Congenital pulmonary veins atresia or stenosis

Congenital pulmonary veins atresia or stenosis (also known as congenital pulmonary vein stenosis, or PVS) is a rare cardiovascular malformation in which one or more of the pulmonary veins — the blood vessels that carry oxygenated blood from the lungs back to the heart — are abnormally narrowed (stenosis) or completely closed (atresia) from birth. This obstruction impedes normal blood flow from the lungs to the left atrium of the heart, leading to pulmonary venous hypertension and, subsequently, pulmonary arterial hypertension. The condition primarily affects the cardiovascular and pulmonary systems. Key clinical features include progressive respiratory distress, recurrent pulmonary infections, failure to thrive, cyanosis (bluish discoloration of the skin), and signs of pulmonary hypertension such as right heart failure. Symptoms typically present in infancy or early childhood and can be severe, particularly when multiple veins are involved (bilateral disease). The condition may occur in isolation or in association with other congenital heart defects, such as atrial septal defect, ventricular septal defect, or total anomalous pulmonary venous return. Treatment remains challenging and outcomes are often guarded. Surgical repair or balloon angioplasty with or without stent placement may be attempted to relieve the obstruction, but restenosis (re-narrowing) is a frequent and significant problem. More recently, the use of drug-eluting stents and adjunctive antiproliferative agents (such as bevacizumab and imatinib) has been explored in some centers to reduce the risk of intimal proliferation and restenosis. In severe, progressive cases unresponsive to intervention, lung transplantation may be considered. Despite advances, congenital pulmonary vein stenosis carries a high morbidity and mortality rate, particularly in bilateral or diffuse forms of the disease.

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