Images in Pulmonary, Critical Care, Sleep Medicine
and the Sciences
Bronchial Varices in Congenital Unilateral
Pulmonary Vein Atresia
Mengshu Cao1*, Hourong Cai1*, Jingjing Ding1, Yi Zhuang1, and Zhengge Wang2
1Department of Respiratory Medicine and2Department of Radiology, Nanjing Drum Tower Hospital,
Nanjing University Medical School, Nanjing, China
and a small left hemithorax (Figure 1A). CT angiography showed a small left main pulmonary artery, complete absence of the
pulmonary veins on the left side, and three collateral arteries supplying the left lung from descending aorta (Figures 1B and 1C).
Bronchoscopic findings revealed mucosal varices and tracheal angioma, mucosal hyperemia, hypertrophy, increased secretions,
narrowed lumen, and varices of the left bronchial tree, whereas only hyperemia, increased secretions, and varices were seen on
the right side (Figures 2A and 2B) (see video in the online supplement). Echocardiography demonstrated left cor triatriatum, secun-
dum atrial septal defect, and moderate pulmonary hypertension. Left pulmonary vein atresia with cardiac defects was demonstrated
by cardiac catheterization and pulmonary angiography. The diagnosis of congenital unilateral pulmonary vein atresia with cardiac
defects was made based on the findings from CT angiography, bronchoscopy, echocardiography, and cardiac catheterization (1, 2).
Patients with congenital unilateral pulmonary vein atresia usually present in infancy or childhood with recurrent episodes of pneu-
monia or hemoptysis (3). Presentation in adulthood is rare but does occur (2). About 32% of patients accompanied with cardiac
defects (4). Bronchial varix due to pulmonary venous obstruction may be a rare cause of hemoptysis (3). Bronchial varices
presented not only in the affected side, but also in the unaffected side, which may be due to compensatory expanding of the
right bronchial veins. Rupture of the dilated bronchial veins was considered as the mechanism of hemoptysis (3). Bronchoscope
lung biopsy should be avoided in these cases, as it could lead to fatal hemoptysis. The circuitous collateral arteries were identified as
supplying the left lung from descending aorta in our patient. Blood in the systemic collateral vessels flowed retrogradely into the
ipsilateral, then contralateral, pulmonary arteries, then coursed through the contralateral lung into the left atrium (2). Bronchial
varix may serve as a hallmark clinical feature of unilateral pulmonary vein atresia.
Figure 1. Chest computed tomography (CT) of the patient. (A) Chest CT showed diffuse thickening of interlobular septa (arrows) and a small left
hemithorax. (B and C) CT angiography images showed a small left main pulmonary artery, complete absence of the pulmonary veins on the left
side, and three circuitous collateral arteries supplying the left lung from descending aorta (arrows). LPA ¼ left pulmonary artery; RPA ¼ right
pulmonary artery; RPV ¼ right pulmonary vein.
*M.C. and H.C. contributed equally to this report.
H.C., J.D., and Y.Z. participated in clinical management. Z.W. provided the images.
This article has a video supplement, which is accessible from this issue’s table of
contents at www.atsjournals.org
Am J Respir Crit Care Med
Copyright ª 2013 by the American Thoracic Society
Internet address: www.atsjournals.org
Vol 187, Iss. 11, pp 1267–1268, Jun 1, 2013