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Total anomalous pulmonary vein drainage: Report of an autopsy case associated with atresia of the common pulmonary vein and left superior pulmonary vein
Author(s) -
Yamada Sohsuke,
Hisaoka Masanori,
Wang KeYong,
Ding Yan,
Guo Xin,
Shimajiri Shohei,
Matsumoto Hayato,
Shirakawa Yoshitsugu,
Sasaguri Yasuyuki
Publication year - 2011
Publication title -
pathology international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.73
H-Index - 74
eISSN - 1440-1827
pISSN - 1320-5463
DOI - 10.1111/j.1440-1827.2010.02617.x
Subject(s) - medicine , left pulmonary vein , autopsy , pulmonary vein , atresia , pulmonary atresia , vein , superior vena cava , left pulmonary artery , lung , radiology , pulmonary artery , surgery , cardiology , ablation
We describe the clinicopathological features of a case of total anomalous pulmonary vein drainage (TAPVD) associated with atresia of the common pulmonary vein (ACPV). A male Japanese infant born at 37 weeks of gestation demonstrated apnea and severe respiratory acidosis immediately after delivery. The patient died of hypoxemic respiratory failure 6 days after birth despite the initiation of artificial ventilation and administration of a surfactant. Autopsy showed the bilateral inferior pulmonary veins joined with a blind confluence, representing ACPV, accompanied by atresia of the left superior pulmonary vein. Moreover, the anomalous and small right superior pulmonary vein drained into the superior vena cava, consistent with partial and supracardiac type TAPVD. A histological examination of the lungs exhibited diffuse dilation of the lymphatic channels in the peribronchial, interlobular, hilar and focally, subpleural areas. The channels were lined with flattened endothelium which was immunohistochemically positive for D2‐40. These findings conformed to a secondary form of pulmonary lymphangiectasis due to the congenital cardiovascular anomalies, including TAPVD and ACPV. To the authors' knowledge, this is the first case of TAPVD associated with ACPV, atresia of left superior pulmonary vein and pulmonary lymphangiectasis.