This article requires a subscription to view the full text. If you have a subscription you may use the login form below to view the article. Access to this article can also be purchased.
Abstract
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. 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 24 major observational studies, 3 societal guidelines, 1 nationwide analysis, 3 systematic reviews, 21 other review/opinion articles, and 18 recent (2017–2021) case reports/series that were synthesized. Key points are that patients with PAVMs have ischemic stroke a decade earlier than routine stroke, losing 9 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 and 2014. The burden of silent cerebral infarction approximates to twice that of clinical stroke. More than 80% of patients have underlying hereditary hemorrhagic telangiectasia. 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 may be effective for secondary stroke prophylaxis, with dual antiplatelet or anticoagulation therapy requiring nuanced risk–benefit analysis given their risk of aggravating iron deficiency. This review summarizes the ischemic stroke burden from PAVMs, the implicative pathophysiology, and relevant diagnostic and treatment overviews to facilitate future incorporation into AHA/ASA guidelines.
Glossary
- AHA/ASA=
- American Heart Association/American Stroke Association;
- AIS=
- acute ischemic stroke;
- AVM=
- arteriovenous malformation;
- BTS=
- British Thoracic Society;
- CI=
- confidence interval;
- CIRSE=
- Cardiovascular and Interventional Radiologic Society of Europe;
- ESUS=
- embolic stroke of undetermined source;
- FAD=
- feeding artery diameter;
- HHT=
- hereditary hemorrhagic telangiectasia;
- MCA=
- middle cerebral artery;
- NIS=
- Nationwide Inpatient Sample;
- OR=
- odds ratio;
- PAVM=
- pulmonary arteriovenous malformation;
- PFO=
- patent foramen ovale;
- RCT=
- randomized clinical trial
Footnotes
Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
- Received June 27, 2021.
- Accepted in final form November 12, 2021.
- © 2021 American Academy of Neurology
AAN Members
We have changed the login procedure to improve access between AAN.com and the Neurology journals. If you are experiencing issues, please log out of AAN.com and clear history and cookies. (For instructions by browser, please click the instruction pages below). After clearing, choose preferred Journal and select login for AAN Members. You will be redirected to a login page where you can log in with your AAN ID number and password. When you are returned to the Journal, your name should appear at the top right of the page.
AAN Non-Member Subscribers
Purchase access
Letters: Rapid online correspondence
REQUIREMENTS
You must ensure that your Disclosures have been updated within the previous six months. Please go to our Submission Site to add or update your Disclosure information.
Your co-authors must send a completed Publishing Agreement Form to Neurology Staff (not necessary for the lead/corresponding author as the form below will suffice) before you upload your comment.
If you are responding to a comment that was written about an article you originally authored:
You (and co-authors) do not need to fill out forms or check disclosures as author forms are still valid
and apply to letter.
Submission specifications:
- Submissions must be < 200 words with < 5 references. Reference 1 must be the article on which you are commenting.
- Submissions should not have more than 5 authors. (Exception: original author replies can include all original authors of the article)
- Submit only on articles published within 6 months of issue date.
- Do not be redundant. Read any comments already posted on the article prior to submission.
- Submitted comments are subject to editing and editor review prior to posting.
