Adults with Down syndrome: safety and long-term outcome of cardiac operation.
OBJECTIVE
As limited data exist, we sought to review the safety and outcome of cardiac surgery in the adult Down syndrome population.


METHODS
We reviewed the data of all patients >or=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).


CONCLUSIONS
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.
Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN 55905, USA. majdalany.david@mayo.edu
David S
DS
Majdalany
Harold M
HM
Burkhart
Heidi M
HM
Connolly
Martin D
MD
Abel
Joseph A
JA
Dearani
Carole A
CA
Warnes
Hartzell V
HV
Schaff
D000293:Adolescent; D000328:Adult; D001145:Arrhythmias, Cardiac / Q000209:etiology; D006348:Cardiac Surgical Procedures / Q000009:adverse effects / Q000401:mortality; D004314:Down Syndrome / Q000150:complications* / Q000401:mortality; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications / Q000401:mortality / Q000601:surgery*; D017052:Hospital Mortality; D006801:Humans; D007902:Length of Stay; D008297:Male; D008875:Middle Aged; D012121:Respiration, Artificial; D012141:Respiratory Tract Infections / Q000209:etiology; D012189:Retrospective Studies; D018570:Risk Assessment; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult