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Total anomalous pulmonary venous drainage. Seventeen-year surgical experience
R K Lamb1, S A Qureshi, J L Wilkinson
1Department of Paediatric Cardiology, Royal Liverpool Children's Hospital, England.
Insights
Surgical correction of total anomalous pulmonary venous drainage in 80 children showed improved survival rates over time. The year of operation significantly impacted outcomes, with later years demonstrating better results for this complex congenital heart defect.
Area of Science:
- Pediatric Cardiac Surgery
- Congenital Heart Disease
- Cardiovascular Surgery
Background:
- Total anomalous pulmonary venous drainage (TAPVD) is a severe congenital heart defect requiring surgical intervention.
- Early diagnosis and surgical correction are crucial for improving outcomes in affected infants and children.
- Surgical techniques and patient management have evolved, potentially impacting survival rates.
Purpose of the Study:
- To evaluate the surgical outcomes and long-term survival of children undergoing correction for total anomalous pulmonary venous drainage.
- To identify factors influencing survival, particularly the impact of the year of operation.
- To assess the incidence of early and late complications, including pulmonary venous obstruction and reoperations.
Main Methods:
- Retrospective analysis of 80 pediatric patients who underwent surgical correction for TAPVD between 1968 and 1985.
- Data collection included patient demographics, type of TAPVD, surgical procedures, and follow-up duration.
- Statistical analysis was performed to identify factors affecting survival, with a focus on the year of operation.
Main Results:
- A total of 80 children (47 boys, 33 girls) underwent surgery, with 87.5% being less than 1 year old and 72.5% weighing under 5 kg.
- The overall early mortality rate was 17.5% (14 deaths) and late mortality was 7.5% (6 deaths).
- Survival rates significantly improved over time, with early mortality decreasing from 29% (1968-1977) to 11% (1978-1985) (p=0.04).
- Pulmonary venous obstruction occurred in 6% of patients, all of whom died.
- Reoperations were performed in 6% of patients for residual shunts or superior vena caval obstruction.
Conclusions:
- The year of surgical intervention is a critical factor in improving survival for children with total anomalous pulmonary venous drainage.
- Advances in surgical timing and management have led to reduced early mortality rates for TAPVD.
- Pulmonary venous obstruction remains a significant challenge, highlighting the need for vigilant postoperative monitoring and management.
Abstract:
Between 1968 and 1985, 80 children underwent correction of total anomalous pulmonary venous drainage. There were 47 boys and 33 girls whose ages ranged from 3 days to 16 years (median 2 months, interquartile range 5 years). Seventy (87.5%) were less than 1 year of age at operation. Fifty-eight (72.5%) weighed less than 5 kg, the range being 1.6 to 42 kg (median 3.7 kg, interquartile range 2.4 kg). Forty-five (56%) patients had supracardiac, 14 (17.5%) cardiac, 15 (19%) infracardiac, and 6 (7.5%) had mixed total anomalous pulmonary venous drainage. Follow-up was complete in 78 (97.5%) and ranged from 6 to 189 months (median 58 months, interquartile range 59 months). There were 14 (17.5%) early and six (7.5%) late deaths. Analysis by various factors revealed year of operation as the only factor to affect survival at the 5% level of significance. Early mortality was 29% between 1968-1977 and 11% between 1978-1985 (p = 0.04). Postoperative pulmonary venous obstruction occurred in five (6%) patients between 6 weeks and 3 months after operation. All 5 died, three after reoperation. Five (6%) other children had reoperations, four for residual shunts and one for superior vena caval obstruction.