Congenital Heart Disease in Children with Down syndrome in Kermanshah, West of Iran during 2002 - 2016

Background Down syndrome is the most common chromosomal anomaly. Dysmorphic features can occur in several organs in this syndrome. Cardiac anomalies with a prevalence of 50% are the most common anomalies responsible for death during the first two years of life. We aimed to determine the prevalence of cardiac anomalies among Down syndrome patients admitted to two tertiary hospitals in Kermanshah, Iran from 2002 to 2016. Materials and Methods In this descriptive study, the medical records of all patients with Down syndrome admitted to two university hospitals namely Imam Ali and Imam Reza, Kermanshah city located in Western part of Iran in the study period were reviewed. All patients had received Echocardiography two-dimensional (2D). The required data including cardiac anomaly type, consanguinity of parents, maternal age, surgical interventions, and survival were collected into a checklist. Results:  During the study period, 166 patients with Down syndrome had received diagnostic and therapeutic services in the studied hospitals. There were 70 males (42.2%) and 96 females (57.8%). Familial consanguinity was documented in 95 patients (57.2%). Mean ± standard deviation (SD) maternal age at delivery was 26.33 (±4.7) years (range, 15 to 45 years). Of 166 studied patients, 123 (74.1%) had cardiac anomaly. Ventricular septal defect (VSD) was the most prevalent single defect seen in 32 (26%) patients, followed by atrial septal defect (ASD) detected in in 22 (17.8%) patients. Seventy patients (42.1%) required surgical interventions. A total of 74 patients experienced relative improvement of the symptoms. Also, seven patients (10.2%) died including five females and two males. Conclusion: The frequency of cardiac anomalies in the studied population of Down syndrome patients was higher than former reported figures. The pattern of the anomalies is compatible with some former reports, but contradicts other reports.