{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T16:06:14Z","timestamp":1753891574415,"version":"3.41.2"},"reference-count":31,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2022,12,15]],"date-time":"2022-12-15T00:00:00Z","timestamp":1671062400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Neurol."],"abstract":"<jats:p>A pulmonary arteriovenous fistula (PAVM) is an abnormal blood vessel that creates a direct connection between a pulmonary artery and its tributary vein bypassing capillary filter, establishing as a consequence of a low-resistance right-to-left shunting (RLS). The vast majority of PAVMs are congenital appearing more often in females than in males. A great number of patients with PAVMs is suffering concurrently from hereditary hemorrhagic telangiectasia (HHT) whose incidence is around 1 in 5,000. Very few cases of acquired PAVMs have been described in the literature. Paradoxical embolism through PAVMs can cause systemic desaturation, cyanosis, and serious cerebrovascular ischemic events (transient ischemic attacks, strokes, and intracranial abscess), even when the abnormal blood vessel is small (diameter &amp;lt;3 mm). Notably, it has been reported a high prevalence of aura migraine (MHA) symptoms in patients with PAVMs and concomitant HHT. We described in this study the case of a young aura migraineur female patient without HHT in whom isolated PAVM below the detection limit of pulmonary angiography and chest computed tomography angiography (CTA) has been documented by contrast Transthoracic and Transesophageal Echocardiography (cTTE\/TEE) showing a delayed (&amp;gt;17 s) RLS coming from left pulmonary veins to left atrium while a patent foramen ovale (PFO), small atrial septal defects or septum primum fenestration could not be detected despite several attempts. Contrast Transcranial Doppler (cTCD) confirmed a delayed (&amp;gt;16 s) RLS with two short \u201cshower\u201d patterns corroborating the diagnosis of an extra-cardiac RLS. During the right heart catheterization and pulmonary angiography, it was impossible to cross the interatrial septum with a multipurpose catheter. The patient was finally discharged with off-label thienopyridine agents (clopidogrel 75 mg die) in terms of primary prophylaxis for paradoxical right-to-left embolization of thrombotic material ultimately. Aura migraine symptoms were nearly abolished by P2Y12 platelet inhibition, suggesting a platelet-based mechanism. During 2 years of clinical follow-up on thienopyridine therapy, the resolution of aura migraine episodes was definitively accomplished with significant improvement in her quality of life.<\/jats:p>","DOI":"10.3389\/fneur.2022.1079959","type":"journal-article","created":{"date-parts":[[2022,12,15]],"date-time":"2022-12-15T06:47:03Z","timestamp":1671086823000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":1,"title":["Can aura migraine be elicited by isolated pulmonary arteriovenous fistula?\u2014A case report"],"prefix":"10.3389","volume":"13","author":[{"given":"Eustaquio Maria","family":"Onorato","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Josephal","family":"Salvia","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mariano","family":"Becchina","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tommaso","family":"Cipolla","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gian Paolo","family":"Anzola","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2022,12,15]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"1154","DOI":"10.1136\/thoraxjnl-2017-210764","article-title":"British Thoracic Society clinical statement on pulmonary arteriovenous malformations","volume":"72","author":"Shovlin","year":"2017","journal-title":"Thorax."},{"key":"B2","doi-asserted-by":"publisher","first-page":"1927","DOI":"10.3390\/jcm9061927","article-title":"Approach to pulmonary arteriovenous malformations: a comprehensive update","volume":"9","author":"Post","year":"2020","journal-title":"J Clin Med."},{"key":"B3","doi-asserted-by":"publisher","first-page":"1677","DOI":"10.2169\/internalmedicine.51.7305","article-title":"Prevalence of pulmonary arteriovenous malformations as estimated by low-dose thoracic CT screening","volume":"51","author":"Nakayama","year":"2012","journal-title":"Intern Med."},{"key":"B4","doi-asserted-by":"publisher","first-page":"191","DOI":"10.1136\/pmj.78.918.191","article-title":"Pulmonary arteriovenous malformation","volume":"78","author":"Khurshid","year":"2002","journal-title":"Postgrad Med J."},{"key":"B5","doi-asserted-by":"publisher","first-page":"353","DOI":"10.1007\/s00270-019-02396-2","article-title":"CIRSE standards of practice on diagnosis and treatment of pulmonary arteriovenous malformations","volume":"43","author":"M\u00fcller-H\u00fclsbeck","year":"2020","journal-title":"Cardiovasc Intervent Radiol"},{"key":"B6","doi-asserted-by":"publisher","first-page":"852","DOI":"10.1016\/S0140-6736(04)15732-2","article-title":"A combined syndrome of juvenile polyposis and hereditary haemorrhagic telangiectasia associated with mutations in MADH4 (SMAD4)","volume":"363","author":"Gallione","year":"2004","journal-title":"Lancet"},{"key":"B7","doi-asserted-by":"publisher","first-page":"570","DOI":"10.5114\/aoms.2014.43750","article-title":"Juvenile polyposis syndrome","volume":"10","author":"Cichy","year":"2014","journal-title":"Arch Med Sci."},{"key":"B8","first-page":"73","article-title":"An isolated fistula between the right pulmonary artery and the right pulmonary vein: an unusual cause of stroke in a young female","volume":"18","author":"Husain","year":"2011","journal-title":"Cardiol J."},{"key":"B9","doi-asserted-by":"publisher","first-page":"145","DOI":"10.4103\/0974-2069.132498","article-title":"Amplatzer vascular plug IV for occlusion of pulmonary arteriovenous malformations in a patient with cryptogenic stroke","volume":"7","author":"Veeram Reddy","year":"2014","journal-title":"Ann Pediatr Cardiol."},{"key":"B10","doi-asserted-by":"publisher","first-page":"439","DOI":"10.1111\/j.1526-4610.2006.00291.x","article-title":"Migraines linked to intrapulmonary right-to-left shunt","volume":"46","author":"Thenganatt","year":"2006","journal-title":"Headache."},{"key":"B11","doi-asserted-by":"publisher","first-page":"2485","DOI":"10.1378\/chest.128.4.2485","article-title":"A pulmonary right-to-left shunt in patients with hereditary hemorrhagic telangiectasia is associated with an increased prevalence of migraine","volume":"128","author":"Post","year":"2005","journal-title":"Chest."},{"key":"B12","doi-asserted-by":"publisher","first-page":"76","DOI":"10.1111\/j.1468-2982.2008.01703.x","article-title":"An association of migraine with hereditary haemorrhagic telangiectasia independently of pulmonary right-to-left shunts","volume":"29","author":"Marziniak","year":"2009","journal-title":"Cephalalgia."},{"key":"B13","doi-asserted-by":"publisher","first-page":"360","DOI":"10.1111\/j.1468-2982.2008.01539.x","article-title":"Is migraine associated with right-to-left shunt a separate disease? 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