The effect of changing attitudes to Down’s syndrome in the management of complete atrioventricular septal defects

• Published in: Archives of disease in childhood Vol. 81; no. 2; pp. 151 - 154
• Main Authors: AMARK, K, SUNNEGARDH, J
• Format: Journal Article
• Language: English
• Published: London BMJ Publishing Group Ltd and Royal College of Paediatrics and Child Health 01.08.1999; BMJ; BMJ Publishing Group LTD; BMJ Group
• Subjects: Adolescent; Age; Age of Onset; Angiography - methods; Attitude; Attitude change; Autism; Autism Spectrum Disorders; Biological and medical sciences; Blood Flow Velocity; Child; Child, Preschool; Children & youth; Chromosome aberrations; congenital heart disease; Decision Making; Defects; Diagnostic Errors; diagnostic techniques; Down Syndrome; Down Syndrome - complications; Down Syndrome - psychology; Down’s syndrome; Echocardiography; Female; Females; Heart Septal Defects, Atrial - psychology; Heart Septal Defects, Atrial - surgery; Humans; Hypertension; Infant; Infant, Newborn; Male; Measurement Techniques; Medical genetics; Medical imaging; Medical sciences; Original; Patients; persistent common atrioventricular canal; Predictor Variables; public opinion; Pulmonary Circulation - physiology; Refusal to Treat; sex; Surgery; Veins & arteries; Chromosomal aberration; Sonography; Human; Endocardial Cushion defect; Trisomy; Echocardiography; Social perception; Decision making; Treatment efficiency; Chromosome G21; Aneuploidy; Cardiovascular disease; Down syndrome; Medical screening; Congenital disease; Association; Newborn; Malformation; Heart disease; Surgery; Early
• ISSN: 0003-9888; 1468-2044
• DOI: 10.1136/adc.81.2.151

OBJECTIVES To describe the evaluation, decision making, and care of children with a complete atrioventricular septal defect (CAVSD). STUDY DESIGN Retrospective study of 136 consecutive cases from 1970 to 1996. RESULTS A total of 115 (85%) children had Down’s syndrome. Denial of surgery without obvious medical reasons was more common in the early years, as was parental refusal of offered surgery and institutional care of the children. Improved results in later years encouraged surgical treatment for all these patients, but more liberal attitudes towards patients with Down’s syndrome preceded the improved results. The use of echocardiography as a screening method for all newborns with Down’s syndrome made it possible to plan for correction within the 1st months of life. CONCLUSIONS Changing attitudes in society and widespread use of echocardiography have significantly improved the management of children with a CAVSD and Down’s syndrome. Key messages Attitudes towards evaluating and offering surgery to Down’s syndrome patients have changed Echocardiography has made it possible to screen all children with Down’s syndrome within weeks of birth Younger age at diagnosis improves the chances of successful surgery while pulmonary hypertension is still reversible Echocardiography has decreased the role of catheterisation as a preoperative investigation method