Working Diagnosis:
Spontaneous Coronary Artery Dissection (SCAD) of the left main artery, left circumflex artery, and ramus artery
Treatment:
Given the results of the angiogram and an intra-procedural EF of 10%, Cardiothoracic Surgery was consulted and performed emergent two vessel coronary artery bypass graft (CABG). Prior to his CABG, the transesophageal echocardiogram (TEE) confirmed an EF of 10-15%. Post-operatively, EF was improved to 30-35%. He was referred to a specialty clinic for SCAD and for genetic testing.
Outcome:
Given the severity of his condition and the unknown etiology of SCAD in this patient, he was referred to a SCAD clinic where he was evaluated for and found to have evidence of Fibromuscular Dysplasia. He was also found to have a genetic mutation in the Ehler-Danlos Syndrome family. He has since been able to run 3 miles, but not cleared for contact sports.
Author's Comments:
SCAD is a rare cause of myocardial infarction accounting for 0.1-4% of cases. SCAD is most common in young females. Risk factors for SCAD include female gender, fibromuscular dysplasia, heavy exercise, postpartum period, atherosclerosis and connective tissue disorders. The typical presentations are chest pain, elevated troponins, sudden cardiac death, and ventricular arrhythmias, which makes this condition difficult to distinguish from other cardiac ischemic events. Given his young age, he was sent for genetic testing as Fibromuscular dysplasia is a possible etiology of SCAD in a young male.
Editor's Comments:
This case shows that spontaneous coronary artery dissection should be in the differential of younger patients with sudden cardiac death, chest pain, and syncope though it is quite rare. SCAD is more common in females, especially young females and around 80% of cases are due to fibromuscular dysplasia. Ten to 15% of cases are males. In a study of SCAD, 16% of patients had a normal EKG, and ST elevation in 46% of EKGs. Initial troponin levels were normal in 20% of patients. Therefore, a high index of suspicion is necessary to make the diagnosis. Diagnosis is made by coronary angiography. Treatment depends on how stable the patient is and the amount of ischemia with most treated conservatively and if unstable or complications then CABG.
References:
1. Nishiguchi T, Tanaka A, Ozaki Y, et al. Prevalence of spontaneous coronary artery dissection in patients with acute coronary syndrome. Eur Heart J Acute Cardiovasc Care. 2016;5(3):263-270. doi:10.1177/2048872613504310
2. Mokhberi V, Bagheri B, Navidi S, Amini SM. Spontaneous Coronary Artery Dissection: A Case Report. J Tehran Heart Cent. 2015 Jul 3;10(3):159-62. PMID: 26697091; PMCID: PMC4685374.
3. Hayes SN, Tweet MS, Adlam D, Kim ESH, Gulati R, Price JE, Rose CH. Spontaneous Coronary Artery Dissection: JACC State-of-the-Art Review. J Am Coll Cardiol. 2020 Aug 25;76(8):961-984. doi: 10.1016/j.jacc.2020.05.084. PMID: 32819471.
4. Johnson et al. Acad Emerg Med. 2022;29(4):423. Epub 2021 Dec 26. PMID
34897898
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