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Fatal rupture of pulmonary arteriovenous malformation in hereditary haemorrhagic telangiectasis and severe PAH

^* Université Paris-Sud 11, Centre National de Référence de l'Hypertension Artérielle Pulmonaire, Service de Pneumologie et Réanimation Respiratoire, Hôpital Antoine-Béclère, Assistance Publique – Hôpitaux de Paris, Clamart, ^# Service de Pneumologie, and ^¶ Service de Radiologie, Université Paris Ile-de-France Ouest, Consultation pluridisciplinaire maladie de Rendu-Osler, Hôpital Ambroise Paré, Assistance Publique – Hôpitaux de Paris, Boulogne, France.

CORRESPONDENCE: D. Montani, Centre National de Référence de l'Hypertension Artérielle Pulmonaire, Service de Pneumologie et Réanimation Respiratoire, Hôpital Antoine-Béclère, 157 rue de la Porte de Trivaux, 92140 Clamart, France. Fax: 33 14630384. E-mail: david.montani{at}abc.aphp.fr

Received: March 17, 2008
Accepted November 11, 2008

ABSTRACT

A 29-yr-old patient with hereditary haemorrhagic telangiectasia was referred to the present authors’ centre with progressive exertional dyspnoea.

Pulmonary arterial hypertension (PAH) was suspected on Doppler echocardiography and confirmed by right heart catheterisation demonstrating severe PAH. Genetic analysis found an activin receptor-like kinase-1 gene missense mutation. Chest radiography and computed tomodensitometry of the chest revealed a pulmonary arteriovenous malformation with a 5-mm diameter feeding artery in the right lower lobe. Embolisation of the arteriovenous malformation was discussed, but was considered a very high-risk procedure that could aggravate PAH and was therefore not performed. Haemodynamics were improved by dual endothelin receptor antagonist and inhaled iloprost but the patient subsequently died suddenly of a rupture of the arteriovenous malformation into the pleural cavity.

Severe PAH is generally considered a contraindication to performing pulmonary arteriovenous malformation embolisation because of the risk of worsening of PAH. However, given the significant risk of rupture, paradoxical embolism and haemoptysis, and the lack of data regarding the evolution of pulmonary pressure after embolisation in PAH, pulmonary arteriovenous malformation embolisation should not be absolutely contraindicated and might be considered in patients with stable PAH.

KEYWORDS: Activin receptor-like kinase-1, arteriovenous malformation, embolisation, hereditary haemorrhagic telangiectasia, pulmonary arterial hypertension, rupture