Effect of pulmonary arteriovenous malformations on the mechanical properties of the lungs

Abstract

BackgroundPulmonary arteriovenous malformations (PAVMs) are present in approximately 15–50% individuals with hereditary hemorrhagic telangiectasia (HHT). They may be isolated but more often are multiple. The goal of this study was to evaluate the influence of PAVMs on lung mechanical properties.MethodsWe reviewed the files of all adult patients (age ≥ 18 years) referred to our Center for evaluation of HHT between 2005 and 2013. The diagnosis of HHT was based on the Curacao criteria and/or the presence of a pathogenic mutation. Exclusion criteria included: chronic cardiac or lung disease (i.e. asthma or COPD), suspicion of pulmonary hypertension on echocardiography, current or past smoking (>10 pack-years), history of thoracic surgery, previous treatment of PAVMs by embolotherapy, lung infection or thromboembolic disease in the past 3 months, pregnancy and obesity (BMI > 30 kg/m2). Chest high resolution CT-scan and pulmonary function tests were performed the same day in all patients as part of our routine work-up.ResultsOne hundred and fifty five patients with HHT were included (age: 44.4 ± 16.7 yrs – mean ± SD -; males: 39%). Eighty eight patients had no PAVM, 45 had 1–3 PAVMS and 22 had at least 4 PAVMs. Thirty eight patients had unilateral PAVMs and 29 bilateral PAVMs. We found no statistical relationship between the number, the size and the laterality of PAVMs and results of lung flows and volumes.ConclusionWe found no evidence that PAVMs have a significant influence on lung mechanical properties as measured using routine pulmonary function tests in adult patients with HHT, even in case of numerous, macroscopic or bilateral malformations.

Severe cerebral abscess associated with pulmonary arteriovenous fistula: case report and literature review

“…The most prominent complication of PAVF is neurologic diseases which have been observed at nearly 35% (range, 5–56%) [ 11 ], and the incidence of brain abscess is approximately 5%. So, the neurosurgeon can meet the PAVF patients whose initial symptom is brain abscesses [ 11 ], hemiplegia [ 12 ], seizures, or migraines [ 13 ], but the symptom of PAVF, such as dyspnea, hemoptysis, cyanosis, or asymptomatic, may be covered [ 14 ]. In addition, PAVF can lead to various neurological complications such as stroke (20%), paradoxical embolism [ 15 ], and other systematic diseases such as dyspnea (50%), migraines (30%) [ 13 ], hemoptysis (15%), epistaxis (73%) [ 16 ], cerebral arteriovenous fistula (5%), porencephaly (5%), encephalomalacia (5%), and atrophy (9%) [ 17 ].…”

A boy with refractory cyanosis: pediatric pulmonary arteriovenous malformation case report and literature review

“…In the existing literature, PAVM is also known as pulmonary arteriovenous stula, hemorrhagic telangiectasia with pulmonary artery aneurysm, pulmonary vasodilation, etc. [7,8]. Over 80% of PAVMs are caused by congenital dysplasia.…”

Pulmonary gas exchange in hereditary hemorrhagic telangiectasia patients with liver arteriovenous malformations

“…Pulmonary AVMs (PAVMs) induce right-to-left shunting, which is routinely assessed by contrast echocardiography and arterial blood gas measurements. Pulmonary functions test (PFTs), completed concomitantly with arterial blood gas measurements, remain normal in HHT patients without PAVMs and respiratory comorbidity [2]. Nevertheless, few studies have evaluated pulmonary gas exchange in HHT patients.…”