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Pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis

MedGen UID:
785618
Concept ID:
C3698354
Disease or Syndrome
Synonyms: pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis; PVOD and/or PCH
 
Monarch Initiative: MONDO:0018554
Orphanet: ORPHA431353

Definition

A disorder that constitutes a rare subgroup of rare pulmonary hypertension characterized by obliterative fibrosis of the small pulmonary veins and venules and/or capillary infiltration of the pulmonary interstitium leading to increased pulmonary vascular resistance and right ventricular dysfunction. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis

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
Ruopp NF, Cockrill BA
JAMA 2022 Apr 12;327(14):1379-1391. doi: 10.1001/jama.2022.4402. PMID: 35412560
Huertas A, Girerd B, Dorfmuller P, O'Callaghan D, Humbert M, Montani D
Expert Rev Respir Med 2011 Apr;5(2):217-29; quiz 230-1. doi: 10.1586/ers.11.15. PMID: 21510732

Recent clinical studies

Etiology

Ruopp NF, Cockrill BA
JAMA 2022 Apr 12;327(14):1379-1391. doi: 10.1001/jama.2022.4402. PMID: 35412560
Kantarjian HM, DeAngelo DJ, Stelljes M, Liedtke M, Stock W, Gökbuget N, O'Brien SM, Jabbour E, Wang T, Liang White J, Sleight B, Vandendries E, Advani AS
Cancer 2019 Jul 15;125(14):2474-2487. Epub 2019 Mar 28 doi: 10.1002/cncr.32116. PMID: 30920645Free PMC Article
Simonneau G, Montani D, Celermajer DS, Denton CP, Gatzoulis MA, Krowka M, Williams PG, Souza R
Eur Respir J 2019 Jan;53(1) Epub 2019 Jan 24 doi: 10.1183/13993003.01913-2018. PMID: 30545968Free PMC Article
Aguilar-Guisado M, Espigado I, Martín-Peña A, Gudiol C, Royo-Cebrecos C, Falantes J, Vázquez-López L, Montero MI, Rosso-Fernández C, de la Luz Martino M, Parody R, González-Campos J, Garzón-López S, Calderón-Cabrera C, Barba P, Rodríguez N, Rovira M, Montero-Mateos E, Carratalá J, Pérez-Simón JA, Cisneros JM
Lancet Haematol 2017 Dec;4(12):e573-e583. Epub 2017 Nov 15 doi: 10.1016/S2352-3026(17)30211-9. PMID: 29153975
Kligerman SJ, Henry T, Lin CT, Franks TJ, Galvin JR
Radiographics 2015 Sep-Oct;35(5):1360-80. Epub 2015 Aug 14 doi: 10.1148/rg.2015140308. PMID: 26274445

Diagnosis

Ruopp NF, Cockrill BA
JAMA 2022 Apr 12;327(14):1379-1391. doi: 10.1001/jama.2022.4402. PMID: 35412560
Simonneau G, Montani D, Celermajer DS, Denton CP, Gatzoulis MA, Krowka M, Williams PG, Souza R
Eur Respir J 2019 Jan;53(1) Epub 2019 Jan 24 doi: 10.1183/13993003.01913-2018. PMID: 30545968Free PMC Article
Montani D, Lau EM, Dorfmüller P, Girerd B, Jaïs X, Savale L, Perros F, Nossent E, Garcia G, Parent F, Fadel E, Soubrier F, Sitbon O, Simonneau G, Humbert M
Eur Respir J 2016 May;47(5):1518-34. Epub 2016 Mar 23 doi: 10.1183/13993003.00026-2016. PMID: 27009171
Richardson PG, Riches ML, Kernan NA, Brochstein JA, Mineishi S, Termuhlen AM, Arai S, Grupp SA, Guinan EC, Martin PL, Steinbach G, Krishnan A, Nemecek ER, Giralt S, Rodriguez T, Duerst R, Doyle J, Antin JH, Smith A, Lehmann L, Champlin R, Gillio A, Bajwa R, D'Agostino RB Sr, Massaro J, Warren D, Miloslavsky M, Hume RL, Iacobelli M, Nejadnik B, Hannah AL, Soiffer RJ
Blood 2016 Mar 31;127(13):1656-65. Epub 2016 Jan 29 doi: 10.1182/blood-2015-10-676924. PMID: 26825712Free PMC Article
Kligerman SJ, Henry T, Lin CT, Franks TJ, Galvin JR
Radiographics 2015 Sep-Oct;35(5):1360-80. Epub 2015 Aug 14 doi: 10.1148/rg.2015140308. PMID: 26274445

Therapy

Ruopp NF, Cockrill BA
JAMA 2022 Apr 12;327(14):1379-1391. doi: 10.1001/jama.2022.4402. PMID: 35412560
Kantarjian HM, DeAngelo DJ, Stelljes M, Liedtke M, Stock W, Gökbuget N, O'Brien SM, Jabbour E, Wang T, Liang White J, Sleight B, Vandendries E, Advani AS
Cancer 2019 Jul 15;125(14):2474-2487. Epub 2019 Mar 28 doi: 10.1002/cncr.32116. PMID: 30920645Free PMC Article
Price LC, Seckl MJ, Dorfmüller P, Wort SJ
Eur Respir Rev 2019 Mar 31;28(151) Epub 2019 Feb 6 doi: 10.1183/16000617.0065-2018. PMID: 30728162Free PMC Article
Aguilar-Guisado M, Espigado I, Martín-Peña A, Gudiol C, Royo-Cebrecos C, Falantes J, Vázquez-López L, Montero MI, Rosso-Fernández C, de la Luz Martino M, Parody R, González-Campos J, Garzón-López S, Calderón-Cabrera C, Barba P, Rodríguez N, Rovira M, Montero-Mateos E, Carratalá J, Pérez-Simón JA, Cisneros JM
Lancet Haematol 2017 Dec;4(12):e573-e583. Epub 2017 Nov 15 doi: 10.1016/S2352-3026(17)30211-9. PMID: 29153975
Montani D, Lau EM, Dorfmüller P, Girerd B, Jaïs X, Savale L, Perros F, Nossent E, Garcia G, Parent F, Fadel E, Soubrier F, Sitbon O, Simonneau G, Humbert M
Eur Respir J 2016 May;47(5):1518-34. Epub 2016 Mar 23 doi: 10.1183/13993003.00026-2016. PMID: 27009171

