.M.), Anesthesia (L.D., L.W.-F.), and Cardiothoracic Surgery (F.L.H., D.B.M.), Lucile Packard Children's Hospital Heart Center Clinical and Translational Research Program, Stanford University School of Medicine, Palo Alto, CA. 2. From the Departments of Pediatrics (H.B.-H., A.B., B.H., M.L., R.A., A.K., C.A.A., S.B.P., A.S., L.F.P., D.B.M.), Anesthesia (L.D., L.W.-F.), and Cardiothoracic Surgery (F.L.H., D.B.M.), Lucile Packard Children's Hospital Heart Center Clinical and Translational Research Program, Stanford University School of Medicine, Palo Alto, CA. doff@stanford.edu.
Abstract
BACKGROUND: Tetralogy of Fallot with major aortopulmonary collateral arteries is a complex and heterogeneous condition. Our institutional approach to this lesion emphasizes early complete repair with the incorporation of all lung segments and extensive lobar and segmental pulmonary artery reconstruction. METHODS AND RESULTS: We reviewed all patients who underwent surgical intervention for tetralogy of Fallot and major aortopulmonary collateral arteries at Lucile Packard Children's Hospital Stanford (LPCHS) since November 2001. A total of 458 patients underwent surgery, 291 (64%) of whom underwent their initial procedure at LPCHS. Patients were followed for a median of 2.7 years (mean 4.3 years) after the first LPCHS surgery, with an estimated survival of 85% at 5 years after first surgical intervention. Factors associated with worse survival included first LPCHS surgery type other than complete repair and Alagille syndrome. Of the overall cohort, 402 patients achieved complete unifocalization and repair, either as a single-stage procedure (n=186), after initial palliation at our center (n=74), or after surgery elsewhere followed by repair/revision at LPCHS (n=142). The median right ventricle:aortic pressure ratio after repair was 0.35. Estimated survival after repair was 92.5% at 10 years and was shorter in patients with chromosomal anomalies, older age, a greater number of collaterals unifocalized, and higher postrepair right ventricle pressure. CONCLUSIONS: Using an approach that emphasizes early complete unifocalization and repair with incorporation of all pulmonary vascular supply, we have achieved excellent results in patients with both native and previously operated tetralogy of Fallot and major aortopulmonary collateral arteries.
BACKGROUND: Tetralogy of Fallot with major aortopulmonary collateral arteries is a complex and heterogeneous condition. Our institutional approach to this lesion emphasizes early complete repair with the incorporation of all lung segments and extensive lobar and segmental pulmonary artery reconstruction. METHODS AND RESULTS: We reviewed all patients who underwent surgical intervention for tetralogy of Fallot and major aortopulmonary collateral arteries at Lucile Packard Children's Hospital Stanford (LPCHS) since November 2001. A total of 458 patients underwent surgery, 291 (64%) of whom underwent their initial procedure at LPCHS. Patients were followed for a median of 2.7 years (mean 4.3 years) after the first LPCHS surgery, with an estimated survival of 85% at 5 years after first surgical intervention. Factors associated with worse survival included first LPCHS surgery type other than complete repair and Alagille syndrome. Of the overall cohort, 402 patients achieved complete unifocalization and repair, either as a single-stage procedure (n=186), after initial palliation at our center (n=74), or after surgery elsewhere followed by repair/revision at LPCHS (n=142). The median right ventricle:aortic pressure ratio after repair was 0.35. Estimated survival after repair was 92.5% at 10 years and was shorter in patients with chromosomal anomalies, older age, a greater number of collaterals unifocalized, and higher postrepair right ventricle pressure. CONCLUSIONS: Using an approach that emphasizes early complete unifocalization and repair with incorporation of all pulmonary vascular supply, we have achieved excellent results in patients with both native and previously operated tetralogy of Fallot and major aortopulmonary collateral arteries.
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Authors: Martin L Tomov; Lilanni Perez; Liqun Ning; Huang Chen; Bowen Jing; Andrew Mingee; Sahar Ibrahim; Andrea S Theus; Gabriella Kabboul; Katherine Do; Sai Raviteja Bhamidipati; Jordan Fischbach; Kevin McCoy; Byron A Zambrano; Jianyi Zhang; Reza Avazmohammadi; Athanasios Mantalaris; Brooks D Lindsey; David Frakes; Lakshmi Prasad Dasi; Vahid Serpooshan; Holly Bauser-Heaton Journal: Adv Healthc Mater Date: 2021-08-08 Impact factor: 11.092