n
880
© Europa Edition 2011. All rights reserved.
HOW SHOULD I TREAT?
EuroIntervention 2011;7:880-885 DOI: 10.4244/EIJV7I7A137
How should I treat pulmonary arteriovenous malformations in
a patient with Rendu-Osler disease presenting with transient
ischaemic attack
Flavio d’Ascenzi
1,3
, MD; Valerio Zacà
2
, MD; Alessandro Iadanza
3
, MD; Sergio Mondillo
1
, MD; Carlo Pierli
3
, MD
1. Department of Cardiovascular Diseases, University of Siena, Siena, Italy; 2. Division of Cardiology, Santa Maria alle Scotte
Hospital, Siena, Italy; 3. Division of Interventional Cardiology, Santa Maria alle Scotte Hospital, Siena, Italy
This paper also includes accompanying supplementary data published at the following website: www.eurointervention.org
*Corresponding author: Department of Cardiovascular Diseases, University of Siena, Viale M. Bracci 16, 53100 Siena, Italy.
E-mail: flavio.dascenzi@libero.it
PRESENTATION OF THE CASE
A 42-year-old man was admitted to the local Neurology Department
to undergo evaluation of an episode of transient ischaemic attack
(TIA). He had no cardiovascular risk factors and his physical exam-
ination was unremarkable. As a part of routine diagnostic proce-
dures, he was referred to our echocardiography laboratory to
undergo a transesophageal echocardiogram (TEE) for evaluation of
potential cardio-embolic causes of the cerebrovascular disease and
to investigate the presence of a patent foramen ovale (PFO). A TEE
with saline contrast was performed, demonstrating the presence of a
significant amount of bubbles in the left atrium (LA) consistent with
the presence of a relevant right-to-left shunt despite no evidence of
atrial or ventricular septal defects. The late timing of appearance of
bubbles in the LA, after five heart cycles, was not compatible with
inter-atrial septal defects such as a PFO but rather strongly sugges-
tive of the presence of an intrapulmonary shunting and allowed us
to differentiate intracardiac shunting from intrapulmonary shunting,
as a result in this case of the presence of arteriovenous malforma-
tions. The patient was affected by nasal telangectasias, recurrent
epistaxis and gastrointestinal bleeding, suggestive of hereditary
haemorrhagic telangectasia (HHT).
The finding was subsequently confirmed as the patient, after
informed consent was obtained, underwent a right heart catheterisa-
CASE SUMMARY
BACKGROUND: A 42-year-old man with no cardiovascular
risk factors or history was admitted for evaluation of an
episode of transient ischaemic attack (TIA). He had no
cardiovascular risk factors and his physical examination
was unremarkable.
INVESTIGATION: Physical examination, electrography,
transesophageal echocardiogram, coronary angiogram.
DIAGNOSIS: Intrapulmonary shunting, in the presence of
arteriovenous malformations, possible hereditary haemor-
rhagic telangectasia
TREATMENT: Transcatheter occlusion of multiple pulmo-
nary arteriovenous malformations using an AMPLATZER
vascular plug.
KEYWORDS: pulmonary arteriovenous malformations,
Rendu-Osler disease, transient ischaemic attack