Outcome after surgery for pulmonary atresia with ventricular septal defect, a long‐term follow‐up study

AIM: To study the long‐term outcome after surgery for pulmonary atresia and ventricular septal defect (PA‐VSD), and to determine association between the contribution of major aorto‐pulmonary collateral arteries (MAPCAs) to the pulmonary blood flow, comorbidity and cause of death. METHODS: Patients w...

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Autores principales: Wiezell, Erik, F. Gudnason, Janus, Synnergren, Mats, Sunnegårdh, Jan
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8248001/
https://www.ncbi.nlm.nih.gov/pubmed/33351279
http://dx.doi.org/10.1111/apa.15732
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author Wiezell, Erik
F. Gudnason, Janus
Synnergren, Mats
Sunnegårdh, Jan
author_facet Wiezell, Erik
F. Gudnason, Janus
Synnergren, Mats
Sunnegårdh, Jan
author_sort Wiezell, Erik
collection PubMed
description AIM: To study the long‐term outcome after surgery for pulmonary atresia and ventricular septal defect (PA‐VSD), and to determine association between the contribution of major aorto‐pulmonary collateral arteries (MAPCAs) to the pulmonary blood flow, comorbidity and cause of death. METHODS: Patients who had undergone surgery for PA‐VSD from January 1st 1994 to December 31st 2017 were studied retrospectively. Survival was cross‐checked against the Swedish National Population Register. RESULTS: Seventy patients were identified, giving an incidence of 5.3 newborns per 100 000 live births. In 41 patients (59%) the pulmonary blood flow originated from a patent ductus arteriosus (PDA), while 29 patients (41%) had contribution of the pulmonary blood flow from MAPCAs. Extracardiac disease was found in 34 patients (49%), 16 of whom had 22q11‐microdeletion syndrome (23%). Survival at follow‐up was similar in patients with and without MAPCAs (72.4% vs. 75.6%, n.s.), with a median follow‐up time of 14.3 years (3.2–41.8 years). No difference was found in mortality in patients with or without any syndrome or extracardiac disease. CONCLUSION: Long‐term survival did not differ between those with and without MAPCAs and no difference in mortality was seen in patients with and without concomitant extracardiac disease or any kind of syndrome.
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spelling pubmed-82480012021-07-02 Outcome after surgery for pulmonary atresia with ventricular septal defect, a long‐term follow‐up study Wiezell, Erik F. Gudnason, Janus Synnergren, Mats Sunnegårdh, Jan Acta Paediatr Regular Articles & Brief Reports AIM: To study the long‐term outcome after surgery for pulmonary atresia and ventricular septal defect (PA‐VSD), and to determine association between the contribution of major aorto‐pulmonary collateral arteries (MAPCAs) to the pulmonary blood flow, comorbidity and cause of death. METHODS: Patients who had undergone surgery for PA‐VSD from January 1st 1994 to December 31st 2017 were studied retrospectively. Survival was cross‐checked against the Swedish National Population Register. RESULTS: Seventy patients were identified, giving an incidence of 5.3 newborns per 100 000 live births. In 41 patients (59%) the pulmonary blood flow originated from a patent ductus arteriosus (PDA), while 29 patients (41%) had contribution of the pulmonary blood flow from MAPCAs. Extracardiac disease was found in 34 patients (49%), 16 of whom had 22q11‐microdeletion syndrome (23%). Survival at follow‐up was similar in patients with and without MAPCAs (72.4% vs. 75.6%, n.s.), with a median follow‐up time of 14.3 years (3.2–41.8 years). No difference was found in mortality in patients with or without any syndrome or extracardiac disease. CONCLUSION: Long‐term survival did not differ between those with and without MAPCAs and no difference in mortality was seen in patients with and without concomitant extracardiac disease or any kind of syndrome. John Wiley and Sons Inc. 2021-01-06 2021-05 /pmc/articles/PMC8248001/ /pubmed/33351279 http://dx.doi.org/10.1111/apa.15732 Text en © 2020 The Authors. Acta Paediatrica published by John Wiley & Sons Ltd on behalf of Foundation Acta Paediatrica. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc-nd/4.0/ (https://creativecommons.org/licenses/by-nc-nd/4.0/) License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made.
spellingShingle Regular Articles & Brief Reports
Wiezell, Erik
F. Gudnason, Janus
Synnergren, Mats
Sunnegårdh, Jan
Outcome after surgery for pulmonary atresia with ventricular septal defect, a long‐term follow‐up study
title Outcome after surgery for pulmonary atresia with ventricular septal defect, a long‐term follow‐up study
title_full Outcome after surgery for pulmonary atresia with ventricular septal defect, a long‐term follow‐up study
title_fullStr Outcome after surgery for pulmonary atresia with ventricular septal defect, a long‐term follow‐up study
title_full_unstemmed Outcome after surgery for pulmonary atresia with ventricular septal defect, a long‐term follow‐up study
title_short Outcome after surgery for pulmonary atresia with ventricular septal defect, a long‐term follow‐up study
title_sort outcome after surgery for pulmonary atresia with ventricular septal defect, a long‐term follow‐up study
topic Regular Articles & Brief Reports
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8248001/
https://www.ncbi.nlm.nih.gov/pubmed/33351279
http://dx.doi.org/10.1111/apa.15732
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