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Surgical palliation of cardiac malformations associated with right isomerism
Insights
Palliative surgery for right isomerism in infants yields poor outcomes, especially with obstructed total anomalous pulmonary venous connection (TAPVC). Early TAPVC repair without cardiopulmonary bypass is recommended for better results.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease Surgery
- Cardiovascular Anomalies
Background:
- Right isomerism is a complex congenital heart defect.
- Palliative surgical interventions were analyzed in pediatric patients with right isomerism.
- Patients were categorized based on the presence or absence of total anomalous pulmonary venous connection (TAPVC).
Purpose of the Study:
- To evaluate the outcomes of palliative surgery in pediatric patients with right isomerism.
- To identify risk factors and suggest improvements for surgical palliation in this population.
Main Methods:
- Retrospective analysis of 13 pediatric patients undergoing palliative surgery between 1985 and 1993.
- Patients divided into two groups: Group 1 (with TAPVC) and Group 2 (without TAPVC).
- Surgical procedures included TAPVC repair, Blalock-Taussig shunt, and pulmonary artery banding.
Main Results:
- Group 1 (TAPVC): High mortality (6/6 patients) due to pulmonary venous obstruction, myocardial failure, and congestive heart failure.
- Group 2 (no TAPVC): Lower mortality (1/7 hospital, 3/7 late deaths), with causes including unknown, shunt failure, and pneumonia.
- Outcomes suggest poor results for surgical palliation in young infants with obstructed TAPVC.
Conclusions:
- Surgical palliation for right isomerism, particularly with obstructed TAPVC, has a high mortality rate in infants.
- Prompt TAPVC repair without cardiopulmonary bypass is advised for infants with diagnosed pulmonary venous obstruction.
- Low-calibrated systemic-pulmonary artery shunts are recommended, especially with atrioventricular valve regurgitation. Long-term follow-up is crucial for survivors.
Abstract:
Between 1985 and 1993, palliative surgery was performed on 13 pediatric patients who had complex cardiovascular anomalies associated with right isomerism. The patients included two neonates, ten infants, and one child who were divided into two groups according to whether or not a total anomalous pulmonary venous connection (TAPVC) was present. Group 1 consisted of six patients with TAPVC and group 2 consisted of seven patients without TAPVC. In group 1, the surgical procedures involved TAPVC repair alone in two patients, combined TAPVC repair with a modified Blalock-Taussig shunt in two, combined TAPVC repair with pulmonary artery banding in one, and a modified Blalock-Taussig shunt alone in one. There were five hospital deaths and one late death in this group: pulmonary venous obstruction in two patients, perioperative myocardial failure in the two neonates, and congestive heart failure caused by increased pulmonary blood flow in two patients. In group 2, all the patients underwent systemic-pulmonary artery shunts, and there was one hospital death and three late deaths, the causes of which were unknown in two patients, and shunt failure and pneumonia in one patient each. These results suggest that surgical palliation for right isomerism produces poor results in young infants with obstructed TAPVC. Thus, we conclude that TAPVC repair should be performed without delay if pulmonary venous obstruction has been diagnosed clinically. Resolving pulmonary venous obstruction without cardiopulmonary bypass (CPB) may be preferable for infants, considering their difficult management. The systemic-pulmonary artery shunt should be of the low-calibrated type, especially if common atrioventricular valve regurgitation exists. If infants survive the surgery, they must be carefully followed up for a long period due to the risk of sudden death or infection.