The presence of Down syndrome is not a risk factor in complete atrioventricular septal defect repair Ruediger Lange MD, PhDa, Thomas Guenther MDa,
, 
, Raymonde Busch MSc, John Hess MD, PhDb and Christian Schreiber MDa aDepartment of Cardiovascular Surgery, German Heart Center, Clinic at the Technical University, Munich, Germany
bDepartment of Pediatric Cardiology, German Heart Center, Clinic at the Technical University, Munich, Germany
cInstitute of Medical Statistics and Epidemiology, Technical University, Munich, Germany.
Received 21 September 2006; revised 2 January 2007; accepted 8 January 2007. Available online 26 July 2007.
Objective Down syndrome is frequently associated with complete atrioventricular septal defect. The aim of this retrospective study was to evaluate the impact of Down syndrome on morphologic features, surgical management, and perioperative and long-term mortality in patients with complete atrioventricular septal defect. Methods Between October 1974 and March 2005, 476 patients with complete atrioventricular septal defect underwent surgery, 341 (71.6%) of whom had Down syndrome. A total of 336 patients (70.6%) underwent primary repair, 95 patients (20%) underwent palliative surgery on an average of 3.4 ± 2.9 years before repair, and 45 patients (9.4%) had no subsequent repair. Results Patients with Down syndrome (group D) exhibited higher preoperative pulmonary artery pressure (P = .012) and resistance (P = .006) compared with patients without Down syndrome (group ND). Rastelli types B and C were more frequent in group D (P = .002), but left atrioventricular valve anomalies were more frequent in group ND (P = .03). No difference was seen regarding operative strategy (P = .117) and timing of repair (P = .32). Thirty-day mortality after complete atrioventricular septal defect repair was 4.9% in group D compared with 5.6% in group ND. Actuarial survival after 20 years is 84% ± 2.5% (group D) and 75% ± 5.7% (group ND) (P = .252), and freedom from left atrioventricular valve-related reoperation is 82% ± 2.9% (group D) and 72% ± 5.3% (group ND) (P = .004). Reinterventions on the left atrioventricular valve were required significantly more often in patients in group ND. Conclusions The presence of Down syndrome in patients with complete atrioventricular septal defect is not a risk factor for surgical repair. Because of the early development of pulmonary vascular disease, primary repair should be performed within the first 6 months of life. In the long term, reintervention on the left atrioventricular valve is more often required in children with a normal chromosomal pattern. 20; 35
Abbreviations: CAVSD, complete atrioventricular septal defect; LAVV, left atrioventricular valve