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Pulmonary venous occlusion

MedGen UID:
907103
Concept ID:
C4280802
Finding
Synonyms: Pulmonary venoocclusive disease; Pulmonary venous stenosis
 
HPO: HP:0006518

Definition

Substantial narrowing or blockage of small pulmonary veins as a result of disorganized smooth muscle hypertrophy and collagen matrix deposition. [from HPO]

Conditions with this feature

Familial pulmonary capillary hemangiomatosis
MedGen UID:
90956
Concept ID:
C0340848
Disease or Syndrome
Pulmonary venoocclusive disease-2 is an autosomal recessive subtype of primary pulmonary hypertension (PPH; see 178600). It is characterized histologically by widespread fibrous intimal proliferation of septal veins and preseptal venules, and is frequently associated with pulmonary capillary dilatation and proliferation. The disorder can cause occult alveolar hemorrhage. High-resolution CT imaging of the chest shows patchy centrilobular ground-glass opacities, septal lines, and lymph node enlargement (summary by Eyries et al., 2014). For a discussion of genetic heterogeneity of pulmonary venoocclusive disease, see PVOD1 (265450).
Pulmonary venoocclusive disease 1
MedGen UID:
854500
Concept ID:
C3887658
Disease or Syndrome
Pulmonary venoocclusive disease primarily affects the postcapillary venous pulmonary vessels and may involve significant pulmonary capillary dilation and/or proliferation. PVOD is an uncommon cause of pulmonary artery hypertension (PPH; see 178600), a severe condition characterized by elevated pulmonary artery pressure leading to right heart failure and death. PVOD accounts for 5 to 10% of 'idiopathic' PPH and has an estimated incidence of 0.1 to 0.2 cases per million. The pathologic hallmark of PVOD is the extensive and diffuse occlusion of pulmonary veins by fibrous tissue, with intimal thickening present in venules and small veins in lobular septa and, rarely, larger veins. Definitive diagnosis of PVOD requires histologic analysis of a lung sample, although surgical lung biopsy is often too invasive for these frail patients. Patients with PVOD respond poorly to available therapy, therefore it is crucial to distinguish PVOD from other forms of PPH. Radiologic characteristics suggestive of PVOD on high-resolution CT of the chest include nodular ground-glass opacities, septal lines, and lymph node enlargement. In addition, because PVOD mainly affects postcapillary vasculature, it causes chronic elevation of pulmonary capillary pressure and thus promotes occult alveolar hemorrhage, which may be a characteristic feature of PVOD (summary by Montani et al., 2008). Genetic Heterogeneity of Pulmonary Venoocclusive Disease See also PVOD2 (234810), caused by mutation in the EIF2AK4 gene (609280) on chromosome 15q15.

Professional guidelines

PubMed

Solinas S, Boucly A, Beurnier A, Kularatne M, Grynblat J, Eyries M, Dorfmüller P, Sitbon O, Humbert M, Montani D
Expert Rev Respir Med 2023 Jul-Dec;17(8):635-649. Epub 2023 Aug 21 doi: 10.1080/17476348.2023.2247989. PMID: 37578057
Franchi-Abella S, Gonzales E, Ackermann O, Branchereau S, Pariente D, Guérin F; International Registry of Congenital Portosystemic Shunt members
Abdom Radiol (NY) 2018 Aug;43(8):2023-2036. doi: 10.1007/s00261-018-1619-8. PMID: 29730740
Baskin JL, Pui CH, Reiss U, Wilimas JA, Metzger ML, Ribeiro RC, Howard SC
Lancet 2009 Jul 11;374(9684):159-69. doi: 10.1016/S0140-6736(09)60220-8. PMID: 19595350Free PMC Article

