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[Mortality and survival in total anomalous pulmonary venous drainage]
G Cabezuelo Huerta1, P Frontera Izquierdo
1Departamento de Pediatría, Hospital Infantil La Fe, Universidad de Valencia.
Insights
This study on isolated total anomalous pulmonary venous connection in 36 children found a high overall mortality of 63.8%. Surgical outcomes for congenital heart disease did not improve over time.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Congenital Heart Disease
Context:
- Isolated total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart defect.
- This study analyzed 36 cases managed between 1971 and 1988.
- TAPVC represented 1.55% of congenital heart disease cases diagnosed via catheterization and angiography.
Purpose:
- To present the management outcomes of isolated total anomalous pulmonary venous connection.
- To evaluate survival rates and identify determinants of outcome.
- To assess trends in surgical results over a 17-year period.
Summary:
- The study included 36 patients with isolated TAPVC (19 Type I, 5 Type II, 8 Type III, 4 mixed).
- Overall mortality was 63.8%. Twenty-six infants underwent surgery at a mean age of 2.5 months, with a 57.7% operative mortality.
- Eleven infants survived surgery; long-term survival was 34.4% overall and 42.3% for operated patients. Lesion type and pulmonary venous obstruction were key survival factors.
Impact:
- The findings highlight the significant challenges in managing isolated TAPVC.
- No improvement in surgical or overall results was observed in the last decade of the study period.
- This data underscores the need for further research into improved treatment strategies for this critical congenital heart defect.
Abstract:
The results in the management of 36 cases of isolated total anomalous pulmonary venous connection are presented. This patients are the 1.55% of the 2,322 children diagnosed of congenital heart disease by catheterization and angiography in our hospital at the 1971-1988 period. Nineteen had a type I, five a type II, eight a type III and four a mixed type lesion. The total mortality rate was 63.8%. Twenty six infants underwent surgical correction at a mean age of 2.5 months with an operative mortality rate of the 57.7%. Eleven infants survived operation. There have been no late deaths but one children developed stenosis at the anastomosis. The final actuarial survival rate was the 34.4% in the total group and the 42.3% in the operated group. The strongest determinants of survival were the type of lesion and pulmonary venous obstruction. No improving in the total and the surgical results has been observed in the last 10 years.