A Difficult Emergency Surgical Diagnosis: Atresia of the Common Pulmonary Vein

Abstract

A newborn female experienced severe respiratory distress immediately after delivery. She presented with intense cyanosis, refractory hypoxemia, and acidosis. The deterioration was rapidly progressive, leading to the child's demise. The autopsy showed common pulmonary vein atresia, the most severe form of pulmonary venous drainage obstruction. Subpleural and interstitial pulmonary lymphagiectasias also were present as well as evidence of pulmonary hypertension. The lung pathology probably resulted from pulmonary venous atresia during prenatal development.

Specific Echocardiographic Findings Useful for the Diagnosis of Common Pulmonary Vein Atresia

“…3 However, echocardiographic examination was found to be difficult in patients with pneumothorax. 4 , 5 In addition, definitive criteria that would allow establishing a diagnosis using this approach have not been reported. Nevertheless, in this study we successfully used echocardiography to observe a reverse flow in the pulmonary artery at end-diastole as well as in the isthmus of the aorta from early systole to end-diastole.…”

Comparison of prenatal ultrasound and postmortem findings in fetuses with common pulmonary vein atresia

“…TAPVR occurs because of early atresia of the common pulmonary vein, while late atresia leads to CPVA . However, some scholars have assumed that the embryological origin of CPVA is similar to TAPVR, and CPVA is only the most serious obstructive type of TAPVR . That is to say, there is no essential difference between the two, but this assumption is inconclusive .…”

Commentary: If more is no good, then less is more

“…Pulmonary vein atresia is a very rare congenital cardiac anomaly that is usually associated with diagnostic challenges leading to missed or delayed identification, in addition to management challenges due to the neonate's poor clinical condition (often requiring extracorporeal membrane oxygenation support) and associated lung disease that complicates early and late postoperative recovery. 2 , 3 , 4 , 5 Surgical repair of common pulmonary vein atresia has been reported in several cases, and although establishment of a patent anastomosis between the common pulmonary vein to the left atrium can be achieved (similar to surgical repair of total anomalous pulmonary venous connection), the results of surgery in common pulmonary vein atresia have varied, with morbidity and mortality largely related to associated lung disease. 2 , 3 , 4 , 5 , 6 There are strong associations between pulmonary vein atresia and spontaneous pneumothorax, hypoplasia of the pulmonary veins, pulmonary lymphangiectasia, and thickening of the pulmonary arteries with subsequent pulmonary hypertension.…”

“… 2 , 3 , 4 , 5 Surgical repair of common pulmonary vein atresia has been reported in several cases, and although establishment of a patent anastomosis between the common pulmonary vein to the left atrium can be achieved (similar to surgical repair of total anomalous pulmonary venous connection), the results of surgery in common pulmonary vein atresia have varied, with morbidity and mortality largely related to associated lung disease. 2 , 3 , 4 , 5 , 6 There are strong associations between pulmonary vein atresia and spontaneous pneumothorax, hypoplasia of the pulmonary veins, pulmonary lymphangiectasia, and thickening of the pulmonary arteries with subsequent pulmonary hypertension. As a result, many of the patients reported in the literature required early extracorporeal membrane oxygenation, prolonged ventilation, and hospitalization for respiratory failure, in addition to showing evidence of chronic lung disease, effusions/chylothorax, and late death due to respiratory decompensation.…”