Long-term outcome after treatment of pulmonary atresia with ventricular septal defect : nationwide study of 109 patients born in 1970-2007

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Kaskinen , A K , Happonen , J-M , Mattila , I P & Pitkanen , O M 2016 , ' Long-term outcome after treatment of pulmonary atresia with ventricular septal defect : nationwide study of 109 patients born in 1970-2007 ' , European Journal of Cardio-Thoracic Surgery , vol. 49 , no. 5 , pp. 1411-1418 . https://doi.org/10.1093/ejcts/ezv404

Title: Long-term outcome after treatment of pulmonary atresia with ventricular septal defect : nationwide study of 109 patients born in 1970-2007
Author: Kaskinen, Anu K.; Happonen, Juha-Matti; Mattila, Ilkka P.; Pitkanen, Olli M.
Contributor: University of Helsinki, Clinicum
University of Helsinki, Children's Hospital
University of Helsinki, Clinicum
University of Helsinki, Clinicum
Date: 2016-05
Language: eng
Number of pages: 8
Belongs to series: European Journal of Cardio-Thoracic Surgery
ISSN: 1010-7940
URI: http://hdl.handle.net/10138/165016
Abstract: OBJECTIVES: Treatment of pulmonary atresia with ventricular septal defect (PA + VSD) has evolved during recent decades, but it still remains challenging. This study evaluated 41-year experience of outcome, survival and treatment of PA + VSD patients. METHODS: Patient records and angiograms of 109 patients with PA + VSD born in Finland between 1970 and 2007, and treated at the Children's Hospital, Helsinki University Central Hospital, were retrospectively analysed in this nationwide study. RESULTS: Of the 109 patients, 66 (61%) had simple PA + VSD without major aortopulmonary collateral arteries (MAPCAs). Although we observed no difference in overall survival between those with or without MAPCAs, the patients without MAPCAs had better probability to achieve repair (64 vs 28%, P <0.0003). Only 3 patients were treated by compassionate care. Overall survival was affected by the size of true central pulmonary arteries on the first angiogram (P = 0.001) and whether repair was achieved (P <0.0001). After successful repair, the survival rate was 93% at 1 year, 91% from the second year, and functional capacity as assessed by New York Heart Association (NYHA) I-II remained in 85% of patients alive at the end of follow-up. Palliated patients at 1, 5, 10 and 20 years of age had Kaplan-Meier estimated survival rates of 55, 42, 34 and 20%, respectively. Patients who underwent repair attempts but were left palliated with right ventricle (RV)-pulmonary artery connection and septal fenestration had better survival than the rest of the palliated patients (P = 0.001). Further, the McGoon index improved after implementation of a systemic-pulmonary artery shunt in the overall PA + VSD population (P <0.0001). CONCLUSIONS: These findings show that achievement of repair and initial size of true central pulmonary arteries affect survival of patients with PA + VSD. Although the overall survival of patients with MAPCAs showed no difference compared with simple PA + VSD patients, they had a higher risk of remaining palliated. However, palliative surgery may have a role in treatment of PA + VSD because the size of pulmonary arteries increased after placement of systemic-pulmonary artery shunt. In addition, subtotal repair by a RV-pulmonary artery connection and septal fenestration improved survival over extracardiac palliation.
Subject: Congenital heart defect
Incidence
McGoon index
Mortality
Pulmonary blood supply
Late results
AORTOPULMONARY COLLATERAL ARTERIES
STAGED REPAIR
UNIFOCALIZATION
EXPERIENCE
TETRALOGY
SURVIVAL
CHILDREN
FALLOT
SHUNT
3121 General medicine, internal medicine and other clinical medicine
3123 Gynaecology and paediatrics
3126 Surgery, anesthesiology, intensive care, radiology
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