Better surgical prognosis for patients with complete atrioventricular septal defect and Down's syndrome.
BACKGROUND
Several studies have shown that Down's syndrome is not a risk factor for biventricular repair of complete atrioventricular septal defects. However, few data are available about the comprehensive outcome of all the cardiac surgical procedures in patients with trisomy 21, including palliative surgery.


METHODS
This is a retrospective study of 206 consecutive patients who underwent cardiac surgery from January 1992 to January 2002. Data about mortality and morbidity were analyzed and the impact of Down's syndrome was evaluated.


RESULTS
Overall mortality was 7.7%. Actuarial survival was 94% among patients with Down's syndrome versus 86% of the group with normal karyotype (p = 0.12). The presence of unbalanced ventricles was the only independent risk factor affecting survival at multivariate analysis (p < 0.0001). The need for a Norwood type surgery was more frequent among non-Down patients (12.0% vs 1.5%, p = 0.02) as was the prevalence of pulmonary artery banding operations (22.9% vs 9.3%, p = 0.04). Cumulative mortality after palliation was higher in non-Down patients (44% vs 2.9%, p = 0.0001). Freedom from reoperation was lower in the group with normal chromosomes in respect to patients with Down's syndrome (81.4% vs 94.6%, p = 0.04), due to the higher prevalence of anomalies of the mitral valve (4.9% vs 1.8%, p = 0.03) or left ventricular outflow tract (7.3% vs 0%, p = 0.01).


CONCLUSIONS
Down patients showed a decreased risk for biventricular repair and lower mortality and morbidity in cases of complex cardiac malformations requiring complex palliative operations.
Pediatric Cardiology and Cardiac Surgery, Policlinico S. Orsola, University of Bologna, Via Massarenti 9, 40138 Bologna, Italy. r.formigari1@tin.it
Roberto
R
Formigari
Roberto M
RM
Di Donato
Gaetano
G
Gargiulo
Duccio
D
Di Carlo
Cristiana
C
Feltri
Fernando M
FM
Picchio
Bruno
B
Marino
D000015:Abnormalities, Multiple; D001017:Aortic Coarctation / Q000150:complications / Q000601:surgery; D006348:Cardiac Surgical Procedures / Q000379:methods; D002675:Child, Preschool; D004314:Down Syndrome / Q000150:complications*; D005260:Female; D006330:Heart Defects, Congenital / Q000235:genetics / Q000601:surgery; D006343:Heart Septal Defects / Q000235:genetics / Q000601:surgery*; D006801:Humans; D007223:Infant; D015993:Life Tables; D008026:Ligation; D008297:Male; D010166:Palliative Care; D011379:Prognosis; D016016:Proportional Hazards Models; D011651:Pulmonary Artery / Q000601:surgery; D012189:Retrospective Studies