Diffuse pulmonary arteriovenous malformations in hereditary hemorrhagic telangiectasia: long-term results of embolization according to the extent of lung involvement.
OBJECTIVE
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.


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 Pao(2) 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 Pao(2) 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. Pao(2) 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.


CONCLUSIONS
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 Pao(2) can be achieved in group 2.
Lacombe
Pascal
P
Department of Radiology, Hôpital Ambroise Paré, Assistance Publique-Hôpitaux de Paris, Université Paris Ile-de-France Ouest, Boulogne-Billancourt, France.
Lagrange
Christine
C
Department of Radiology, Hôpital Ambroise Paré, Assistance Publique-Hôpitaux de Paris, Université Paris Ile-de-France Ouest, Boulogne-Billancourt, France.
Beauchet
Alain
A
Department of Statistics and Public Health, Hôpital Ambroise Paré, Assistance Publique-Hôpitaux de Paris, Université Paris Ile-de-France Ouest, Boulogne-Billancourt, France.
El Hajjam
Mostafa
M
Department of Radiology, Hôpital Ambroise Paré, Assistance Publique-Hôpitaux de Paris, Université Paris Ile-de-France Ouest, Boulogne-Billancourt, France.
Chinet
Thierry
T
Department of Pulmonology, Hôpital Ambroise Paré, Assistance Publique-Hôpitaux de Paris, Université Paris Ile-de-France Ouest, Boulogne-Billancourt, France.
Pelage
Jean-Pierre
JP
Department of Radiology, Hôpital Ambroise Paré, Assistance Publique-Hôpitaux de Paris, Université Paris Ile-de-France Ouest, Boulogne-Billancourt, France. Electronic address: jean-pierre.pelage@apr.aphp.fr.
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001165:Arteriovenous Malformations / Q000000981:diagnostic imaging / Q000628:therapy*; D002648:Child; D004417:Dyspnea / Q000209:etiology; D004621:Embolization, Therapeutic / Q000009:adverse effects / Q000379:methods; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011652:Pulmonary Circulation; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications*; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome