Pulmonary vein stenosis

Abstract

Pulmonary vein stenosis is a relatively rare, complex, challenging, and often lethal condition among pediatric heart diseases, with a guarded prognosis. The pathophysiology is poorly understood, and the search for an effective treatment remains a source of frustration. There are two different forms of pulmonary vein stenosis. The primary “congenital”from, which is isolated or associated with other congenital heart diseases, has a severe prognosis. Acquired or secondary pulmonary vein stenosis, which occurs most commonly after repair of total anomalous pulmonary venous return (TAPVR), fosters a better prognosis. This chapter will discuss embryological, anatomical, physiological, clinical, and diagnostic details; medical and surgical management; and outcomes of the disease.