The Outcome of Children Born with Pulmonary Atresia and Intact Ventricular Septum in Sweden from 1980 to 1999

• Published in: Scandinavian cardiovascular journal : SCJ Vol. 35; no. 3; pp. 192 - 198
• Main Authors: JOELSSON, B.-M. Ekman, SUNNEGARDH, J, HANSEUS, K, BERGGREN, H, JONZON, A, JÖGI, P, LUNDELL, B
• Format: Journal Article
• Language: English
• Published: Copenhagen Informa UK Ltd 2001; Oslo Taylor & Francis; Stockholm Scandinavian University Press
• Subjects: Biological and medical sciences; Cardiac and Cardiovascular Systems; Cardiology. Vascular system; Child; Child, Preschool; Children Long-TERM Follow-UP Pulmonary Atresia And Intact Ventricular Septum Surgery; Electrocardiography; Female; Heart; Heart Septal Defects; Heart Septal Defects - physiopathology; Heart Septal Defects - surgery; Heart Septum; Heart Septum - physiopathology; Heart Septum - surgery; Humans; Infant; Infant, Newborn; Kardiologi; Male; Medical sciences; Medicin och hälsovetenskap; Newborn; physiopathology; Preschool; Pulmonary Atresia; Pulmonary Atresia - physiopathology; Pulmonary Atresia - surgery; surgery; Sweden; Treatment Outcome; Tumors of the heart; Sweden; Europe; Human; Prognosis; Respiratory disease; Cardiovascular disease; Congenital disease; Epidemiology; Pulmonary artery; Arterial disease; Vascular disease; Malformation; Atresia; Ventricular septum; Child; Public health
• ISSN: 1401-7431; 1651-2006
• DOI: 10.1080/140174301750305072

Objective - To study the outcome of all children born with pulmonary atresia and intact ventricular septum in Sweden between 1980 and 1999. Design - Retrospective study of medical records with review of the initial, preoperative angiocardiographic and echocardiographic examinations. Results - A total of 84 children were born with pulmonary atresia and intact ventricular septum, giving an incidence of 4.2 per 100 000 live births. In all, 77 were operated on with a 1-year survival rate of 75%. Thirty-six children had ventriculocoronary communications, with a 1-year survival rate of 50%. At the end of the study period, 52 children were alive, 32 with biventricular repair, and 19 with univentricular repair. Follow-up time was 14 days-20 years (median, 6 years). Statistical analysis of incremental risk factors for death showed statistical significance for low birth weight, male sex, muscular pulmonary atresia, and having a systemic-to-pulmonary shunt as the sole initial intervention. Conclusion - Complete national data of all patients born with pulmonary atresia and intact ventricular septum during 1980-1999 in Sweden revealed a total mortality in accordance with previous reports for results for surgery. Further improvements demand a thorough preoperative investigation of the cardiac anatomy, particularly of the ventriculocoronary communications, to enable right ventricular decompression whenever possible.