Isolated Unilateral Pulmonary Vein Atresia in Adult Patients: A Case Report and Literature Review
DOI:
https://doi.org/10.1532/HSF98.20101078Abstract
Unilateral pulmonary vein atresia is a rare abnormality that usually presents in infants with recurrent hemoptysis and pneumonia. Presentation in adulthood without additional congenital heart disease is rare but does occur. Anatomic variations in the pulmonary vessels that supply and drain the affected lung can explain the mildly symptomatic process. The diagnosis of isolated unilateral pulmonary vein atresia is usually made angiographically. Pneumonectomy is indicated once symptoms or complications are present so that irreversible pulmonary hypertension can be avoided.References
Artero MI, Serrano PF, Padin Marin MI, Serrano RF. 2008. Congenital unilateral pulmonary vein atresia: imaging findings [in Spanish]. Radiologia 50:82-5.nCabrera A, Vazquez C, Lekuona I. 1985. Isolated atresia of the left pulmonary veins. Int J Cardiol 7:298-302.nCullen S, Deasy PF, Tempany, Duff DF. 1990. Isolated pulmonary vein atresia. Br Heart J 63:350-4.nHarrison JK, Hearne SE, Baker WA, et al. 1996. Esophageal varices in association with unilateral pulmonary vein atresia. Cathet Cardiovasc Diagn 38:387-92.nHeyneman LE, Nolan RL, Harrison JK, McAdams HP. 2001. Congenital unilateral pulmonary vein atresia: radiologic findings in three adult patients. AJR Am J Roentgenol 177:681-5.nKingston HM, Patel RG, Watson GH. 1983. Unilateral absence or extreme hypoplasia of pulmonary veins. Br Heart J 49:148-53.nMataciunas M, Gumbiene L, Cibiras S, Tarutis V, Tamosiunas AE. 2009. CT angiography of mildly symptomatic, isolated, unilateral right pulmonary vein atresia. Pediatr Radiol 39:1087-90.nPourmoghadam KK, Moore JW, Khan M, et al. 2003. Congenital unilateral pulmonary venous atresia: definitive diagnosis and treatment. Pediatr Cardiol 24:73-9.nTissot C, Corbelli R, Aggoun Y, Beghetti M, da Cruz E. 2008. Bronchoscopic diagnosis of asymptomatic unilateral pulmonary vein atresia in an infant. Pediatr Cardiol 29:976-9.nUssia GP, Marasini M, Rimini A, Pongiglione G. 2004. Atresia of right pulmonary veins with intact atrial septum and major aorto-pulmonary collateral treated with percutaneous stent implantation and embolization. J Interv Cardiol 17:183-7.nvan der Werf TS, Mannes GP, Hew JM, et al. 1994. Improved gas exchange after pneumonectomy in an adult with incomplete pulmonary vein atresia. Thorax 49:723-5.n