Congenital pulmonary vein atresia

Abstract

Congenital pulmonary vein atresia (PVA) is a rare anomaly in clinic.Patients with congenital PVA are usually symptomatic and present with recurrent episodes of pneumonia or haemoptysis in infancy or childhood.Because PVA often occurs in the setting of other congenital heart lesions, so repeated infection of lung might be consi-dered to associate with left-to-right intracardiac shunt with increased pulmonary blood, but ignore further examination of more rare cause.Diagnosis of PVA is based primarily on imaging test, chest radiograph, lung CT and fiberoptic bronchoscopy might reveal some indirect characteristic changes of the disease.Three dimensional contrast enhanced cardiac CT can make a definite diagnosis.The disease is difficult to cure at present, the aim of the treatment is to preserve lung function.The prognosis is associated with the number of involved pulmonary veins. Key words: Pulmonary vein atresia; Congenital heart disease; Pneumonia; Haemoptysis