Download Peripheral pulmonary artery stenoses in the setting of Moyamoya

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doi:10.1093/ehjci/jew006
Online publish-ahead-of-print 9 February 2016
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Peripheral pulmonary artery stenoses in the setting of Moyamoya
Pamela Moceri1*, Jeremy Laı̈k1, Hélène Bouvaist2, Alain Fraisse3, and Emile Ferrari1
1
Cardiology Department, Hôpital Pasteur, CHU de Nice, Nice, FR; 2Cardiology Department, CHU de Grenoble, Grenoble, FR; and 3Paediatric Cardiology Department,
Royal Brompton Hospital, London, UK
* Corresponding author. E-mail: [email protected]; [email protected]
Moyamoya is an uncommon cerebral vasculopathy characterized by progressive stenosis of the
circle of Willis and its principle branches. Moyamoya is more frequent in east-Asian countries.
Two entities should be separated: Moyamoya disease, the isolated cerebral angiopathy, multifactorial and polygenic, and Moyamoya syndrome
associating cerebral angiopathy with other symptoms and various modes of inheritance.
A 21-year-old Caucasian man was referred to
our hospital for pulmonary arterial (PA) hypertension. He had history of Moyamoya, diagnosed
at the age of 13 and treated with antiplatelet therapy. Brain magnetic resonance reported reduced
flow voids in the cerebral arteries and prominent
collateral arteries (Panel A).
On admission, the patient complained of
NYHA class III dyspnoea; his physical examination revealed right sternal border systolic murmur and right heart failure. Trans-thoracic
echocardiography (Panel B) revealed severe right ventricular hypertrophy, dilatation, and systolic dysfunction. Dilatation of the main PA
was observed, without any evidence of stenosis of the proximal branches. Right heart catheterization confirmed the elevated PA pressures (s/d/m 85/25/45 mmHg –systemic pressures s/d/m 130/70/90 mmHg) with normal capillary wedge pressure (10 mmHg), increased
pulmonary vascular resistance (16.8 Wood U), elevated diastolic gradient (15 mmHg), and decreased cardiac index (1.5 L/min/m2). CT
scan (Panel C) revealed multiple peripheral PA stenoses with post-stenotic aneurysms. Pulmonary angiography (Panel D) confirmed
the multiple and distal character of PA branch stenoses and allowed to perform a procedure of pulmonary angioplasty. After the attempt
of left PA angioplasty, we obtained a reduction in PA pressures (74/38/20) with preserved cardiac index. Unfortunately, the patient
developed reperfusion pulmonary injury complicated by acute heart failure and finally died from complications.
Only few reports suggest the co-existence of pulmonary vascular disease with Moyamoya. This case illustrates the incremental value of
multimodality imaging in patients with pulmonary vascular disease.
Published on behalf of the European Society of Cardiology. All rights reserved. & The Author 2016. For permissions please email: [email protected].