ST-Elevation Myocardial Infarction (STEMI) in a Patient with Moyamoya Disease

Abstract

Moyamoya disease is a rare condition that is primarily reported in Asian populations, characterized by stenoocclusive intracranial angiopathy with small, fragile, and multiple collateral vessel formation. Extracranial complications, mainly abnormalities within the renal vasculature, have been described; however, there are very few case reports of cardiovascular complications in patients with Moyamoya disease. We report a 26-year-old Caucasian female with known Moyamoya disease who presented with both typical and atypical chest pain, mimicking symptoms of a previous non-ST-elevation myocardial infarction. Approximately six months prior to the current hospital admission, she underwent coronary angiography requiring percutaneous coronary intervention (PCI) with two drug-eluting stents to the right coronary artery (RCA) for a critical stenosis. Despite medical management, our patient developed inferior lead ST-elevations leading to a repeat left heart catheterization which showed clinically significant stenosis of the first obtuse marginal branch. Development of significant coronary artery stenosis in a short period of time demonstrates the clinical significance of minimal atherosclerosis in the setting of underlying fibrocellular thickening as seen in patients with Moyamoya disease. Clinicians need to be aware of the possibility of coronary involvement in addition to intracranial vascular complications in patients with Moyamoya disease and take appropriate measures to prevent or delay the development of atherosclerosis in these arteries.

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“…Interestingly, a recent case report by James Livesay and Jeffrey Johnson indicated a direct incidence of STEMI in an MMD patient. Perhaps, to the best of our knowledge, this is one of the first-in-class direct case reports of CAD in a Caucasian female with reported MMD, presenting with cerebral vasculature complications ( 79 ). They emphasized a critical notion that extracranial vascular complications in MMD patients are rare but can significantly impact a patient’s overall morbidity and mortality.…”

“…MMD could likely lead to occlusion of coronary arteries through certain systemic etiologic factors in the blood. Many other studies also supported a correlation between MMD and CAD (67,79). Now, concerning the RA cases, a recent report showed that a patient with a history of RA for 15 years also suffered from cerebral rheumatoid vasculitis (81).…”

“…Thus, clinicians should be aware of the cardiovascular complications, including the coronary plaque progression seen in MMD patients, and assess for adequate medical management. Another contemporary study by Peng et al ( 79 ) stated that CAD used to share many clinical symptoms with MMD, including progressive narrowing or occlusion of involved arteries. MMD has emerged partially analogous to CAD on the peripheral blood transcriptomic level plausibly due to the underlying inflammations ( 79 ).…”

“…Another contemporary study by Peng et al ( 79 ) stated that CAD used to share many clinical symptoms with MMD, including progressive narrowing or occlusion of involved arteries. MMD has emerged partially analogous to CAD on the peripheral blood transcriptomic level plausibly due to the underlying inflammations ( 79 ). In addition, cases of CAD in patients with MMD have been extensively reported ( 65 , 72 , 77 , 79 , 80 ).…”

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“…Furthermore, Xing Peng et al discovered that MDD patients with downregulated GZMB were also enriched with the downregulated genes of CAD (Peng et al, 2019). Many other studies also supported a correlation between MDD and CAD (Livesay & Johnson, 2019;Nam et al, 2015). Concerning the RA cases, a recent report showed that a patient with a history of RA for 15 years also suffered from cerebral rheumatoid vasculitis (El-Sudany et al, 2021).…”