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Misalignment of the pulmonary veins

MedGen UID:
Concept ID:
Anatomical Abnormality
HPO: HP:0033186


The term is commonly used to describe a putative abnormal location of pulmonary vein branches adjacent to pulmonary arteries within the same adventitial sheath. However, evidence has been provided that the vessels in question are not pulmonary veins, however represent dilated bronchial veins. [from HPO]

Term Hierarchy

Conditions with this feature

Surfactant metabolism dysfunction, pulmonary, 1
MedGen UID:
Concept ID:
Disease or Syndrome
Inborn errors of pulmonary surfactant metabolism are genetically heterogeneous disorders resulting in severe respiratory insufficiency or failure in full-term neonates or infants. These disorders are associated with various pathologic entities, including pulmonary alveolar proteinosis (PAP), desquamative interstitial pneumonitis (DIP), or cellular nonspecific interstitial pneumonitis (NSIP) (Clark and Clark, 2005). A clinically similar disorder characterized by respiratory distress (267450) can affect preterm infants, who show developmental deficiency of surfactant. Acquired PAP (610910) is an autoimmune disorder characterized by the presence of autoantibodies to CSF2 (138960). Genetic Heterogeneity of Pulmonary Surfactant Metabolism Dysfunction See also SMDP2 (610913), caused by mutation in the SPTPC gene (178620) on 8p21; SMDP3 (610921), caused by mutation in the ABCA3 gene (601615) on 16p13; SMDP4 (300770), caused by mutation in the CSF2RA gene (306250) on Xp22; and SMDP5 (614370), caused by mutation in the CSF2RB gene (138981) on 22q12.
Alveolar capillary dysplasia with pulmonary venous misalignment
MedGen UID:
Concept ID:
Congenital Abnormality
Congenital alveolar capillary dysplasia with misalignment of pulmonary veins (ACDMPV) is characterized histologically by failure of formation and ingrowth of alveolar capillaries that then do not make contact with alveolar epithelium, medial muscular thickening of small pulmonary arterioles with muscularization of the intraacinar arterioles, thickened alveolar walls, and anomalously situated pulmonary veins running alongside pulmonary arterioles and sharing the same adventitial sheath. Less common features include a reduced number of alveoli and a patchy distribution of the histopathologic changes. The disorder is associated with persistent pulmonary hypertension of the neonate and shows varying degrees of lability and severity (Boggs et al., 1994). Affected infants present with respiratory distress resulting from pulmonary hypertension in the early postnatal period, and the disease is uniformly fatal within the newborn period (Vassal et al., 1998). Additional features of ACDMPV include multiple congenital anomalies affecting the cardiovascular, gastrointestinal, genitourinary, and musculoskeletal systems, as well as disruption of the normal right-left asymmetry of intrathoracic or intraabdominal organs (Sen et al., 2004).

Professional guidelines


Onda T, Akimoto T, Hayasaka I, Ikeda M, Furuse Y, Ando A, Nakamura Y, Honjo R, Manabe A, Furuta I, Cho K
Early Hum Dev 2021 Apr;155:105323. Epub 2021 Jan 26 doi: 10.1016/j.earlhumdev.2021.105323. PMID: 33578219

Recent clinical studies


Lin YB, Xia B, Cao J, Tang ZJ
J Int Med Res 2022 Sep;50(9):3000605221126876. doi: 10.1177/03000605221126876. PMID: 36173014Free PMC Article


Reiter J, Szafranski P, Breuer O, Perles Z, Dagan T, Stankiewicz P, Kerem E
Pediatr Pulmonol 2016 Sep;51(9):921-7. Epub 2016 May 4 doi: 10.1002/ppul.23425. PMID: 27145217

Clinical prediction guides

Reiter J, Szafranski P, Breuer O, Perles Z, Dagan T, Stankiewicz P, Kerem E
Pediatr Pulmonol 2016 Sep;51(9):921-7. Epub 2016 May 4 doi: 10.1002/ppul.23425. PMID: 27145217

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