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Long-term follow-up of children with Down syndrome with cardiac lesions
Insights
Cardiac surgery improves functional class in Down syndrome patients with heart disease. Non-surgical management leads to increased mortality and functional decline, primarily due to pulmonary vascular disease.
Area of Science:
- Cardiology
- Genetics
- Pediatrics
Background:
- Down syndrome (DS) is associated with a high prevalence of congenital heart defects.
- Atrioventricular canal defects are common cardiac lesions in DS patients.
- Cardiac function and long-term outcomes in DS patients with heart disease require further investigation.
Purpose of the Study:
- To evaluate the impact of cardiac surgery on functional class and survival in patients with Down syndrome and cardiac lesions.
- To compare outcomes between surgically and non-surgically managed DS patients with heart disease.
Main Methods:
- Retrospective review of 284 patients with Down syndrome seen between 1951-1989.
- Analysis of cardiac diagnoses, functional class (FC), treatment (surgical vs. non-surgical), and survival.
- Assessment of mortality causes and functional status at follow-up.
Main Results:
- Of 114 patients with cardiac murmurs, 47 had definitive diagnoses, with 38 undergoing long-term follow-up.
- Fifteen patients had atrioventricular canals; 18 survived (13 surgical, 5 non-surgical), and 20 died (4 surgical, 16 non-surgical).
- Surgical management improved functional class, while non-surgical management resulted in higher mortality and functional deterioration, often due to pulmonary vascular disease.
Conclusions:
- Cardiac surgery offers significant benefits for patients with Down syndrome and heart disease, improving functional class and survival.
- Non-surgical management of cardiac lesions in DS patients is associated with poor outcomes, including increased mortality and functional decline.
- Pulmonary vascular disease and congestive heart failure are major contributors to mortality in non-surgically treated DS patients with cardiac conditions.
Abstract:
Two hundred and eighty four patients with Down Syndrome (DS) were seen between 1951-1989. One-hundred and fourteen (40.1%) had a [corrected] cardiac murmur at presentation. A definitive cardiac diagnosis was established in 47 (41%) patients, of which 38 had long term follow-up. Fifteen (33%) patients had atrioventricular canals. There were 21 males and 17 females, with a mean age of 5.3 years. Fifteen (39%) patients were in functional class (FC) I, 16 (42%) in FC II, six (15%) in FC III, and one patient in FC IV at the time of presentation. There were 18 survivors (13 in the surgical group and five in the nonsurgical group) and 20 nonsurvivors (four in the surgical group and 16 in the nonsurgical group). Causes of death in the nonsurgical group included congestive heart failure, pneumonia, and pulmonary vascular disease, and occurred at a mean age of 8.4 years. Post-operative complications accounted for deaths in three of the four surgical patients. The survivors in the surgical group are presently in FC I/II. In the nonsurgical group, there was increased mortality, especially in those who presented in an earlier era, and a deterioration in functional class on follow-up due to the development of pulmonary vascular disease. Our data suggest that a) patients with Down syndrome and heart disease are helped by cardiac surgery with stabilization and improvement of their functional class; b) deterioration in functional class is seen in patients with Down syndrome with cardiac lesions who are managed nonsurgically [corrected] and c) mortality remains high in such patients treated nonsurgically due to development of pulmonary vascular disease and congestive heart failure.(ABSTRACT TRUNCATED AT 250 WORDS)