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Pulmonary arteriovenous malformation(PAVM)

MedGen UID:
341826
Concept ID:
C1857690
Anatomical Abnormality; Finding
Synonyms: PAVM; Pulmonary arteriovenous fistula; Pulmonary AVM
 
HPO: HP:0006548

Definition

Pulmonary arteriovenous malformation, a condition most commonly associated with hereditary hemorrhagic telangiectasia, is an abnormal communication between the pulmonary artery and pulmonary vein without an intervening capillary communication. HRCT images usually show a coarse spidery appearance of the peripheral vascular markings in the lungs. More specific findings are obtained in the pulmonary angiogram where the normally invisible capillary phase is replaced by irregular vascular channels bridging the peripheral branches of pulmonary arteries and veins. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Pulmonary arteriovenous malformation

Conditions with this feature

Juvenile polyposis/hereditary hemorrhagic telangiectasia syndrome
MedGen UID:
331400
Concept ID:
C1832942
Disease or Syndrome
Hereditary hemorrhagic telangiectasia (HHT) is characterized by the presence of multiple arteriovenous malformations (AVMs) that lack intervening capillaries and result in direct connections between arteries and veins. The most common clinical manifestation is spontaneous and recurrent nosebleeds (epistaxis) beginning on average at age 12 years. Telangiectases (small AVMs) are characteristically found on the lips, tongue, buccal and gastrointestinal (GI) mucosa, face, and fingers. The appearance of telangiectases is generally later than epistaxis but may be during childhood. Large AVMs occur most often in the lungs, liver, or brain; complications from bleeding or shunting may be sudden and catastrophic. A minority of individuals with HHT have GI bleeding, which is rarely seen before age 50 years.
Telangiectasia, hereditary hemorrhagic, type 2
MedGen UID:
324960
Concept ID:
C1838163
Disease or Syndrome
Hereditary hemorrhagic telangiectasia (HHT) is characterized by the presence of multiple arteriovenous malformations (AVMs) that lack intervening capillaries and result in direct connections between arteries and veins. The most common clinical manifestation is spontaneous and recurrent nosebleeds (epistaxis) beginning on average at age 12 years. Telangiectases (small AVMs) are characteristically found on the lips, tongue, buccal and gastrointestinal (GI) mucosa, face, and fingers. The appearance of telangiectases is generally later than epistaxis but may be during childhood. Large AVMs occur most often in the lungs, liver, or brain; complications from bleeding or shunting may be sudden and catastrophic. A minority of individuals with HHT have GI bleeding, which is rarely seen before age 50 years.
Hereditary hemorrhagic telangiectasia type 4
MedGen UID:
341824
Concept ID:
C1857688
Disease or Syndrome
Hereditary hemorrhagic telangiectasia (HHT) is characterized by the presence of multiple arteriovenous malformations (AVMs) that lack intervening capillaries and result in direct connections between arteries and veins. The most common clinical manifestation is spontaneous and recurrent nosebleeds (epistaxis) beginning on average at age 12 years. Telangiectases (small AVMs) are characteristically found on the lips, tongue, buccal and gastrointestinal (GI) mucosa, face, and fingers. The appearance of telangiectases is generally later than epistaxis but may be during childhood. Large AVMs occur most often in the lungs, liver, or brain; complications from bleeding or shunting may be sudden and catastrophic. A minority of individuals with HHT have GI bleeding, which is rarely seen before age 50 years.
Partial lipodystrophy, congenital cataracts, and neurodegeneration syndrome
MedGen UID:
813897
Concept ID:
C3807567
Disease or Syndrome
Lipodystrophies are rare disorders characterized by loss of body fat from various regions and predisposition to metabolic complications of insulin resistance and lipid abnormalities. FPLD7 is an autosomal dominant disorder with a highly variable phenotype. Additional features, including early-onset cataracts and later onset of spasticity of the lower limbs, have been noted in some patients (summary by Garg et al., 2015). For a general phenotypic description and a discussion of genetic heterogeneity of familial partial lipodystrophy (FPLD), see 151660.
Telangiectasia, hereditary hemorrhagic, type 1
MedGen UID:
1643786
Concept ID:
C4551861
Disease or Syndrome
Hereditary hemorrhagic telangiectasia (HHT) is characterized by the presence of multiple arteriovenous malformations (AVMs) that lack intervening capillaries and result in direct connections between arteries and veins. The most common clinical manifestation is spontaneous and recurrent nosebleeds (epistaxis) beginning on average at age 12 years. Telangiectases (small AVMs) are characteristically found on the lips, tongue, buccal and gastrointestinal (GI) mucosa, face, and fingers. The appearance of telangiectases is generally later than epistaxis but may be during childhood. Large AVMs occur most often in the lungs, liver, or brain; complications from bleeding or shunting may be sudden and catastrophic. A minority of individuals with HHT have GI bleeding, which is rarely seen before age 50 years.

