Pulmonary Arteriovenous Fistula: Presentation, Diagnosis, and Treatment

Pick, Adrian; Deschamps, Claude; Stanson, Anthony W.

doi:10.1007/s002689900634

Cited by 87 publications

(58 citation statements)

References 49 publications

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“…The timing of appearance of the bubbles in the left atrium helps locating the shunt, but may be influenced by the intensity of the shunt [112]. Contrast enhancement may also be seen in a particular pulmonary vein in a patient with PAVM [113]. …”

Section: Assessment Of Right-to-left Shuntingmentioning

confidence: 99%

Pulmonary Vascular Manifestations of Hereditary Hemorrhagic Telangiectasia (Rendu-Osler Disease)

et al. 2007

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Hereditary hemorrhagic telangiectasia (HHT) or Rendu-Osler-Weber disease is a genetic disorder with autosomal dominance and variable penetrance, characterized by epistaxis, telangiectasia and visceral manifestations of the disease. The estimated minimal prevalence is 1/10,000 inhabitants. The diagnosis is established on clinical criteria, and may be further confirmed by the identification of causative mutations in either the ENG or the ACVRL1 gene coding for endoglin and ALK1, respectively. Pulmonary vascular manifestations of HHT include pulmonary arteriovenous malforma- tions (PAVMs; especially in patients with ENG mutations) and less frequently pulmonary hypertension (especially in patients with ACVRL1 mutations). In 15–33% of patients with HHT, PAVMs consist of abnormal communications between pulmonary arteries and pulmonary veins, causing right-to-left shunting, and thus, frequently hypoxemia and dyspnea on exertion, although PAVMs may remain asymptomatic and frequently undiagnosed unless complications occur. PAVMs result in severe and frequent complications often at a young age, which may reveal the diagnosis, e.g. transient ischemic attack and cerebral stroke (10–19% of patients), systemic severe infections and abscesses (including cerebral abscess in 5–19% of patients), and rarely massive hemoptysis or hemothorax. Infections in HHT are related to the right-to-left shunting that bypasses the pulmonary capillaries and facilitates the passage of septic or aseptic emboli into the systemic and especially cerebral circulation, and potentially to minor defects in innate immunity. Treatment of PAVMs based on transcatheter coil vaso-occlusion of the feeding artery significantly decreases right-to-left shunting, hypoxemia and dyspnea on exertion, and reduces the risk of systemic complications. Long-term follow-up is warranted after transcatheter vaso-occlusion of PAVMs due to frequent recanalization of treated PAVMs and development or growth of untreated PAVMs. Patients with HHT should be informed of the risk of PAVM and potentially severe complications occurring in heretofore asymptomatic subjects. All adult patients with HHT should be proposed systematic screening for PAVM, by contrast echocardiography (preceded by anteroposterior chest radiograph) or computed tomography of the chest. Pulmonary hypertension is rare in HHT, and may be due either to systemic arteriovenous shunting in the liver increasing cardiac output or be clinically and histologically indistinguishable from idiopathic pulmonary arterial hypertension. Pulmonary hypertension is detected by systematic examination of right cardiac cavities and tricuspid regurgitation flow at echocardiography, and the diagnosis is established by right heart catheterization.

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Section: Assessment Of Right-to-left Shuntingmentioning

confidence: 99%

Pulmonary Vascular Manifestations of Hereditary Hemorrhagic Telangiectasia (Rendu-Osler Disease)

et al. 2007

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“…In plain chest X-rays, PAVM may manifest in the form of the spread of hemorrhaging to parenchyma, or atelectasis due to bronchus compression. Microvascular telangiectasis can create a normal chest X-ray, or pulmonary vascular signs may increase vaguely (2,7,12). In dynamic examination, PAVMs typically enhanced contrast after the right ventricle or pulmonary artery, before the left atrium and ventricle.…”

Section: Discussionmentioning

confidence: 99%

Surgical treatment of pulmonary arteriovenous malformations

Biçakçioğlu¹,

Gülhan²,

Sayilir³

et al. 2017

Turk J Med Sci

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Background/aim: Pulmonary arteriovenous malformations (PAVMs) are direct communications between the branches of pulmonary arteries and veins. This study evaluates surgically treated cases of pulmonary arteriovenous malformations. Materials and methods:We retrospectively examined 41 cases of PAVM that were operated in our clinic between 1995 and 2012. We obtained the clinical, radiological, and surgical data of the patients from their files. Results:The 41 cases comprised 27 males and 14 females. Their mean age at diagnosis was 39.8 years (range: 9-71). The symptoms were hemoptysis in 28 cases, dyspnea in five, cough in three, and epistaxis in two; three patients were asymptomatic. Twenty-three right and 19 left posterolateral thoracotomies were performed, including one case which was operated bilaterally. Lower lobectomy was performed in 17 patients, lower lobectomy and lingulectomy in two, upper lobectomy in ten, middle lobectomy in two, segmentectomy in seven, and wedge resection in four. Postoperative histopathology was arteriovenous malformation in all cases. Conclusion:PAVMs are rare clinical conditions. Surgery remains the first choice when embolization treatment cannot be performed or is not successful, in symptomatic and complicated patients with PAVM, and/or in cases where the PAVM diagnosis cannot be established.

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“…Acquired pulmonary AVMs can occur in the context of a variety of physical injuries or disease processes such as liver cirrhosis, chest trauma, infection, metastatic carcinoma, mitral stenosis or systemic amyloidosis (7,8). Pulmonary metastases of GTD have the potential to form pulmonary AVMs, which can cause life-threatening hemorrhage or hemoptysis (2).…”

Section: Discussionmentioning

confidence: 99%