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Original Research: PULMONARY ARTERIOVENOUS MALFORMATIONS |

Diffuse Pulmonary Arteriovenous Malformations in Hereditary Hemorrhagic Telangiectasia: Long-term Results of Embolization According to the Extent of Lung Involvement

Pascal Lacombe, MD; Christine Lagrange, MD; Alain Beauchet, MD; Mostafa El Hajjam, MD; Thierry Chinet, MD; Jean-Pierre Pelage, MD, PhD
Author and Funding Information

*From the Departments of Radiology (Drs. Lacombe, Lagrange, El Hajjam, and Pelage), Statistics and Public Health (Dr. Beauchet), and Pulmonology (Dr. Chinet), Hôpital Ambroise Paré, Assistance Publique-Hôpitaux de Paris, Université Paris Ile-de-France Ouest, Boulogne-Billancourt, France.

Correspondence to: Jean-Pierre Pelage, MD, PhD, Department of Radiology, Hôpital Ambroise Paré, Assistance Publique-Hôpitaux de Paris, Université Paris Ile-de-France Ouest, 9 Ave Charles de Gaulle, 92104 Boulogne-Billancourt Cedex, France; e-mail: jean-pierre.pelage@apr.aphp.fr


The authors have reported to the ACCP that no significant conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (www.chestjournal.org/misc/reprints.shtml).


Chest. 2009;135(4):1031-1037. doi:10.1378/chest.08-1794
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Objectives:  To review the safety of embolization in patients affected with hereditary hemorrhagic telangiectasia (HHT) presenting with diffuse pulmonary arteriovenous malformations (PAVMs). To correlate the initial presentation and long-term results of embolization according to the distribution of PAVMs.

Materials and methods:  All consecutively treated patients were divided into three groups, according to the involvement of every subsegmental pulmonary artery (group 1), segmental artery (group 2), or both (group 3) of at least one lobe. Age, sex, initial clinical presentation, and Pao2 were recorded before embolization. Per and postprocedural complications were carefully recorded. Clinical outcome and imaging follow-up were obtained at 6 months and annually thereafter.

Results:  Thirty-nine patients (31 women, 8 men; mean age, 35 years), all of them with bilateral lung involvement, were treated. Group 1 consisted of 8, group 2 of 17, and group 3 of 14 patients. Dyspnea was present in 35 of the patients (90%) and cyanosis in 17 patients (44%). Preembolization Pao2 was different between groups 1 (52.6 ± 11.6 mm Hg) and 3 (70.7 ± 14.1 mm Hg). Neurologic events were more frequently reported before treatment in group 1 (62.5%) than in group 2 (35%) or in group 3 (43%). Eighty percent of patients reported improvement in their dyspnea after embolization. Pao2 levels improved more in group 2 than in groups 1 and 3. Eight ischemic or infectious complications occurred in 4 patients (10%) due to reperfusion of embolized PAVMs or enlargement of non-embolized PAVMs. Complete and partial treatment success was reported using CT scanning in 59% and 38% of cases, respectively.

Conclusion:  Dyspnea and paradoxical embolism are frequently encountered in HHT patients with diffuse PAVMs. Prevention of complications and improvement of dyspnea can be achieved after successful embolization in most patients. Better improvement of Pao2 can be achieved in group 2.

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