Images that Teach
Coronary vasculopathy due to moyamoya disease

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Abstract

Moyamoya disease is a rare disorder associated with progressive intracranial arterial stenosis with fragile, small collateralization that gives an angiographic appearance of a puff of smoker or, in Japanese, “moya-moya”. We report a case of coronary artery ostial occlusive disease as an extracranial manifestation of Moyamoya. In the case, we demonstrate that thigh risk features of cardiac positron emission tomography (PET) that ultimately lead to the diagnosis of coronary artery occlusion.

Section snippets

Case presentation

Forty-six year old right handed Caucasian female with a long standing history of bilateral intracranial artery (ICA) occlusion with collateralization diagnosed 18 years prior to current presentation. Computed tomographic angiography (CTA) head and neck revealed chronic total occlusion of bilateral ICAs in the cavernous segment due to Moyamoya phenomenon.

She presented to outpatient cardiology clinic with complaint of chest pain with exertion, pressure like in the center of her chest radiating to

Discussion

Moyamoya is a rare disorder and patients predominantly present with intracerebral hemorrhage. Diagnosis can be made using noninvasive modalities via magnetic resonance angiography (MRA) or CTA. Invasive modality of choice is catheter angiography. Rarely extracranial arterial involvement due to endothelial hyperplasia has been reported. There have been a few reports of ostial coronary artery stenosis as a systemic manifestation of Moyamoya disease.2,3 The histopathology of these lesions showed

New knowledge gained

Cardiac manifestations of moyamoya disease can present as proximal coronary artery stenosis due to intimal proliferation. Diagnosis and management of this necessitates a multimodality imaging approach as detailed.

Teaching points

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    PET Rubidium Cardiac study has several high risk features all of which can we observed in our case. These include

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    Increased right ventricular uptake

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    Transient ischemic dilatation

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    Decrease in ejection fraction with stress

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    Low myocardial blood flow reserves (< 1.8)

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    Large area of ischemia/scar > 20%

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    Dobutamine PET is the modality of choice for assessment of ischemia in anomalous coronary arteries

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    It is important to review the attenuation CT to assess for any anomalous coronaries.

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    Moyamoya disease can be

Disclosure

Simrat Kaur, Parth Parikh, Jaikirshan Khatri and Wael Jaber have no conflicts of interests to declare and meet the criteria for authorship - drafting of case report or revising it critically for important intellectual content, and final approval of the manuscript. All the authors have nothing additional to disclose.

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A correction to this article is available online at https://doi.org/10.1007/s12350-022-03195-y.

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