Adults with down syndrome: Safety and long-term outcome of cardiac operation

David S. Majdalany, Harold M. Burkhart, Heidi M. Connolly, Martin D. Abel, Joseph A. Dearani, Carole A. Warnes, Hartzell V. Schaff

Research output: Contribution to journalArticle

15 Scopus citations

Abstract

Objective.: As limited data exist, we sought to review the safety and outcome of cardiac surgery in the adult Down syndrome population. Design.: We reviewed the data of all patients ≥18 years old with Down syndrome who underwent cardiac surgery (1969-2008) at our hospital. Results.: Fifty patients underwent 57 surgeries (mean age 33 years). Fifteen patients had prior cardiac operations in childhood: 7 complete and 1 partial atrioventricular canal, 2 secundum atrial septal defect, 2 Tetralogy of Fallot, 1 patent ductus arteriosus, 1 combined atrioventricular canal and Tetralogy of Fallot, and 1 ventricular septal defect.Operations in adult Down syndrome patients included repair of partial atrioventricular canal in 17, aortic valve replacement in 7, mitral valve replacement/repair in 7, ventricular septal defect in 6, atrial septal defect in 3, Tetralogy of Fallot in 3, pulmonary valve replacement in 3, and other in 11. There was 1 in-hospital death (1.8%) from multiorgan failure. The mean hospital stay was 10.6 days. Average ventilatory support was 2.4 days (range 0-32). Atrial arrhythmias occurred in 14 patients (25%). Six patients had early postoperative pulmonary infections. Mean follow-up was 6 years, maximum 29 years. There were eight late deaths at an average age of 52 years (range 30-58) occurring 15 years postoperatively (range 32 days-29 years); two in the setting of dementia (mean age 56 years). Conclusion.: At an experienced center, adult patients with Down syndrome can undergo cardiac surgery with a low risk of mortality and acceptable morbidity. Atrial arrhythmias and pulmonary infections are common postoperatively.

Original languageEnglish (US)
Pages (from-to)38-43
Number of pages6
JournalCongenital Heart Disease
Volume5
Issue number1
DOIs
StatePublished - Jan 1 2010

Keywords

  • Adult congenital heart disease
  • Cardiac surgery
  • Down syndrome

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Surgery
  • Radiology Nuclear Medicine and imaging
  • Cardiology and Cardiovascular Medicine

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    Majdalany, D. S., Burkhart, H. M., Connolly, H. M., Abel, M. D., Dearani, J. A., Warnes, C. A., & Schaff, H. V. (2010). Adults with down syndrome: Safety and long-term outcome of cardiac operation. Congenital Heart Disease, 5(1), 38-43. https://doi.org/10.1111/j.1747-0803.2009.00349.x