ABSTRACT
Aims: Down syndrome (DS) is the most common chromosome abnormality among live born infants. The aim of this study is to determine the Clinical outcome of patients with DS and congenital heart disease (CHD). Study Design: A cross-sectional study. Place and Duration of Study: On 100 patients with DS and CHD who underwent diagnostic and therapeutic work up in the pediatric cardiology department from September 2001 through September 2012 in Imam Reza hospital (Mashhad, Iran). Methodology: All data collected according to a designed checklist. Most of these patients had previous history of admission in the pediatric cardiology ward. Some of these data were derived from the patient's file. Others data got by taking a history from the parents of patients and direct follow up of the patients by pediatric assistant and echocardiography by a pediatric cardiologist. Surgery was done in Imam Reza hospital. Palliative surgeries include Pulmonary Artery Banding (PAB) +/- PDA closure (if the PDA was present) and total corrections include complete correction of the defect such as ASD closure, VSD closure and repair of other defects. Medical treatments include Digoxin, Captopril, Sildenafil and Propranolol which were administered based on the type of disorders. Independent t-test, and Chi-square test were used to compare quantitative and qualitative variables between groups, respectively. Data analysis was done with SPSS ver. 11.5 and P<0.05 was considered as statistically significant. Results: The mean age of CHD diagnosis was 3.10±2.52 months and mean age of refer to pediatric cardiologist was 5.46±8.60 months. 52% of subjects were females. 50 patients underwent surgical therapy and 50 patients underwent non-surgical therapy (Medical therapy). Of 100 patients, 43(43%) patients were expired, 36(36%) patients had improved signs, and complications were detected in 25(43.85%) of live patients. The mean age of death in patients was 15.24±11.69 months. The Pulmonary Arterial Pressure (PAP) decreased in 68.4 percent of patients after surgical therapy and 16.6 percent of patients with non-surgical therapy. There is a significant correlation between the type of therapy and PAP after treatment (P=.001). Complications of CHD were detected in 8 patients of the surgical group (27.58% of live patients), 5(33.33%) patients of non-surgical (Medical therapy) group and in 12(92.3%) the refusing surgery group. Conclusion: The early diagnosis and therapeutic intervention especially cardiac surgery is critical in this group.