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Congenital pulmonary veins atresia or stenosis

Synonym(s) -
Prevalence Unknown
Inheritance Not applicable
Age of onset Childhood
  • Q26.8
  • C0265915
  • C0344679
MeSH -
MedDRA -


Pulmonary veins (PV) stenosis is an heterogeneous entity including stenosis of normally positioned individual PV and PV obstruction accompanying total anomalous pulmonary venous connection. Congenital stenosis or atresia of individual PV is a very rare anomaly encountered in about 0.5% of autopsy in children. It may manifest as an isolated lesion or associated with other cardiac defects (more than 50% of cases). In all cases of congenital and acquired stenosis of PV, the lesion is progressive and includes narrowing of one or more PV at their junction with the left atrium, or hypoplasia with narrowed intra- or extrapulmonary portions at variable distances. Most commonly, all PV of one lung are affected, causing pulmonary hypertension (PH) and consecutive pulmonary arterial hypertension (PAH). By contrast, lesions of a single PV may not lead to PH. In case of PH and PAH, severe symptoms of pulmonary edema occur in early infancy including dyspnea, tachypnea, repeated pulmonary infections, hemoptysis, and in the most severe cases cyanosis and signs of right heart failure. Etiology of congenital stenosis or atresia of individual PV is a fibrotic stenosis. Acquired stenosis of PV may be due to constrictive pericarditis, mediastinitis, tuberculosis, obstructive tumors or operative scar. Angiocardiography may allow identification of the precise anatomy of PV stenosis. Without surgical treatment, patients with significant stenosis of all or most PV die before reaching adulthood and often much sooner. Overall prognosis is bad when at least 50% of PV are stenotic.

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