Bronchial Varices in Congenital Unilateral Pulmonary Vein Atresia

1 Department of Respiratory Medicine and.
American Journal of Respiratory and Critical Care Medicine (Impact Factor: 13). 06/2013; 187(11):1267-1268. DOI: 10.1164/rccm.201211-1974IM
Source: PubMed
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    • "Physiologic reversal of flow to the pulmonary veins and increased flow through the esophageal veins results in the formation of “downhill” paraesophageal varices rather than the uphill esophageal varices commonly encountered in portal venous hypertension. Additionally, systemic-pulmonary artery collateralization as seen in this case has also been reported in cases of isolated pulmonary vein atresia [7]. "
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    ABSTRACT: Cor triatriatum sinistrum is a rare congenital disorder defined as a division of the left atrium by a diaphragmatic membrane resulting in two left atrial chambers. The membranous division of the atrium can be partial or complete and can affect either atrium, with involvement of the right atrium referred to as cor triatriatum dexter. The presence of fenestrations within the membrane allows for communication and forward passage of blood into the true atrium. Absence of fenestrations leads to early symptomatic engorgement of the lungs. We report the case of a young adult male presenting with recurrent hematemesis due to variceal bleeding. On CT imaging the patient was found to have cor triatriatum sinistrum, with a vertical membrane resulting in total obstruction of the pulmonary venous drainage on the right, with normal pulmonary venous drainage on the left. There was extensive pulmonary-systemic arterial collateralization to the right lung suggesting retrograde filling of the right pulmonary artery with effective flow reversal in the right lung.
    12/2013; 2013(4):538259. DOI:10.1155/2013/538259
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    ABSTRACT: We present a rare case of hemoptysis secondary to isolated unilateral pulmonary vein atresia. Isolated pulmonary vein atresia is a rare condition in which patients typically acquire a diagnosis in infancy and early childhood [1] and [2]. Our patient presented during puberty with several previous episodes of hemoptysis prior to her admission and diagnosis. The initial diagnosis was suspected in our patient from chest computerized tomography (CT), and confirmed with cardiac catheterization and pulmonary angiography. Treatment aim is to preserve lung function and minimize irreversible pulmonary remodeling [2] and [3]. Conservative monitoring can be considered with milder or asymptomatic cases, while others may require preoperative collateral artery banding, surgical anastomosis between the pulmonary vein (PV) & left atrium (LA) and even pneumonectomy [2].
    Respiratory Medicine Case Reports 05/2015; 15. DOI:10.1016/j.rmcr.2015.05.009