Idiopathic and hereditary haemorrhagic telangiectasia associated pulmonary arteriovenous malformations: comparison of clinical and radiographic characteristics

Kroon, Steven; Heuvel, D.A.F. van den; Vos, Jan A.; Leersum, Marc van; Strijen, M.J.L. van; Post, Marco C.; Mager, Johannes J.; Snijder, Repke J.

doi:10.1016/j.crad.2021.01.011

Cited by 10 publications

(11 citation statements)

References 20 publications

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“…Recent observational studies highlight that PAVMs also occur outside of the setting of HHT. Case series from the United States 7 and the Netherlands 31 of consecutive patients with PAVM diagnosed during 13-year 31 to 18-year 7 intervals found that non-HHT PAVMs account for up to 22% of cases. Non-HHT PAVMs are mostly single (83%–90%), unlike in HHT, where they tend to be multiple.…”

Section: Pavm Etiology and Complicationsmentioning

confidence: 99%

Ischemic Stroke and Pulmonary Arteriovenous Malformations

et al. 2022

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The potential of covert pulmonary arteriovenous malformations (PAVMs) to cause early onset, preventable ischemic strokes is not well known to neurologists. This is evident by their lack of mention in serial American Heart Association/ American Stroke Association (AHA/ASA) Guidelines, and the single case-report biased literature of recent years. To inform, we performed PubMed and Cochrane database searches for major studies on ischemic stroke and PAVMs published from January 1, 1974 through April 3, 2021. This identified twenty-four major observational studies, three societal guidelines, one nationwide analysis, three systematic reviews, twenty-one other review/opinion articles, and eighteen recent (2017-2021) case-reports/series that were synthesized. Key points are that patients with PAVMs suffer ischemic stroke a decade earlier than routine stroke, losing nine extra healthy-life-years per patient in the recent US nationwide analysis (2005-2014). Large-scale thoracic CT screens of the general population in Japan estimate PAVM prevalence to be 38/100,000 (95% confidence interval 18-76), with ischemic stroke rates exceeding 10% across PAVM series dating back to the 1950s, with most PAVMs remaining undiagnosed until the time of clinical stroke. Notably, the rate of PAVM diagnoses doubled in US ischemic stroke hospitalizations between 2005-2014. The burden of silent cerebral infarction approximates to twice that of clinical stroke. Over 80% patients have underlying hereditary hemorrhagic telangiectasia (HHT). The predominant stroke mechanism is paradoxical embolization of platelet-rich emboli, with iron-deficiency emerging as a modifiable risk-factor. PAVM related ischemic strokes may be cortical or subcortical, but very rarely cause proximal large vessel occlusions. Single antiplatelet therapy maybe effective for secondary stroke prophylaxis, with dual antiplatelet or anticoagulation therapy requiring nuanced risk-benefit analysis given their risk of aggravating iron deficiency. In conclusion, this review summarizes the current ischemic stroke burden from PAVMs, the implicative pathophysiology, and relevant diagnostic and treatment overviews to facilitate future incorporation into AHA/ASA guidelines.

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Section: Pavm Etiology and Complicationsmentioning

confidence: 99%

Ischemic Stroke and Pulmonary Arteriovenous Malformations

et al. 2022

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“…In the general population, PAVMs are more common in females (1.5-1.8:1 M:F ratio), primarily unilateral (90%), and two-thirds are found in the lower lobes [ 9 ]. PAVMs in HHT are more evenly distributed across sex and lung lobes and are more often multifocal [ 10 ]. PAVMs can be classified as simple (most common), where a single segmental artery drains into one or more veins, complex, where multiple segmental arteries drain into multiple veins, or rarely, diffuse, with hundreds of malformations present.…”

Section: Discussionmentioning

confidence: 99%

“…Often patients with PAVMs are asymptomatic and found incidentally during adulthood on CT performed for another indication [ 3 , 4 ]. Symptomatic patients most often have dyspnea on exertion, palpitations, cough, and chest pain [ 10 , 13 , 14 ]. Patients with HHT may also present with epistaxis or hemoptysis, though this may also occur with PAVM rupture.…”

Section: Discussionmentioning

confidence: 99%

Large pulmonary arteriovenous malformation lost to follow-up with 10 years of asymptomatic interval growth: A case report

Kemper,

Myers,

Thompson

et al. 2024

Radiology Case Reports

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“…If 3 of the criteria are met, the diagnosis of HHT can be established (8,9). Secondary PAVM includes trauma, infection, long-term cirrhosis, mitral stenosis, and other factors (10).…”

Section: Discussionmentioning

confidence: 99%