ABSTRACT: A 49-year-old female with systemic fibromuscular dysplasia (FMD) presented with recurrent non-ST-segment elevation myocardial infarctions (NSTEMIs) with coronary angiography revealing complete normalization of a previously tapered posterior descending artery occlusion and new angiographic evidence of coronary FMD in her 2nd and 3rd diagonal branches illustrating the dynamic nature of coronary FMD.
VASCULAR DISEASE MANAGEMENT 2014;11(2):E34-E36
Key words: coronary artery disease, catheterization, nuclear cardiology
A 49-year-old female with renovascular hypertension and systemic fibromuscular dysplasia (FMD) of the iliac and renal arteries with previous balloon angioplasty of the right renal artery presented to our institution in 2006 with chest pain and non-ST-segment elevation myocardial infarction (NSTEMI). Coronary angiography revealed a tapered occlusion of the right posterior descending artery (PDA) (Figure 1A) and mild luminal irregularities of the diagonal branches (Figure 1B). Medical therapy for coronary artery disease was recommended.
She remained chest pain free with unlimited exercise tolerance until 2013 when she presented with chest pain 4 hours after running 6 miles in cold weather. Nuclear stress test revealed reversible ischemia in the mid to distal anterior and anterolateral walls. She subsequently developed recurrent chest pain and had an elevated troponin level of 4.4 ng/mL. Coronary angiography demonstrated complete normalization of the previously tapered PDA occlusion (Figure 2A) and angiographic evidence of coronary FMD in her 2nd and 3rd diagonal branches with a tapered, smooth contour of the mid to distal segments (Figure 2B), unresponsive to intracoronary nitroglycerin.
Coronary FMD is rare to encounter in the antemortem period although several groups have reported a series of cases with various clinical presentations ranging from atypical chest pain to NSTEMI to fulminant heart failure necessitating heart transplant.1,2 The exact prevalence of coronary FMD is unknown, as it is often challenging to make clinical distinction between coronary FMD and atherosclerosis. Advanced imaging techniques such as intravascular ultrasound and optical coherence tomography can support the diagnosis of FMD and will likely increase the detection of the disease, particularly in patients with spontaneous coronary artery dissections, many of which have been recently shown to have underlying coronary and systemic FMD.3,4 Our patient had a unique finding of an occluded right PDA in 2006, which was found to be spontaneously patent in 2013. While intravascular ultrasound was not performed in our case, her systemic evidence of iliac and renal artery FMD increases the likelihood of coronary FMD. The patient’s angiogram is consistent with severe coronary vasospasm or a recanalized coronary dissection in the setting of coronary FMD and illustrates the dynamic nature of coronary FMD.
Once the diagnosis of coronary FMD is established, and coronary atherosclerosis is excluded as a major contributing process, management strategy should focus on administration of coronary vasodilators and screening for involvement of other vascular beds, including carotid, renal, vertebral, and mesenteric arteries.
Editor’s Note: Disclosure: The author has completed and returned the ICMJE Form for Disclosure of Potential Conflicts of Interest. Dr. Feldman reports honoraria and educational or speaking engagements from Eli Lilly, Daiichi Sankyo, Abbott Vascular, Pfizer, and Bristol-Myers Squibb. Dr. Al’Aref and Dr. Swaminathan report no related disclosures.
Manuscript received May 4, 2013, provisional acceptance May 21, 2013, accepted September 13, 2013.
Address for correspondence: Rajesh V. Swaminathan, MD, Cardiac Catheterization Laboratories, New York Presbyterian Hospital, Weill Cornell Medical College, 520 East 70th Street, Starr-434 Pavilion, New York, New York 10021, United States. Email: firstname.lastname@example.org
Acknowledgements: The authors would like to thank Drs. Erica C. Jones, Mark S. Pecker, and Luke K. Kim for their consultation in this case and review of the manuscript for important intellectual content.
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- Castellano JM, Vaishnava P, Castillo JG, Anyanwu AC, Fuster V. Coronary vasospasm attributable to fibromuscular dysplasia: the long bridge to transplant. Circ Heart Fail. 2013;6(3):e31-e32.
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