PMID- 26620210 OWN - NLM STAT- MEDLINE DCOM- 20170314 LR - 20170817 IS - 1873-734X (Electronic) IS - 1010-7940 (Linking) VI - 49 IP - 5 DP - 2016 May TI - Long-term outcome after treatment of pulmonary atresia with ventricular septal defect: nationwide study of 109 patients born in 1970-2007. PG - 1411-8 LID - 10.1093/ejcts/ezv404 [doi] AB - 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. CI - (c) The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved. FAU - Kaskinen, Anu K AU - Kaskinen AK AD - Division of Pediatric Cardiology and Cardiac and Transplantation Surgery, Children's Hospital, University of Helsinki and Helsinki University Central Hospital, Helsinki, Finland anu.kaskinen@helsinki.fi. FAU - Happonen, Juha-Matti AU - Happonen JM AD - Division of Pediatric Cardiology and Cardiac and Transplantation Surgery, Children's Hospital, University of Helsinki and Helsinki University Central Hospital, Helsinki, Finland. FAU - Mattila, Ilkka P AU - Mattila IP AD - Division of Pediatric Cardiology and Cardiac and Transplantation Surgery, Children's Hospital, University of Helsinki and Helsinki University Central Hospital, Helsinki, Finland. FAU - Pitkanen, Olli M AU - Pitkanen OM AD - Division of Pediatric Cardiology and Cardiac and Transplantation Surgery, Children's Hospital, University of Helsinki and Helsinki University Central Hospital, Helsinki, Finland. LA - eng PT - Journal Article DEP - 20151129 PL - Germany TA - Eur J Cardiothorac Surg JT - European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery JID - 8804069 SB - IM MH - Female MH - Finland/epidemiology MH - Follow-Up Studies MH - Heart Septal Defects, Ventricular/epidemiology/*mortality/*surgery MH - Humans MH - Incidence MH - Infant MH - Kaplan-Meier Estimate MH - Male MH - Pulmonary Atresia/epidemiology/*mortality/*surgery MH - Retrospective Studies MH - Severity of Illness Index MH - Treatment Outcome OTO - NOTNLM OT - Congenital heart defect OT - Incidence OT - Late results OT - McGoon index OT - Mortality OT - Pulmonary blood supply EDAT- 2015/12/02 06:00 MHDA- 2017/03/16 06:00 CRDT- 2015/12/02 06:00 PHST- 2015/08/07 00:00 [received] PHST- 2015/10/15 00:00 [accepted] PHST- 2015/12/02 06:00 [entrez] PHST- 2015/12/02 06:00 [pubmed] PHST- 2017/03/16 06:00 [medline] AID - ezv404 [pii] AID - 10.1093/ejcts/ezv404 [doi] PST - ppublish SO - Eur J Cardiothorac Surg. 2016 May;49(5):1411-8. doi: 10.1093/ejcts/ezv404. Epub 2015 Nov 29.