Congenital pulmonary venous stenosis

Definition:

Congenital pulmonary venous stenosis is a rare condition with fibromyxoid intimal proliferation leading to obstruction of the large pulmonary veins and of blood flow from the lungs to the left atrium. This results in elevated pulmonary venous pressure and pulmonary edema, pulmonary hypertension, potentially cardiac failure, and death. Clinical signs of the disease include failure to thrive, dyspnea, hemoptysis, recurrent respiratory tract infections, cyanosis, and subcostal retractions.

References:

[1]. Patcharapong Suntharos, et al. Treatment of Congenital and Acquired Pulmonary Vein Stenosis. Curr Cardiol Rep. 2020 Oct 3;22(11):153. [Content Brief]

[2]. Tilman Humpl, et al. The many faces and outcomes of pulmonary vein stenosis in early childhood. Pediatr Pulmonol. 2021 Mar;56(3):649-655. [Content Brief]

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