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Postsurgical course of patients with congenitally corrected transposition of the great arteries
M Voskuil1, M G Hazekamp, L J Kroft
1Department of Cardiology, Academic Medical Center, University of Amsterdam, The Netherlands.
Insights
Congenitally corrected transposition significantly reduces survival due to systemic right ventricle issues and tricuspid valve regurgitation. Early interventions may not prevent long-term functional decline in these rare congenital heart conditions.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Congenital Heart Disease
Background:
- Congenitally corrected transposition (CCT) is a rare congenital heart anomaly with limited understanding of its long-term prognosis.
- The systemic right ventricle's function in CCT patients is a subject of ongoing debate and concern.
Purpose of the Study:
- To evaluate the functional status and clinical course of patients with congenitally corrected transposition.
- To assess the long-term outcomes and survival rates in a cohort of CCT patients, with a focus on systemic right ventricular function.
Main Methods:
- Retrospective analysis of 73 patients (aged 10 days to 73 years) with CCT.
- Long-term follow-up (1 week to 37 years) assessing survival, functional status, and interventions.
- Detailed evaluation of systemic right ventricular function and tricuspid valve regurgitation.
Main Results:
- Overall survival was significantly reduced compared to the general population, with an 11% mortality rate.
- Right ventricular function deterioration and increased tricuspid regurgitation were common, often starting at a young age.
- Intracardiac operations were associated with more frequent right ventricular and tricuspid valve dysfunction compared to palliative or no surgery.
Conclusions:
- Survival for patients with congenitally corrected transposition is substantially lower than expected.
- Deterioration of systemic right ventricular and tricuspid valve function is a significant long-term complication.
- Associated cardiac defects and the intrinsic nature of CCT contribute to reduced survival and functional decline.
Abstract:
Because congenitally corrected transposition is a rare congenital anomaly, little is known about the prognosis of patients with this syndrome. The present study evaluated the functional status and clinical course of 73 patients (42 male and 31 female) aged 10 days to 73 years. Follow-up was 1 week to 37 years (mean 12.7 years). Particular attention was paid to the systemic right ventricle, considering the current controversies about long-term right ventricular function. Survival of patients with this condition in general was significantly below normal. The total mortality rate was 11% (8 patients) after a mean follow-up of 12.7 years (range 10 days to 37 years). Mean age at death was 18.5 years (range 6 days to 63 years). The mean age of the survivors at latest follow-up was 21.4 years (range 4 months to 73 years). In most patients, right ventricular function deteriorated and tricuspid valve regurgitation increased, which began at a very young age. Patients without associated lesions developed complications at a higher age compared with the total group. Right ventricular and tricuspid valvular function deteriorated more frequently in patients following intracardiac operation (28% and 52%, respectively) compared with patients undergoing palliative intervention (16%) or no surgery at all (28%). We conclude that survival of patients with this condition is substantially reduced compared with the natural history of an age- and gender-matched general population. This could be explained by the associated cardiac defects, but also by the anatomic condition itself.