Professional guidelines

PubMed

Kulkarni CB, Sutphin P, Iqbal S, Kalva SP
Acad Radiol 2023 Mar;30(3):441-452. Epub 2022 Sep 9 doi: 10.1016/j.acra.2022.08.012. PMID: 36089478
Adachi A, Ohta K, Jahangiri Y, Matsui Y, Horikawa M, Geeratikun Y, Chansanti O, Yata S, Fujii S, Steinberger J, Keller FS, Farsad K
Jpn J Radiol 2020 Apr;38(4):382-386. Epub 2020 Jan 7 doi: 10.1007/s11604-019-00916-1. PMID: 31912422
Hanley M, Ahmed O, Chandra A, Gage KL, Gerhard-Herman MD, Ginsburg M, Gornik HL, Johnson PT, Oliva IB, Ptak T, Steigner ML, Strax R, Rybicki FJ, Dill KE
J Am Coll Radiol 2016 Jul;13(7):796-800. Epub 2016 May 19 doi: 10.1016/j.jacr.2016.03.020. PMID: 27209598

Recent clinical studies

Etiology

Lau KK, Steinke K, Reis S, Cherukuri SP, Cejna M
Respirology 2022 Aug;27(8):581-599. Epub 2022 Jun 27 doi: 10.1111/resp.14315. PMID: 35758539Free PMC Article
Tellapuri S, Park HS, Kalva SP
Int J Cardiovasc Imaging 2019 Aug;35(8):1421-1428. Epub 2018 Nov 1 doi: 10.1007/s10554-018-1479-x. PMID: 30386957
Hsu CC, Kwan GN, Evans-Barns H, van Driel ML
Cochrane Database Syst Rev 2018 Jan 4;1(1):CD008017. doi: 10.1002/14651858.CD008017.pub5. PMID: 29298459Free PMC Article
Cummings KW, Bhalla S
Clin Chest Med 2015 Jun;36(2):235-48, viii. Epub 2015 Mar 26 doi: 10.1016/j.ccm.2015.02.007. PMID: 26024602
Hsu CC, Kwan GN, Thompson SA, Evans-Barns H, van Driel ML
Cochrane Database Syst Rev 2015 Jan 29;1:CD008017. doi: 10.1002/14651858.CD008017.pub4. PMID: 25634560

Diagnosis

Scarpato BM, McDonald J, Bayrak-Toydemir P, Elliott CG, Cahill BC, Emerson LL, Keenan LM
Chest 2023 May;163(5):e201-e205. doi: 10.1016/j.chest.2023.01.020. PMID: 37164583Free PMC Article
Lee HN, Hyun D
Korean J Radiol 2022 Feb;23(2):202-217. Epub 2022 Jan 4 doi: 10.3348/kjr.2021.0417. PMID: 35029077Free PMC Article
Tellapuri S, Park HS, Kalva SP
Int J Cardiovasc Imaging 2019 Aug;35(8):1421-1428. Epub 2018 Nov 1 doi: 10.1007/s10554-018-1479-x. PMID: 30386957
Agrawal A, Palkar A, Talwar A
Respir Med 2017 Aug;129:31-38. Epub 2017 May 31 doi: 10.1016/j.rmed.2017.05.016. PMID: 28732833
Khurshid I, Downie GH
Postgrad Med J 2002 Apr;78(918):191-7. doi: 10.1136/pmj.78.918.191. PMID: 11930021Free PMC Article

Therapy

Tellapuri S, Park HS, Kalva SP
Int J Cardiovasc Imaging 2019 Aug;35(8):1421-1428. Epub 2018 Nov 1 doi: 10.1007/s10554-018-1479-x. PMID: 30386957
Hsu CC, Kwan GN, Evans-Barns H, van Driel ML
Cochrane Database Syst Rev 2018 Jan 4;1(1):CD008017. doi: 10.1002/14651858.CD008017.pub5. PMID: 29298459Free PMC Article
Hsu CC, Kwan GN, Thompson SA, Evans-Barns H, van Driel ML
Cochrane Database Syst Rev 2015 Jan 29;1:CD008017. doi: 10.1002/14651858.CD008017.pub4. PMID: 25634560
Hsu CC, Kwan GN, Thompson SA, Evans-Barns H, van Driel ML
Cochrane Database Syst Rev 2012 Aug 15;(8):CD008017. doi: 10.1002/14651858.CD008017.pub3. PMID: 22895972
Hsu CC, Kwan GN, Thompson SA, van Driel ML
Cochrane Database Syst Rev 2010 May 12;(5):CD008017. doi: 10.1002/14651858.CD008017.pub2. PMID: 20464760

