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Coronary Tortuosity: A Clue to the Diagnosis of Fibromuscular Dysplasia?

BACKGROUND: Fibromuscular dysplasia (FMD) is a systemic, nonatherosclerotic, noninflammatory vasculopathy that is often overlooked by clinicians. Clinical clues could help in selecting patients for further evaluation for the presence of FMD. Recently, it was observed that tortuosity of the coronary arteries is often present in patients with FMD-related abnormalities of the coronary artery. Therefore, we wondered if the presence of coronary tortuosity might provide a clinical clue to the diagnosis of extracoronary FMD.

CASES: We describe 5 cases of FMD in whom diagnostic studies for FMD were initiated because of the presence of coronary tortuosity. FMD was found in all 5 patients in the renal and/or cervical arteries.

CONCLUSIONS: Our 5 cases suggest that exertional chest pain in the presence of coronary tortuosity may be helpful in selecting patients for further evaluation for the presence of FMD. Further research should focus on the prevalence of FMD among patients with coronary tortuosity and whether the presence of additional clinical clues (such as the presence of hypertension at young age or pulsatile tinnitus) next to coronary tortuosity can predict the risk for FMD in individual patients.

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