Bilateral Congenital Pulmonary Vein Stenosis with a Normal Connection

Introduction Congenital pulmonary vein stenosis (CPVS) is one of cardiac anomalies producing generalized pulmonary venous hypertension such as mitral stenosis, a supravalvar mitral ring, mitral regurgitation, and total anomalous pulmonary venous connection. The prognosis is poor with the development of progressive pulmonary venous congestion followed by pulmonary arterial hypertension and eventual death. The disorder is frequently overlooked during clinical examination and routine echocardiography because it is rare. Here, we report a patient with a history of cough, tachypnea and hemoptysis who was diagnosed with CPVS with a normal connection. The diagnosis was suspected based on an abnormal thoracic roentgenogram and confirmed by echocardiography, lung perfusion scanning, computerized tomography (CT) with angiogram, and cardiac catheterization with angiography.