Adults with Down Syndrome: Safety and Long-term Outcome of Cardiac Operation

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 Ն18 years old with Down syndrome who underwent cardiac surgery 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.

Totally endoscopic robotic atrial septal defect closure in an adult with Down syndrome

“…[2,3] Previously, Nisli et al, [3] evaluated the incidence and types of coronary heart disease patterns in the Turkish children with Down syndrome. The authors reported that, in 421 patients with Down syndrome, 77.6% had a single cardiac lesion, while the remaining 22.4% had multiple defects.…”

“…[1][2][3] Several studies have shown that the probability of concomitant emergence of congenital disorders with Down syndrome as high as 44%. [1] Although complex forms of cardiovascular anomalies, such as atrioventricular canal defects and ventricular septal defects (VSDs) are diagnosed in the early postnatal period, atrial septal defects (ASDs) may present with an incidence of about 16.7% in adulthood.…”

“…[1] Although complex forms of cardiovascular anomalies, such as atrioventricular canal defects and ventricular septal defects (VSDs) are diagnosed in the early postnatal period, atrial septal defects (ASDs) may present with an incidence of about 16.7% in adulthood. [2,3] Early ASD closure prevents the potential risks of right ventricular dilatation and pulmonary dysfunction. [2,3] Alternative to traditional sternotomy and thoracotomy incisions, totally endoscopic robotic ASD closure surgery has proved its safety in adults.…”

“…[2,3] Early ASD closure prevents the potential risks of right ventricular dilatation and pulmonary dysfunction. [2,3] Alternative to traditional sternotomy and thoracotomy incisions, totally endoscopic robotic ASD closure surgery has proved its safety in adults. [4][5][6][7] Robotic surgery can be a preferred approach to minimize the risks of conventional sternotomy and thoracotomy incisions.…”

Szemléletváltozás a Down-szindrómás szívbetegek szívsebészeti kezelésében, 1974–2016

“…Jelentősen meghosszabbodott az élet-koruk; például az Amerikai Egyesült Államokban 1983-tól 1997-ig átlagéletkoruk 25 évről 49 évre emelkedett [8], míg Európában Bittles és mtsai [9] szerint 40 év alatt 12 évről 60 évre. Majdalany és mtsai [10] szerint a betegek 14%-a már megéri a 70 éves életkort.…”