Prognosis

Song JK
J Am Soc Echocardiogr 2023 Sep;36(9):895-905.e3. Epub 2023 May 15 doi: 10.1016/j.echo.2023.05.004. PMID: 37196905
Maruyama T, Kariya S, Nakatani M, Ono Y, Ueno Y, Komemushi A, Tanigawa N
Medicine (Baltimore) 2021 Dec 23;100(51):e28340. doi: 10.1097/MD.0000000000028340. PMID: 34941138Free PMC Article
DePietro DM, Curnes NR, Chittams J, Ferrari VA, Pyeritz RE, Trerotola SO
Chest 2020 May;157(5):1278-1286. Epub 2019 Nov 30 doi: 10.1016/j.chest.2019.11.023. PMID: 31794700
Chick JFB, Reddy SN, Pyeritz RE, Trerotola SO
Cardiovasc Intervent Radiol 2017 Jul;40(7):1003-1009. Epub 2017 Feb 10 doi: 10.1007/s00270-017-1604-6. PMID: 28188364
Bélanger C, Chartrand-Lefebvre C, Soulez G, Faughnan ME, Tahir MR, Giroux MF, Gilbert P, Perreault P, Bouchard L, Oliva VL, Therasse E
Eur J Radiol 2016 Jan;85(1):150-157. Epub 2015 Nov 10 doi: 10.1016/j.ejrad.2015.11.014. PMID: 26724660

Clinical prediction guides

Mojadidi MK, Ruiz JC, Chertoff J, Zaman MO, Elgendy IY, Mahmoud AN, Al-Ani M, Elgendy AY, Patel NK, Shantha G, Tobis JM, Meier B
Cardiol Rev 2019 Jan/Feb;27(1):34-40. doi: 10.1097/CRD.0000000000000205. PMID: 29570476
Hsu CC, Kwan GN, Evans-Barns H, van Driel ML
Cochrane Database Syst Rev 2018 Jan 4;1(1):CD008017. doi: 10.1002/14651858.CD008017.pub5. PMID: 29298459Free PMC Article
Hsu CC, Kwan GN, Thompson SA, Evans-Barns H, van Driel ML
Cochrane Database Syst Rev 2015 Jan 29;1:CD008017. doi: 10.1002/14651858.CD008017.pub4. PMID: 25634560
Velthuis S, Buscarini E, Gossage JR, Snijder RJ, Mager JJ, Post MC
J Am Soc Echocardiogr 2015 Mar;28(3):255-63. Epub 2015 Jan 23 doi: 10.1016/j.echo.2014.12.008. PMID: 25623000
Hsu CC, Kwan GN, Thompson SA, Evans-Barns H, van Driel ML
Cochrane Database Syst Rev 2012 Aug 15;(8):CD008017. doi: 10.1002/14651858.CD008017.pub3. PMID: 22895972

Recent systematic reviews

Agarwal J, LaBranche J, Dhillon S, Allison WT, Jeerakathil T, Vethanayagam D
Can J Neurol Sci 2023 Jul;50(4):561-572. Epub 2022 Jun 10 doi: 10.1017/cjn.2022.80. PMID: 35686303
Hsu CC, Kwan GN, Evans-Barns H, van Driel ML
Cochrane Database Syst Rev 2018 Jan 4;1(1):CD008017. doi: 10.1002/14651858.CD008017.pub5. PMID: 29298459Free PMC Article
Hsu CC, Kwan GN, Thompson SA, Evans-Barns H, van Driel ML
Cochrane Database Syst Rev 2015 Jan 29;1:CD008017. doi: 10.1002/14651858.CD008017.pub4. PMID: 25634560
Hsu CC, Kwan GN, Thompson SA, Evans-Barns H, van Driel ML
Cochrane Database Syst Rev 2012 Aug 15;(8):CD008017. doi: 10.1002/14651858.CD008017.pub3. PMID: 22895972
Hsu CC, Kwan GN, Thompson SA, van Driel ML
Cochrane Database Syst Rev 2010 May 12;(5):CD008017. doi: 10.1002/14651858.CD008017.pub2. PMID: 20464760

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