Background: Congenital heart disease (CHD) are present in patient with Down syndrome (DS) in approximately 40-50%. The most common CHD in most study is ventricular septal defect (VSD) and atrioventricular septal defect (AVSD). The object of this study is to find the prevalence of each type of CHD in DS patients and analyze outcome who were underwent surgery.
Method: DS aged <15 year who underwent echocardiogram from pediatric cardiology unit from 1 Jan 2006 – 31 Dec 2017 were reviewed. Clinical history and outcome of treatment were retrospectively study by reviewing electronic medical and surgical records.
Result: Among 287 DS patients who was underwent echocardiography, 256 patients (89.19%) had CHD. The first three most common CHD included VSD in 127 patients (50%), AVSD in 50 patients (23%) and PDA in 33 patients (13%). 151 patients (58.98%) was underwent surgery or device occlusion. Overall total mortality rate was 10.93% (28 /256) and 13 out of 28 patient died before surgery or device occlusion, the most common cause of death was infection included pneumonia in 9 patients (32%) and sepsis in 8 patients. Of whom 151 who underwent surgery or device occlusion, In-hospital mortality after surgery or device occlusion was 2% (6 patients) and later mortality was 4% (9 patients). Factors associated with mortality included AVSD (p value = 0.04), birth with low birth weight or less weight (p value = 0.029) and post-operative disseminated intravascular coagulation (p value = 0.003)
Conclusion: Although AVSD, low birth weight and post-operative DIC are associated with mortality in DS with CHD, cardiac surgery in DS had low mortality rate .Corrective cardiac surgery is recommended in every DS otherwise contraindicated.
