Pulmonary arteriovenous malformations: lung transplantation as a therapeutic option

Reynaud‐Gaubert, Martine; Thomas, Philippe; Gaubert, J; Pietri, P; Garbe, L; Giudicelli, R; Orehek, J; Fuentès, P.

doi:10.1183/09031936.99.14614259

Cited by 31 publications

(15 citation statements)

References 11 publications

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“…A combined therapeutic approach with endovascular and video-assisted thoracoscopic procedure has been described for the treatment of hemothorax due to a ruptured PAVM [135]. Lung transplantation has been done in few HHT patients with severe and diffuse PAVMs [136,137,138]. …”

Section: Treatment Of Pavmsmentioning

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: Treatment Of Pavmsmentioning

confidence: 99%

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

et al. 2007

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“…Pulmonary transplantation has been performed in diffuse forms associated with respiratory insufficiency [26] Percutaneous image-guided embolotherapy is nowadays preferable in most disease cases. The aim of transcatheter embolization is to occlude all the PAVM feeding arteries by a selective catheterization of pulmonary arteries by using a coaxial system.…”

Section: Consequences Of the Hypothesis And Discussionmentioning

confidence: 99%

Roles of cyclooxygenase 2 and hepatic venous flow in patients with HHT or hepatopulmonary syndrome

Marcy¹,

Thariat²,

Sellier³

et al. 2014

Medical Hypotheses

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“…Three successful lung transplantations for cases of diffuse pulmonary AVMs have been reported to date. The first case was an adult male who underwent bilateral lung transplantation because he had failed to respond to embolotherapy . The second case was an adult female with diffuse pulmonary AVMs as a component of HHT who underwent single lung transplantation .…”

Section: Discussionmentioning

confidence: 99%

“…On the other hand, the therapeutic strategy for diffuse pulmonary AVMs is still controversial as transcatheter embolotherapy is not necessarily effective over the long term. Lung transplantation is obviously an option for such patients: there have been three reports of successful lung transplantation in patients with diffuse AVMs and HHT . However, in a case with pulmonary AVMs and HHT the long‐term prognosis was unfavorable .…”

Section: Introductionmentioning

confidence: 99%

Successful Lung Transplantation in a Case With Diffuse Pulmonary Arteriovenous Malformations and Hereditary Hemorrhagic Telangiectasia

Fukushima

Mitsuhashi

Oto

et al. 2013

American Journal of Transplantation

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Diffuse pulmonary arteriovenous malformations (AVMs) are associated with a poor prognosis and the therapeutic strategy remains controversial. We describe a pediatric patient with diffuse pulmonary AVMs associated with hereditary hemorrhagic telangiectasia (HHT), who presented with two cerebral AVMs in the parietal and occipital lobes as well. Of note, successful bilateral lung transplantation not only improved the hypoxemia but also resulted in size reduction of the cerebral AVMs. Although it is essential to consider involvements other than pulmonary AVMs, especially brain AVMs, to decide the indication, lung transplantation can be a viable therapeutic option for patients with diffuse pulmonary AVMs and HHT.

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Pulmonary arteriovenous malformations: lung transplantation as a therapeutic option

Cited by 31 publications

References 11 publications

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

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

Roles of cyclooxygenase 2 and hepatic venous flow in patients with HHT or hepatopulmonary syndrome

Successful Lung Transplantation in a Case With Diffuse Pulmonary Arteriovenous Malformations and Hereditary Hemorrhagic Telangiectasia

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