Abstract
Pulmonary arteriovenous malformations (PAVMs) can cause stroke, brain abscess or life-threatening hemorrhage. Most PAVMs are associated with hereditary hemorrhagic telangiectasia (HHT). We aimed to describe the clinical presentation and treatment outcomes of those with idiopathic PAVMs, previously never described in the literature.
Patients with idiopathic PAVMs were identified at our HHT centre. Retrospective review of charts and imaging was performed.
Twenty patients were identified with idiopathic PAVMs. Most common symptoms reported were dyspnoea and migraines (50% and 30% of patients, respectively). Previous complications of PAVMs included hemoptysis (20%), stroke (20%), and brain abscess (5%). A total of 28 focal PAVMs were identified. Most patients (80%) had a solitary PAVM. Thirteen 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.
- ERS