PURPOSE: Pulmonary arteriovenous malformations (PAVM) can cause stroke, brain abscess or life-threatening hemorrhage. Approximately 80 percent of cases are associated with hereditary hemorrhagic telangiectasia (HHT). Our aim was to describe the clinical presentation, imaging characteristics and treatment outcomes of patients with idiopathic PAVM.
METHODS: Patients with idiopathic PAVM were identified from the St. Michael's Hospital HHT Clinic Database. Retrospective review of charts and imaging was performed.
RESULTS: Nineteen patients were identified between May 1999 and February 2007 as having idiopathic PAVM. Mean age was 49 years (range: 25- 86); 14/19 (74%) were female. Seven/19 patients (37%) presented with symptoms or complications from PAVM. In 12 patients, PAVM were found incidentally. Most common symptoms reported at initial assessment were dyspnea on exertion in 10/19 patients (55%) and migraines in 8/19 patients (42%). Previous complications of PAVM included hemoptysis in 5/19 patients (26%), stroke in 4/19 (21%) and brain abscess in 1/19 (5%). CT chest review in 18/19 patients revealed 28 focal PAVM. Fourteen patients (74%) had only 1 PAVM, 3 patients (18%) had 2 PAVM, 1 patient (5%) had 8 PAVM, and 1 patient (5%) had diffuse PAVM. Two/28 PAVM (7%) were complex while the remainder were simple. Transcatheter embolotherapy was performed for 26/28 PAVM in 21 sessions and was successful in improving oxygenation in all cases. No major procedural complications occurred, such as paradoxical embolism or hemoptysis. Minor post-embolization infarct was noted in 2/21(10%) sessions. Reperfusion occurred in 3/26 (12%) treated PAVM.
CONCLUSION: Patients with idiopathic PAVM may be more likely to have single PAVM than patients with HHT. Otherwise, patients with idiopathic PAVM present with similar symptoms, complications and imaging characteristics as patients with HHT-related PAVM. Finally, in this small study, transcatheter embolotherapy appeared to be safe and effective for treatment of idiopathic PAVM.
CLINICAL IMPLICATIONS: Similarities between idiopathic and HHT-related PAVM suggest that they may warrant similar follow-up and management.
DISCLOSURE: Harvey Wong, No Product/Research Disclosure Information; Other M.E.F: St. Michael's Hospital Research Institute, Nelson Arthur Hyland Foundation.