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Pulmonary arteriovenous malformation
Other Resources UpToDate PubMed

Pulmonary arteriovenous malformation

Contributors: Casey Silver MD, Abhijeet Waghray MD, Ryan Hoefen MD, PhD, Paritosh Prasad MD
Other Resources UpToDate PubMed

Synopsis

Pulmonary arteriovenous malformations (PAVM) are abnormal direct communications between the pulmonary arteries and veins without the usual capillary bed. While pulmonary symptoms may include dyspnea, hypoxemia, and hemoptysis, these malformations are typically asymptomatic and are most commonly found incidentally on chest imaging. If symptoms occur, they often manifest in the fourth through sixth decades of life, although patients with hereditary hemorrhagic telangiectasia (HHT) may present earlier. Most patients have multiple PAVMs, although number, size, and distribution vary.

Most PAVMs are congenital and occur in patients with HHT, also known as Osler-Weber-Rendu syndrome. They can also occur in acquired conditions such as cirrhosis (most common), penetrating chest trauma, mitral stenosis, schistosomiasis, actinomycosis, Fanconi syndrome, and thyroid carcinoma. Some are idiopathic. The cause of PAVM is unknown.

Codes

ICD10CM:
Q25.72 – Congenital pulmonary arteriovenous malformation

SNOMEDCT:
303070000 – Pulmonary arteriovenous malformation

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Hereditary hemorrhagic telangiectasia (HHT)
  • Atrial septal defect
  • Liver cirrhosis
  • Schistosomiasis
  • Actinomycosis
  • Congenital Fanconi syndrome

Best Tests

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Management Pearls

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Therapy

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References

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Last Reviewed:06/05/2019
Last Updated:01/23/2022
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Pulmonary arteriovenous malformation
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A medical illustration showing key findings of Pulmonary arteriovenous malformation : Cyanosis, Nail clubbing, Dyspnea, Epistaxis, Hemoptysis
Copyright © 2024 VisualDx®. All rights reserved.