Migraines linked to intrapulmonary RLS have been described ( 10– 12) and are sometimes the presenting clinical manifestation of PAVMs. Cryptogenic strokes from isolated PAVMs have been described but they are rare ( 8, 9). PAVMs may be present with a variety of clinical manifestations such as dyspnea, hypoxemia, chest pain, and pulmonary hypertension. A third gene, the one that codes for the transcription factor SMAD4, is responsible for the HHT associated with Juvenile Polyposis ( 6, 7). Their incidence has been reported as high as 1 in 2,600 ( 3) and occur in about one-third of all patients with hereditary hemorrhagic telangiectasia (HHT) also known as Rendu–Osler–Weber disease, an autosomal dominant disorder caused by a mutation of endoglin gene (HHT type 1) or activin receptor-like kinase 1 (HHT type 2) and characterized by abnormalities of vascular structures involving multiple organs ( 4, 5). The vast majority of PAVMs are congenital. 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.Ī pulmonary arteriovenous fistula (PAVM) is defined as a congenital abnormal communication between the pulmonary artery and its tributary vein creating a low-resistance, high-flow right-to-left shunting (RLS) bypassing the capillary bed ( 1, 2). Aura migraine symptoms were nearly abolished by P2Y12 platelet inhibition, suggesting a platelet-based mechanism. 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. During the right heart catheterization and pulmonary angiography, it was impossible to cross the interatrial septum with a multipurpose catheter. Contrast Transcranial Doppler (cTCD) confirmed a delayed (>16 s) RLS with two short “shower” patterns corroborating the diagnosis of an extra-cardiac RLS. 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 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. Very few cases of acquired PAVMs have been described in the literature. A great number of patients with PAVMs is suffering concurrently from hereditary hemorrhagic telangiectasia (HHT) whose incidence is around 1 in 5,000. The vast majority of PAVMs are congenital appearing more often in females than in males. 3Neurology Clinic and Rehabilitation Department, Casa di Cura Villa Barbarano, Brescia, ItalyĪ 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).2Cardiology Department, Istituto Fondazione G.Ospedale Galeazzi-Sant'Ambrogio GSD, Milan, Italy Eustaquio Maria Onorato 1 *, Josephal Salvia 2, Mariano Becchina 2, Tommaso Cipolla 2 and Gian Paolo Anzola 3
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