Myocardial ischemia in the absence of epicardial coronary artery disease in Friedreich's ataxia.
We present the first in vivo detection of microvascular abnormality in a patient with Friedreich's ataxia (FA) without epicardial coronary artery disease using cardiac magnetic resonance (CMR). The patient had exertional chest pain and dyspnea prompting referral for cardiac evaluation. These symptoms were reproduced during intravenous adenosine infusion, and simultaneous first-pass perfusion imaging showed a significant subendocardial defect; both symptoms and perfusion deficit were absent at rest. Epicardial coronaries were free of disease by invasive angiography; together, these findings support the notion of impaired myocardial perfusion reserve in FA.
Raman
Subha V
SV
The Ohio State University and Nationwide Children's Hospital, Columbus, OH, USA. raman.1@osu.edu
Dickerson
Jennifer A
JA
Al-Dahhak
Roula
R
D000241:Adenosine / Q000008:administration & dosage; D000328:Adult; D000787:Angina Pectoris / Q000209:etiology / Q000473:pathology; D017023:Coronary Angiography; D003324:Coronary Artery Disease / Q000473:pathology; D003326:Coronary Circulation; D004417:Dyspnea / Q000209:etiology / Q000473:pathology; D005260:Female; D005621:Friedreich Ataxia / Q000150:complications* / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D007262:Infusions, Intravenous; D008279:Magnetic Resonance Imaging; D008833:Microcirculation / Q000473:pathology / Q000503:physiopathology; D017202:Myocardial Ischemia / Q000209:etiology / Q000473:pathology* / Q000503:physiopathology; D010496:Pericardium / Q000473:pathology; D014665:Vasodilator Agents / Q000008:administration & dosage
D014665:Vasodilator Agents; D000241:Adenosine