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Surgical experience with congenital heart disease in Down's syndrome
U Parvathy1, K R Balakrishnan, M S Ranjith
1Department of Cardiothoracic Surgery, Sri Ramachandra Medical College University Hospital, Chennai.
Insights
Cardiac surgery offers a good prognosis for children with Down's syndrome and congenital heart defects. Surgical intervention significantly reduces hospitalizations and improves developmental outcomes in these complex pediatric patients.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Down Syndrome Genetics
Background:
- Children with Down's syndrome frequently present with congenital heart defects, posing significant management challenges.
- Congenital heart disease (CHD) is a common comorbidity in Down's syndrome, impacting patient health and requiring specialized care.
Purpose of the Study:
- To evaluate the role and outcomes of cardiac surgery in pediatric patients with Down's syndrome and congenital heart defects.
- To assess the surgical management, post-operative course, and long-term follow-up of children with Down's syndrome and CHD.
Main Methods:
- Retrospective review of clinical data, surgical procedures, and follow-up information for 21 patients with Down's syndrome and CHD.
- Analysis of patient demographics, cardiac lesion types, surgical interventions (palliative vs. primary repair), and post-operative complications.
Main Results:
- Atrioventricular septal defect was the most common lesion (33.3%).
- All patients survived the initial operation, with 81% undergoing primary repair.
- Post-operative complications occurred in 28.5% of patients; late mortality was 9.5%.
- Significant reduction in hospitalizations reported by parents, with 50% showing normalized pulmonary artery pressure and 60% demonstrating developmental improvement.
Conclusions:
- Cardiac surgery is a safe and effective intervention for children with Down's syndrome and congenital heart defects.
- Surgical management leads to improved clinical outcomes, reduced hospitalizations, and enhanced developmental progress in this population.
Abstract:
Children with Down's syndrome and congenital heart defects have multiple problems. The role of cardiac surgery in the management of these patients was investigated by reviewing the clinical data, hospital course and follow-up of 21 patients (9 males and 12 females, age range 1 month to 14 years) with Down's syndrome and congenital heart defects operated in our institute. Twelve (57%) of these were infants and nine (43%), older children. Five were in congestive cardiac failure, four were hypothyroid. The heart lesions ranked in incidence as follows: atrioventricular septal defect 7 (33.3%), tetralogy of Fallot 3 (14.3%), tetralogy of Fallot & atrioventricular septal defect both 2 (9.5%), double outlet right ventricle with pulmonary stenosis 1 (4.8%), patent ductus arteriosus 2 (9.5%), patent ductus arteriosus plus coarctation 1 (4.8%), ventricular septal defect 2 (9.5%), atrial septal defect plus ventricular septal defect 1 (4.8%), atrial septal defect plus patent ductus arteriosus plus right pulmonary artery stenosis 1 (4.8%) and transposition of great arteries with multiple ventricular septal defect 1 (4.8%). Four (19%) patients had palliative procedures while the rest (81%) underwent primary repair. All survived the operation. The post-operative period was complicated in 6 (28.5%), with respiratory infections in 3, pulmonary hypertensive crisis in 2 and complete heart block in 1. The early mortality was 0, while there were 2 (9.5%) late deaths. The number of hospitalisations was markedly reduced according to the parents. Follow-up showed near normal pulmonary artery pressure in 50 percent children with large shunts and a good developmental spurt was seen in 60 percent. From a purely surgical viewpoint, the prognosis for children with Down's syndrome and congenital heart disease is good.