Idiopathic pulmonary arteriovenous malformations: clinical and imaging characteristics

Abstract

Pulmonary arteriovenous malformations (PAVMs) can cause stroke, brain abscess or life-threatening haemorrhage. Most PAVMs are associated with hereditary haemorrhagic telangiectasia (HHT). The aim of the present study was to describe the clinical presentation and treatment outcomes of those with idiopathic PAVMs, which has not previously been described in the literature.Patients with idiopathic PAVMs were identified at our HHT centre. Retrospective review of charts and imaging were performed. 20 patients were identified with idiopathic PAVMs. The most common symptoms reported were dyspnoea and migraines (50 and 30% of patients, respectively). Previous complications of PAVMs included haemoptysis (20%), stroke (20%) and brain abscess (5%). A total of 28 focal PAVMs were identified. Most patients (80%) had a solitary PAVM. 13 out of 28 PAVMs (46%) were located in the lower lobes. Most were simple and fistulous rather than complex and plexiform. Transcatheter embolotherapy was performed in 17 patients and was successful in improving oxygenation in all cases.The clinical manifestations and complications of idiopathic PAVMs are similar to those associated with HHT. Idiopathic PAVMs are anatomically similar to HHT-related PAVMs except for a greater number of solitary PAVMs and a lack of lower lobe predominance. Transcatheter embolotherapy is a safe and effective method for treating idiopathic PAVMs.

Brain abscess as the first manifestation of pulmonary arteriovenous malformation: A case report

“…Idiopathic PAVM are similar in shape and imaging findings, except for greater number of solitary versus multiple AVM and also lack of lower lobe predominance, in idiopathic cases. [10]…”

Brain Abscesses Associated with Asymptomatic Pulmonary Arteriovenous Fistulas

“…Five suffered recurrent brain abscesses. Idiopathic pulmonary AVFs are more frequently solitary, around 80%, compared with that of HHT (<40%)30). It means idiopathic pulmonary AVFs are less likely to be associated with large right-to-left shunt, this might in part explain why patients with idiopathic PAVMs might have a lower frequency of cyanosis and polycythemia.…”

“…Associated neurological events included migraine, transient ischemic attack, stroke, abscess, and seizure17,26,30). The incidence of brain abscess in patients with a pulmonary AVF is around 1 to 5%8).…”

“…The characteristic findings of cyanosis, clubbing and an extra-cardiac murmur do not always accompany pulmonary AVF, and diagnosis may be difficult. Asymptomatic patients are common and account for 13% to 56% of patients8,9,30). An absence of symptoms does not preclude diagnosis of pulmonary AVF.…”

Hämoptysen in der Schwangerschaft

“…PAVM werden am häufigsten mit Luftnot (13 -56 %) und Hämoptysen (7 -30 %), seltener mit Auftreten eines Hämatothorax klinisch symptomatisch [8,9]. Neurologische Symptome und Komplikationen wie Kopfschmerzen, Schwindel, Krampfanfälle bis hin zu Schlaganfällen oder Hirnabszessen werden in 9 -41 % der Fälle beschrieben [4,10]. Die von unserer Patientin im Rahmen der notfallmäßigen Wiederaufnahme beklagte Schwindelsymptomatik sowie der fremdanamnestisch berichtete kurze Krampfanfall muss in diesem Kontext als passagere thrombembolische Komplikation gewertet werden.…”