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Correction of total anomalous pulmonary venous connection in infancy
F M Lupinetti1, T J Kulik, R H Beekman
1Department of Surgery, University of Michigan School of Medicine, Ann Arbor.
Insights
Operative correction of total anomalous pulmonary venous connection in infants is effective, with low mortality rates. Surgical repair offers good long-term outcomes for most patients, minimizing the need for reoperations.
Area of Science:
- Pediatric Cardiac Surgery
- Congenital Heart Disease
- Thoracic Surgery
Background:
- Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart defect requiring surgical intervention.
- Infants with TAPVC present with varying types of venous connections and degrees of obstruction.
- Early diagnosis and surgical correction are crucial for improving outcomes in affected infants.
Purpose of the Study:
- To evaluate the efficacy and outcomes of operative correction for isolated total anomalous pulmonary venous connection in infants.
- To analyze surgical techniques, complications, and long-term results in a cohort of pediatric patients.
Main Methods:
- Retrospective review of 41 infants (<1 year) undergoing surgical repair of TAPVC between 1985 and 1993.
- Surgical approaches included supracardiac, cardiac, infracardiac, and mixed repairs using deep hypothermia and circulatory arrest.
- Preoperative stabilization involved mechanical ventilation and extracorporeal membrane oxygenation when necessary.
Main Results:
- Operative mortality was low (2/41 patients), with causes including persistent pulmonary hypertension and pulmonary lymphangiectasia.
- The majority of patients (39/41) had good long-term outcomes with a mean follow-up of 26 months.
- Reoperations were infrequent, required for persistent pulmonary venous or superior vena cava obstruction in two patients.
Conclusions:
- Surgical correction of isolated total anomalous pulmonary venous connection in infants can be achieved with low mortality.
- The described operative techniques provide effective long-term results with a low incidence of reoperations.
- Timely surgical intervention for TAPVC in infants leads to favorable outcomes and improved survival rates.
Abstract:
From January 1985 through January 1993, 41 patients less than 1 year of age underwent operative correction of isolated total anomalous pulmonary venous connection. There were 24 boys and 17 girls. The median age at operation was 13 days (range 1 to 282 days) and weight was 3.6 kg (2.5 to 5.2 kg). Locations of the connections were supracardiac in 19, cardiac in 9, infracardiac in 11, and mixed supracardiac and cardiac in 2. Obstruction of the pulmonary veins was severe in 24, mild in 3, and absent in 14. Preoperative stabilization included mechanical ventilation for 15 patients for a mean duration of 2 1/2 days and extracorporeal membrane oxygenation for 1 patient for 1 day. All operations were performed with deep hypothermia and circulatory arrest (mean arrest time 34 minutes). Supracardiac connections were repaired by performing a side-to-side anastomosis between the pulmonary venous confluence and the dome of the left atrium through a superior approach between the superior vena cava and the aorta. Coronary sinus connections were repaired by enlarging the atrial septal defect and the coronary sinus communication with the left atrium and closing the atrial defect with a large patch. Infracardiac repairs included elevation and rotation of the heart to the right and an elongated side-to-side anastomosis between the common venous confluence and the left atrium. One patient died 1 week postoperatively of persistent pulmonary hypertension. Another patient, who was supported by extracorporeal membrane oxygenation before the operation, died 3 months after the operation as a consequence of pulmonary lymphangiectasia. All other patients are alive and well with a mean follow-up of 26 months (range 3 to 77 months). One patient required two subsequent reoperations for persistent pulmonary venous obstruction, and another patient had superior vena cava obstruction necessitating reoperation. Operative treatment of total anomalous pulmonary venous connection in infants can be performed with low mortality and an infrequent need for reoperations.