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 Citations (Scopus)

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 2010

Fingerprint

Down Syndrome
Safety
Tetralogy of Fallot
Thoracic Surgery
Ventricular Heart Septal Defects
Cardiac Arrhythmias
Pulmonary Valve
Lung
Patent Ductus Arteriosus
Infection
Aortic Valve
Mitral Valve
Dementia
Length of Stay
Morbidity
Mortality
Population

Keywords

  • Adult congenital heart disease
  • Cardiac surgery
  • Down syndrome

ASJC Scopus subject areas

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

Cite this

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

Adults with down syndrome : Safety and long-term outcome of cardiac operation. / Majdalany, David S.; Burkhart, Harold M.; Connolly, Heidi M.; Abel, Martin D.; Dearani, Joseph A.; Warnes, Carole A.; Schaff, Hartzell V.

In: Congenital Heart Disease, Vol. 5, No. 1, 01.2010, p. 38-43.

Research output: Contribution to journalArticle

Majdalany, DS, Burkhart, HM, Connolly, HM, Abel, MD, Dearani, JA, Warnes, CA & Schaff, HV 2010, 'Adults with down syndrome: Safety and long-term outcome of cardiac operation', Congenital Heart Disease, vol. 5, no. 1, pp. 38-43. https://doi.org/10.1111/j.1747-0803.2009.00349.x
Majdalany DS, Burkhart HM, Connolly HM, Abel MD, Dearani JA, Warnes CA et al. Adults with down syndrome: Safety and long-term outcome of cardiac operation. Congenital Heart Disease. 2010 Jan;5(1):38-43. https://doi.org/10.1111/j.1747-0803.2009.00349.x
Majdalany, David S. ; Burkhart, Harold M. ; Connolly, Heidi M. ; Abel, Martin D. ; Dearani, Joseph A. ; Warnes, Carole A. ; Schaff, Hartzell V. / Adults with down syndrome : Safety and long-term outcome of cardiac operation. In: Congenital Heart Disease. 2010 ; Vol. 5, No. 1. pp. 38-43.
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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.",
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