Holly Bauser-Heaton1, Alejandro Borquez1, Ritu Asija1, Lisa Wise-Faberowski2, Yulin Zhang3, Laura Downey2, Stanton B Perry1, Andrew Koth1, Lynn F Peng1, Claudia A Algaze1, Frank L Hanley3, Doff B McElhinney4. 1. Department of Pediatrics, Clinical and Translational Research Program, Lucile Packard Children's Hospital Heart Center, Stanford University School of Medicine, Palo Alto, Calif. 2. Department of Anesthesia, Clinical and Translational Research Program, Lucile Packard Children's Hospital Heart Center, Stanford University School of Medicine, Palo Alto, Calif. 3. Department of Cardiothoracic Surgery, Clinical and Translational Research Program, Lucile Packard Children's Hospital Heart Center, Stanford University School of Medicine, Palo Alto, Calif. 4. Department of Pediatrics, Clinical and Translational Research Program, Lucile Packard Children's Hospital Heart Center, Stanford University School of Medicine, Palo Alto, Calif; Department of Cardiothoracic Surgery, Clinical and Translational Research Program, Lucile Packard Children's Hospital Heart Center, Stanford University School of Medicine, Palo Alto, Calif. Electronic address: doff@stanford.edu.
Abstract
BACKGROUND: Our institutional approach to tetralogy of Fallot (TOF) with major aortopulmonary collaterals (MAPCAs) emphasizes unifocalization and augmentation of the reconstructed pulmonary arterial (PA) circulation and complete intracardiac repair in infancy, usually in a single procedure. This approach yields a high rate of complete repair with excellent survival and low right ventricular (RV) pressure. However, little is known about remodeling of the unifocalized and reconstructed pulmonary circulation or about reinterventions on the reconstructed PAs or the RV outflow tract conduit. METHODS: We reviewed patients who underwent complete repair of TOF with MAPCAs at our center at <2 years of age, either as a single-stage procedure or after previous procedures. Outcomes included freedom from conduit or PA intervention after repair, which were assessed by Cox regression and Kaplan-Meier analysis. RESULTS: The study cohort included 272 patients. There were 6 early deaths and a median of follow-up of 3.6 years after complete repair. Reinterventions on the pulmonary circulation were performed in 134 patients, including conduit interventions in 101 patients, branch PA interventions in 101, and closure of residual MAPCAs in 9. The first conduit reintervention consisted of surgical conduit replacement in 77 patients, transcatheter pulmonary valve replacement with a Melody valve in 14, and angioplasty or bare metal stenting in 10. Surgical PA reinterventions were performed in 46 patients and transcatheter reinterventions in 75 (both in 20). Most PA reinterventions involved a single lung, and most transcatheter reinterventions a single vessel. Freedom from conduit replacement or transcatheter pulmonary valve replacement was 70 ± 3% at 5 years and was shorter in patients with smaller initial conduit size. Freedom from any PA reintervention was 64 ± 3% at 5 years, with the greatest rate during the first year. On multivariable analysis, factors associated with longer freedom from any PA reintervention included lower postrepair RV:aortic pressure ratio and larger original conduit size. CONCLUSIONS: We were able to obtain follow-up data for the majority of patients, which demonstrated freedom from PA reintervention for two thirds of patients. The time course of and risk factors for conduit reintervention in this cohort appeared similar to previously reported findings in patients who received RV-PA conduits in early childhood for other anomalies. Relative to the severity of baseline pulmonary vascular anatomy in TOF with MAPCAs, reinterventions on the reconstructed PAs were uncommon after repair according to our approach, and major reinterventions were rare. Nevertheless, PA reinterventions are an important aspect of the overall management strategy.
BACKGROUND: Our institutional approach to tetralogy of Fallot (TOF) with major aortopulmonary collaterals (MAPCAs) emphasizes unifocalization and augmentation of the reconstructed pulmonary arterial (PA) circulation and complete intracardiac repair in infancy, usually in a single procedure. This approach yields a high rate of complete repair with excellent survival and low right ventricular (RV) pressure. However, little is known about remodeling of the unifocalized and reconstructed pulmonary circulation or about reinterventions on the reconstructed PAs or the RV outflow tract conduit. METHODS: We reviewed patients who underwent complete repair of TOF with MAPCAs at our center at <2 years of age, either as a single-stage procedure or after previous procedures. Outcomes included freedom from conduit or PA intervention after repair, which were assessed by Cox regression and Kaplan-Meier analysis. RESULTS: The study cohort included 272 patients. There were 6 early deaths and a median of follow-up of 3.6 years after complete repair. Reinterventions on the pulmonary circulation were performed in 134 patients, including conduit interventions in 101 patients, branch PA interventions in 101, and closure of residual MAPCAs in 9. The first conduit reintervention consisted of surgical conduit replacement in 77 patients, transcatheter pulmonary valve replacement with a Melody valve in 14, and angioplasty or bare metal stenting in 10. Surgical PA reinterventions were performed in 46 patients and transcatheter reinterventions in 75 (both in 20). Most PA reinterventions involved a single lung, and most transcatheter reinterventions a single vessel. Freedom from conduit replacement or transcatheter pulmonary valve replacement was 70 ± 3% at 5 years and was shorter in patients with smaller initial conduit size. Freedom from any PA reintervention was 64 ± 3% at 5 years, with the greatest rate during the first year. On multivariable analysis, factors associated with longer freedom from any PA reintervention included lower postrepair RV:aortic pressure ratio and larger original conduit size. CONCLUSIONS: We were able to obtain follow-up data for the majority of patients, which demonstrated freedom from PA reintervention for two thirds of patients. The time course of and risk factors for conduit reintervention in this cohort appeared similar to previously reported findings in patients who received RV-PA conduits in early childhood for other anomalies. Relative to the severity of baseline pulmonary vascular anatomy in TOF with MAPCAs, reinterventions on the reconstructed PAs were uncommon after repair according to our approach, and major reinterventions were rare. Nevertheless, PA reinterventions are an important aspect of the overall management strategy.
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
Authors: Pieter C van de Woestijne; Jamie L R Romeo; Ingrid van Beynum; Maarten Witsenburg; M Mostafa Mokhles; Ad J J C Bogers Journal: JTCVS Open Date: 2021-09-24
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