Early and Medium-Term Results After Correction of Total Anomalous Pulmonary Venous Drainage in Infancy

Abstract

The aim of this clinical study is to identify whether the surgical management of total anomalous pulmonary venous drainage (TAPVD) in the neonatal and infant patient is adequate.

Since October 1977, 39 consecutive infants have had repair of TAPVD. The age at operation ranged between 1 and 300 days, 17 were neonates. The weight was 2.2–8.4 kg (mean 3.9 kg). The site of drainage was supracardiac in 13 (33.3%), cardiac in 10 (25.6%), infracardiac in 13 (33.3%), and mixed in three patients (7.7%). Of the 36 patients who had cardiac catheter, 31 had pulmonary hypertension; two-thirds had a preoperative RV/LV pressure ratio of 1 or higher. Twenty-two patients (56.4%) were intubated and ventilated prior to operation.

Postoperative pulmonary hypertensive crises were frequent (16 patients) and treated according to a “hypertensive crises” protocol. There were five early deaths (12.8%). Two children died late (5.1%), 118 and 334 days postoperatively, with intrapulmonary vein stenosis, confirmed at autopsy. Thirty-two (82.1%) patients are survivors 3 months to 11 years after total correction. All are in NYHA class I on no drugs, and leading normal lives.

Total correction of TAPVD together with aggressive pre- and postoperative management of pulmonary hypertensive crises carries a decreasing early risk. For all early survivors — except for patients with intrapulmonary vein sclerosis — the operation was curative.