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Case Reports
. 2022 Dec 15;61(24):3683-3686.
doi: 10.2169/internalmedicine.9516-22. Epub 2022 May 14.

Moyamoya Disease Complicating Ebstein's Anomaly

Itaru Goto  1 , Ryuji Okamoto  2   3 , Yu Sato  4 , Akihiro Tsuji  5 , Takashi Tanigawa  1 , Kaoru Dohi  2
Affiliations
Case Reports

Moyamoya Disease Complicating Ebstein's Anomaly

Itaru Goto et al. Intern Med. .

Abstract

Ebstein's anomaly is an uncommon congenital disorder affecting the tricuspid valve. We herein report a 38-year-old woman who experienced consciousness and sensory disturbance during treatment for heart failure caused by Ebstein's anomaly. Urgent magnetic resonance imaging and cerebral angiography demonstrated acute cerebral infarction and internal carotid artery obstruction with the development of collateral arteries. We diagnosed her with multiple cerebral infarctions due to moyamoya disease. Ebstein's anomaly concomitant with moyamoya disease is extremely rare. However, we should consider the possibility of this rare but important concurrence when treating patients with heart failure due to Ebstein's anomaly to avoid excessive diuresis and vasodilation and irreversible brain injury.

Keywords: Ebstein's anomaly; cerebral infarction; heart failure; moyamoya disease.

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Conflict of interest statement

The authors state that they have no Conflict of Interest (COI).

Figures

Figure 1.
Figure 1.
Multi-modal imaging demonstrating the findings in the present case. A: Electrocardiographic findings on hospital day 1 showing sinus tachycardia, pulmonary P waves in leads II and aVF and complete right bundle branch block. B: Supine chest X-ray showing prominent cardiomegaly and pleural effusion. C: Transthoracic echocardiography showing 29-mm (17.6-mm/m2) apical displacement of the septal tricuspid leaflet from the insertion of the anterior mitral leaflet. LA: left atria, LV: left ventricle, RA: right atria, RV: right ventricle, D: Color Doppler imaging showing severe tricuspid regurgitation (TR).
Figure 2.
Figure 2.
Brain magnetic resonance imaging (MRI), magnetic resonance angiography (MRA) and selective angiography. A: Diffusion-weighted MRI showing acute ischemic infarction (red arrows) in the bilateral frontal lobes (upper panel) and in the left genu of the corpus callosum (lower panel). B: MRA showing stenosis and occlusion of the terminal portions of the internal carotid arteries (red arrowheads). C, D: Selective cerebral angiogram showing severe stenosis (red arrowheads) from the right internal carotid artery to the middle cerebral artery (M1 portion), occlusion of the left carotid artery, and right (C) and left (D) carotid injection, frontal views.
See this image and copyright information in PMC

References

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Publication types

Supplementary concepts