Prognosis

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
Kantarjian HM, DeAngelo DJ, Stelljes M, Liedtke M, Stock W, Gökbuget N, O'Brien SM, Jabbour E, Wang T, Liang White J, Sleight B, Vandendries E, Advani AS
Cancer 2019 Jul 15;125(14):2474-2487. Epub 2019 Mar 28 doi: 10.1002/cncr.32116. PMID: 30920645Free PMC Article
Price LC, Seckl MJ, Dorfmüller P, Wort SJ
Eur Respir Rev 2019 Mar 31;28(151) Epub 2019 Feb 6 doi: 10.1183/16000617.0065-2018. PMID: 30728162Free PMC Article
Simonneau G, Montani D, Celermajer DS, Denton CP, Gatzoulis MA, Krowka M, Williams PG, Souza R
Eur Respir J 2019 Jan;53(1) Epub 2019 Jan 24 doi: 10.1183/13993003.01913-2018. PMID: 30545968Free PMC Article
Montani D, Lau EM, Dorfmüller P, Girerd B, Jaïs X, Savale L, Perros F, Nossent E, Garcia G, Parent F, Fadel E, Soubrier F, Sitbon O, Simonneau G, Humbert M
Eur Respir J 2016 May;47(5):1518-34. Epub 2016 Mar 23 doi: 10.1183/13993003.00026-2016. PMID: 27009171

Clinical prediction guides

Ruopp NF, Cockrill BA
JAMA 2022 Apr 12;327(14):1379-1391. doi: 10.1001/jama.2022.4402. PMID: 35412560
Manaud G, Nossent EJ, Lambert M, Ghigna MR, Boët A, Vinhas MC, Ranchoux B, Dumas SJ, Courboulin A, Girerd B, Soubrier F, Bignard J, Claude O, Lecerf F, Hautefort A, Florio M, Sun B, Nadaud S, Verleden SE, Remy S, Anegon I, Bogaard HJ, Mercier O, Fadel E, Simonneau G, Vonk Noordegraaf A, Grünberg K, Humbert M, Montani D, Dorfmüller P, Antigny F, Perros F
Am J Respir Cell Mol Biol 2020 Jul;63(1):118-131. doi: 10.1165/rcmb.2019-0015OC. PMID: 32209028
Kantarjian HM, DeAngelo DJ, Stelljes M, Liedtke M, Stock W, Gökbuget N, O'Brien SM, Jabbour E, Wang T, Liang White J, Sleight B, Vandendries E, Advani AS
Cancer 2019 Jul 15;125(14):2474-2487. Epub 2019 Mar 28 doi: 10.1002/cncr.32116. PMID: 30920645Free PMC Article
Richardson PG, Riches ML, Kernan NA, Brochstein JA, Mineishi S, Termuhlen AM, Arai S, Grupp SA, Guinan EC, Martin PL, Steinbach G, Krishnan A, Nemecek ER, Giralt S, Rodriguez T, Duerst R, Doyle J, Antin JH, Smith A, Lehmann L, Champlin R, Gillio A, Bajwa R, D'Agostino RB Sr, Massaro J, Warren D, Miloslavsky M, Hume RL, Iacobelli M, Nejadnik B, Hannah AL, Soiffer RJ
Blood 2016 Mar 31;127(13):1656-65. Epub 2016 Jan 29 doi: 10.1182/blood-2015-10-676924. PMID: 26825712Free PMC Article
Huertas A, Girerd B, Dorfmuller P, O'Callaghan D, Humbert M, Montani D
Expert Rev Respir Med 2011 Apr;5(2):217-29; quiz 230-1. doi: 10.1586/ers.11.15. PMID: 21510732

Recent systematic reviews

Corbacioglu S, Topaloglu O, Aggarwal S
Clin Drug Investig 2022 Jun;42(6):465-476. Epub 2022 May 20 doi: 10.1007/s40261-022-01140-y. PMID: 35594010Free PMC Article
Thanh DQL, Giau HTN, Huong TNG, Linh TNU, Phuc VM, Vuong NL
Pediatr Cardiol 2022 Jun;43(5):943-951. Epub 2022 Apr 15 doi: 10.1007/s00246-022-02904-1. PMID: 35426500
Luo Q, Jin Q, Zhao Z, Zhao Q, Yu X, Yan L, Zhang Y, Xiong C, Liu Z
BMC Pulm Med 2019 Dec 19;19(1):257. doi: 10.1186/s12890-019-1031-3. PMID: 31856792Free PMC Article
McGee M, Whitehead N, Martin J, Collins N
Clin Toxicol (Phila) 2018 Sep;56(9):801-809. Epub 2018 Mar 6 doi: 10.1080/15563650.2018.1447119. PMID: 29508628
Liu C, Chen J
Cochrane Database Syst Rev 2006 Jul 19;(3):CD004434. doi: 10.1002/14651858.CD004434.pub3. PMID: 16856046

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