Recent clinical studies

Etiology

Takeda Y, Takeda Y, Yamamoto K, Tomimoto S, Tani T, Narita H, Ohte N, Kimura G
BMC Pulm Med 2011 Oct 6;11:47. doi: 10.1186/1471-2466-11-47. PMID: 21974838Free PMC Article
Nehra D, Liberman M, Vagefi PA, Evans N, Inglessis I, Kradin RL, Ono J, Kanarek DJ, Gaissert HA
Ann Thorac Surg 2009 Jan;87(1):292-5. doi: 10.1016/j.athoracsur.2008.06.060. PMID: 19101316
Reisner SA, Rinkevich D, Markiewicz W, Tatarsky I, Brenner B
Am J Med 1992 Nov;93(5):498-504. doi: 10.1016/0002-9343(92)90576-w. PMID: 1442851

Diagnosis

Venkataraman R, Loyalka P, Encarnacion C, Safi HJ, Cheema FH, Rajagopal K
Ann Thorac Surg 2023 Feb;115(2):e33-e35. Epub 2022 Mar 22 doi: 10.1016/j.athoracsur.2022.03.005. PMID: 35331701
Nehra D, Liberman M, Vagefi PA, Evans N, Inglessis I, Kradin RL, Ono J, Kanarek DJ, Gaissert HA
Ann Thorac Surg 2009 Jan;87(1):292-5. doi: 10.1016/j.athoracsur.2008.06.060. PMID: 19101316
Massumi A, Woods L, Mullins CE, Nasser WK, Hall RJ
Am J Cardiol 1981 Sep;48(3):585-9. doi: 10.1016/0002-9149(81)90092-8. PMID: 7270465

Therapy

Venkataraman R, Loyalka P, Encarnacion C, Safi HJ, Cheema FH, Rajagopal K
Ann Thorac Surg 2023 Feb;115(2):e33-e35. Epub 2022 Mar 22 doi: 10.1016/j.athoracsur.2022.03.005. PMID: 35331701
Fernández-Navarro L, Moya-Sánchez E, Segura-Rodríguez D, Ruiz-Carazo E
Arch Bronconeumol (Engl Ed) 2018 Jun;54(6):338-340. Epub 2018 Feb 13 doi: 10.1016/j.arbres.2017.12.010. PMID: 29422339
Fender EA, Packer DL, Holmes DR Jr
EuroIntervention 2016 May 17;12 Suppl X:X31-X34. doi: 10.4244/EIJV12SXA7. PMID: 27174109
Hayashi M, Minezawa T, Imaizumi K, Sobue Y, Watanabe E, Ozaki Y, Okazawa M
Respiration 2013;86(3):252-3. Epub 2013 Aug 27 doi: 10.1159/000351598. PMID: 23988516
Nehra D, Liberman M, Vagefi PA, Evans N, Inglessis I, Kradin RL, Ono J, Kanarek DJ, Gaissert HA
Ann Thorac Surg 2009 Jan;87(1):292-5. doi: 10.1016/j.athoracsur.2008.06.060. PMID: 19101316

Prognosis

West TE, Keene CD, Schmidt RA, Smith KD
Respir Care 2013 May;58(5):e56-9. doi: 10.4187/respcare.02084. PMID: 23050515
Nehra D, Liberman M, Vagefi PA, Evans N, Inglessis I, Kradin RL, Ono J, Kanarek DJ, Gaissert HA
Ann Thorac Surg 2009 Jan;87(1):292-5. doi: 10.1016/j.athoracsur.2008.06.060. PMID: 19101316
McConnell ME, Pacifico AD, Johnson WH Jr, Mroczek E
Pediatr Cardiol 1994 Mar-Apr;15(2):95-9. doi: 10.1007/BF00817617. PMID: 7997423
Reisner SA, Rinkevich D, Markiewicz W, Tatarsky I, Brenner B
Am J Med 1992 Nov;93(5):498-504. doi: 10.1016/0002-9343(92)90576-w. PMID: 1442851

Clinical prediction guides

Reisner SA, Rinkevich D, Markiewicz W, Tatarsky I, Brenner B
Am J Med 1992 Nov;93(5):498-504. doi: 10.1016/0002-9343(92)90576-w. PMID: 1442851

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