Pulmonary Arteriovenous Malformations

Kjeldsen, Anette Drøhse; Oxhøj, H; Andersen, Poul Erik; Elle, Bo; Jacobsen, Jens Peter; Vase, P.

doi:10.1378/chest.116.2.432

Cited by 135 publications

(28 citation statements)

References 31 publications

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“…It also remains positive after endovascular treatment of PAVM in up to 90% of patients, even when no residual PAVM is seen on pulmonary angiography [114]. Such positive contrast echocardiography in the absence of permeable PAVM may correspond to false-positive test results, or more likely to microscopic and diffuse PAVMs causing genuine right-to-left shunting [72, 115] with yet unknown clinical significance. Quantification of the shunt by echocardiography suggested by several groups [109,114,115,116] may allow better understanding of right-to-left shunting [114].…”

Section: Assessment Of Right-to-left Shuntingmentioning

confidence: 99%

“…Such positive contrast echocardiography in the absence of permeable PAVM may correspond to false-positive test results, or more likely to microscopic and diffuse PAVMs causing genuine right-to-left shunting [72, 115] with yet unknown clinical significance. Quantification of the shunt by echocardiography suggested by several groups [109,114,115,116] may allow better understanding of right-to-left shunting [114]. A recently developed scoring system of shunts by transthoracic contrast echocardiography improves the prediction of PAVMs by this method, with a positive predictive value of only 0.02 for a score of 1, to a value of 1.0 for a maximal score of 4 [112], although PAVMs and potentially severe complications may occur even in patients with a score of 1.…”

Section: Assessment Of Right-to-left Shuntingmentioning

confidence: 99%

“…Self-reported dyspnea, room air PaO 2 , AaPO 2 (while breathing room air or using the 100% oxygen method), pulse oximetry and radionuclide lung scanning are also insufficient for screening [95]. Contrast echocardiography is the most sensitive test to detect right-to-left shunting, with a sensitivity and a negative predictive value of 93% [95], and is most appropriate for screening for PAVM [71, 115, 139]. In a retrospective study of 105 patients with HHT, an approach combining anteroposterior chest radiography and contrast echocardiography achieved 100% sensitivity and negative predictive value [95], and is now considered an excellent screening procedure, with the advantage of very large availability, noninvasiveness, lower costs and much lower radiation exposure than chest CT. A proposed screening algorithm (fig.…”

Section: Screening For Pavmsmentioning

confidence: 99%

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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%

Section: Assessment Of Right-to-left Shuntingmentioning

confidence: 99%

Section: Screening For Pavmsmentioning

confidence: 99%

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

et al. 2007

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“…Acute complications from AVMs are the main cause of increased mortality, though inadequate healthcare maintenance plays a role as well [47]. There is a bimodal distribution of mortality, with peaks at age 50 and then from 60 to 79 [48]. The most life-threatening AVM sites are in the brain, the GI tract, and the lungs, which can lead to life-threatening cerebral hemorrhage, gastrointestinal bleeds, or severe hemoptysis [49].…”

Section: Introductionmentioning

confidence: 99%

Gastrointestinal Manifestations of Hereditary Hemorrhagic Telangiectasia (HHT): A Systematic Review of the Literature

et al. 2017

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Hereditary hemorrhagic telangiectasia (HHT), also called Osler–Weber–Rendu syndrome, is an autosomal dominant genetic disease that affects the vasculature of numerous organs. The prevalence of HHT is estimated to be between 1.5 and 2 persons per 10,000. While there is still much to learn about this condition, there is an increasing understanding its underlying pathophysiology, genetic basis, presentations, and management. Recognizing that the clinical manifestations of HHT can involve a number of organ systems will provide clinicians with a higher index of suspicion for the disease. This early diagnosis and genotyping can greatly reduce mortality for a patient with HHT through appropriate screening for complications. This review will focus on the gastrointestinal manifestations of HHT and how these can dictate treatment and prognosis.

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“…The ‘gold standard’ for the diagnosis of PAVM is a high-resolution CT scan of the thorax and/or angiography of the pulmonary vessels [18, 19]. …”

Section: Pulmonary Arteriovenous Malformationmentioning

confidence: 99%