Bilateral Congenital Pulmonary Vein Stenosis with a Normal Connection

ABSTRACT

Congenital pulmonary vein stenosis (CPVS) with an anatomically normal connection is a rare cardiac malformation. This cardiac anomaly usually is accompanied by other cardiac abnormalities. Bilateral CPVS is a more severe form of a CPVS and it usually leads to progressive pulmonary hypertension and death if it is not treated. Here, we report a patient with a history of cough, tachypnea and hemoptysis and suspected CPVS due to an abnormal thoracic roentgenogram with dilated right pulmonary arteries and pulmonary cornus. The two-dimensional and color Doppler echocardiography demonstrated three stenosed pulmonary veins connected to the left atrium. However, the fourth vessel could not be visualized. There were no other cardiac malformations associated with the CPVS. The Technetium-99m macro-aggregate lung perfusion scan showed absent or diminished perfusion to the affected lobes of the lungs. In addition, the chest computed tomography with angiogram and cardiac catheterization confirmed the findings of the echocardiogram.