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New Definition and Natural History of Patients with Diffuse Pulmonary Arteriovenous Malformations: 27 Year Experience

¹Department of Respiratory Medicine, School of Medicine, Policlinic of Bari, Bari, Italy ² Department of Pediatrics, Section of Cardiology, Yale University School of Medicine, New Haven, CT, USA ³ Department of Diagnostic Radiology, Section of Interventional Radiology, Yale University School of Medicine, New Haven, CT, USA ⁴ Yale University School of Nursing, New Haven, CT, USA

katharine.henderson{at}yale.edu

Abstract

BackgroundPatients with diffuse pulmonary arteriovenous malformations (PAVM), a small but important subset of the PAVM population, have significant morbidity and mortality.

Methods36 patients (21 female, 15 male) with diffuse PAVM, from a cohort of 821 consecutive patients with PAVM, were evaluated. Diffuse PAVM were categorized angiographically: involvement of one or more segmental pulmonary arteries in one or both lungs. Hereditary hemorrhagic telangiectasia (HHT) status, gender, presence or absence of large (= or > than 3mm diameter artery) focal PAVM, oxygen saturations, complications including hemoptysis, years of follow-up and survival were tabulated.

ResultsHHT was present in 29/36 (81%) of the patients and diffuse PAVM were more commonly bilateral 26/36 (72%) than unilateral 10/36 (28%) (p=0.02). Female gender was associated with bilateral diffuse PAVM 19/26 (73%) (p=0.01). Focal PAVM were present in both groups but more commonly in patients with bilateral involvement 16/26 (62%) (p=0.02). Initial 0₂ saturations (pulse oximetry standing) of patients with unilateral and bilateral diffuse PAVM were 87% ± 7% and 79% ± 8% (p=0.02) respectively. The last or current values for patients with unilateral and bilateral involvement are 95% ± 3% and 85% ± 7% (p < .0001) respectively. Nine deaths occurred and all were in patients with bilateral involvement. Deaths were due to hemoptysis of bronchial artery origin (2), hemorrhage from duodenal ulcer (1), spontaneous liver necrosis (3), brain hemorrhage (1), brain abscess (1), and operative death during attempted lung transplant (1).

ConclusionsPatients with diffuse PAVM are a high risk group and yearly follow up is recommended.

Key Words: arteriovenous malformations • diffuse • pulmonary angiography • hereditary hemorrhagic telangiectasia • transcatheter embolization