Jerome Soquet1, Matthew Liava'a1, Lucas Eastaugh2, Igor E Konstantinov3, Johann Brink1, Christian P Brizard3, Yves d'Udekem4. 1. Department of Cardiac Surgery, the Royal Children's Hospital, Parkville, Victoria, Australia. 2. Department of Cardiology, the Royal Children's Hospital, Parkville, Victoria, Australia; Murdoch Childrens Research Institute, Parkville, Victoria, Australia. 3. Department of Cardiac Surgery, the Royal Children's Hospital, Parkville, Victoria, Australia; Department of Pediatrics, University of Melbourne, Parkville, Victoria, Australia; Murdoch Childrens Research Institute, Parkville, Victoria, Australia. 4. Department of Cardiac Surgery, the Royal Children's Hospital, Parkville, Victoria, Australia; Department of Pediatrics, University of Melbourne, Parkville, Victoria, Australia; Murdoch Childrens Research Institute, Parkville, Victoria, Australia. Electronic address: yves.dudekem@rch.org.au.
Abstract
BACKGROUND: A strategy of rehabilitation for pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) comprises repetitive shunting and patching procedures of the central pulmonary arteries. We wanted to determine the feasibility and limitations of a strategy of rehabilitation. METHODS: The outcomes of 37 consecutive patients operated from June 2003 to December 2014 for PA/VSD/MAPCAs were reviewed. The patients were directed to a rehabilitation strategy, except when they presented in heart failure with very large collaterals. RESULTS: Four patients with very large MAPCAs underwent a one-stage repair with unifocalization of collateral vessels at a median age of 8.6 months. There was no mortality in this group after a median follow-up of 4.6 years. Following a strategy of staged rehabilitation, 33 patients had 2.01 ± 0.9 procedures before repair. Median age at primary shunting was 3.3 weeks (0.4 to 31.9 weeks). Repair rate was 73% (22 patients), at a median age of 1.7 years. Three patients (10%) were left palliated and 3 patients (10%) died. Median follow-up in this group was 4.5 years. Complementary procedures to the rehabilitation strategy consisted in pulmonary artery reconstruction in 25 patients (76%) and MAPCAs ligation in 7 patients (21%). Pulmonary balloon angioplasty was required in 12 patients (36%) and MAPCAs coil occlusion in 8 patients (24%). CONCLUSIONS: A strategy of rehabilitation can be implemented in almost 90% of the cases, with a low mortality rate. Following this strategy, 73% of the patients can be successfully repaired.
BACKGROUND: A strategy of rehabilitation for pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) comprises repetitive shunting and patching procedures of the central pulmonary arteries. We wanted to determine the feasibility and limitations of a strategy of rehabilitation. METHODS: The outcomes of 37 consecutive patients operated from June 2003 to December 2014 for PA/VSD/MAPCAs were reviewed. The patients were directed to a rehabilitation strategy, except when they presented in heart failure with very large collaterals. RESULTS: Four patients with very large MAPCAs underwent a one-stage repair with unifocalization of collateral vessels at a median age of 8.6 months. There was no mortality in this group after a median follow-up of 4.6 years. Following a strategy of staged rehabilitation, 33 patients had 2.01 ± 0.9 procedures before repair. Median age at primary shunting was 3.3 weeks (0.4 to 31.9 weeks). Repair rate was 73% (22 patients), at a median age of 1.7 years. Three patients (10%) were left palliated and 3 patients (10%) died. Median follow-up in this group was 4.5 years. Complementary procedures to the rehabilitation strategy consisted in pulmonary artery reconstruction in 25 patients (76%) and MAPCAs ligation in 7 patients (21%). Pulmonary balloon angioplasty was required in 12 patients (36%) and MAPCAs coil occlusion in 8 patients (24%). CONCLUSIONS: A strategy of rehabilitation can be implemented in almost 90% of the cases, with a low mortality rate. Following this strategy, 73% of the patients can be successfully repaired.
Authors: Peter D Winch; Dmitry Tumin; Jeffrey Moore; Samantha J Vizzini; Darren P Berman; Aymen N Naguib Journal: Pediatr Cardiol Date: 2018-05-09 Impact factor: 1.655
Authors: Pieter van de Woestijne; Mostafa Mokhles; Ingrid van Beynum; Peter de Jong; Jeroen Wilschut; Ad Bogers Journal: J Card Surg Date: 2022-02-09 Impact factor: 1.778