congenital pulmonary veins atresia or stenosis https://www.malacards.org/card/congenital_pulmonary_veins_atresia_or_stenosis pulmonary vein stenosis UMLS:C5680865 MEDGEN:1826176 SCTID:234062003 MONDO:0017864 Congenital pulmonary vein (PV) stenosis or atresia is a rare progressive life-threatening great vessels anomaly characterized by narrowing and obstruction of one or more normally positioned PV at their junction with the left atrium, that usually presents during early infancy with dyspnea, tachypnea, and repeated pulmonary infections, and eventually, when all PV of one lung are affected, results in pulmonary hypertension (PH) and consecutive pulmonary arterial hypertension (PAH). It may manifest as an isolated lesion or associated with other cardiac defects such as congenital pulmonary venous return anomaly and septal defects. NANDO:2200273 pulmonary veins stenosis GARD:0004598 NANDO:1200964 Orphanet:3188 icd11.foundation:469101490 congenital anomaly of the great arteries