Abstract
A 49-year-old woman affected by fibromuscular dysplasia (FMD) of the carotid artery with recurrent coronary vasospasm, refractory to medical therapy was admitted for anginal pain. Coronary involvement related to FMD is uncommon and its diagnosis “in vivo” still represents a challenge. Although a clear association between FMD and spontaneous coronary artery dissections is described, the presentation as severe coronary vasospasm is rare and it is associated with unfavorable outcome, especially when treated by means of percutaneous coronary intervention. The use of intravascular ultrasound played a key role in choosing the appropriate strategy for the management of the patient.
< Learning objective: During the past years fibromuscular dysplasia (FMD) has been recognized as an underdiagnosed underlying pathology possibly associated with acute coronary syndromes, especially in the setting of young women. Coronary involvement has been recently associated with vasospasm, reported in previous cases as severe refractory vasospasm. Intravascular imaging techniques are able to detect FMD structural coronary abnormalities, aiming for clinicians to choose the appropriate strategy for the management of the patient.>
Introduction
The involvement of the coronary artery in fibromuscular dysplasia (FMD) is uncommon. This case is about a 49-year-old woman admitted for refractory vasospastic angina with subsequent detection of bilateral “mouse tail” aspect of internal carotid arteries compatible with FMD. Based on intravascular ultrasound, clinicians decided to avoid interventional strategy in favor of a conservative option, with the introduction of optimized medical therapy leading to symptom relief.
Case report
The case is about a 49-year-old woman admitted to the emergency department of our institution due to severe and worsening episodes of typical chest pain. She had no cardiovascular risk factors, except for previous smoking habits. Despite the young age, she had a complex past medical history. She experienced two transient ischemic attacks (TIA) at the age of thirty, characterized by slurred speech and weakness of both arms. In 2015 she was admitted to her hometown hospital for chest pain, with both negative electrocardiogram (ECG), coronary angiography (CA), and a clinical presentation suggestive of vasospastic angina. Moreover, she was hospitalized for epileptic seizure in 2017.
Chronic medical therapy included acetylsalicylic acid (75 mg/day), statins (simvastatin 20 mg/day), beta-blockers (metoprolol 50 mg twice a day), and oral nitrates (isosorbide mononitrate 20 mg three times daily). The patient also took sublingual nitrate (isosorbide dinitrate sublingual 5 mg) twice a month, for episodic anginal pain.
At the admission, the blood tests showed a slight elevation of troponin T (peak 156 ng/L) and the echocardiogram revealed a focal hypokinesis of the anterior distal septal region of the left ventricle with a slightly depressed ejection fraction (50%). During the examination, the patient complained of an intense episode of chest pain associated with profuse sweating and nausea. The ECG ( Fig. 1 B) showed a concomitant significant ST elevation in the anterior precordial leads, which led to an urgent CA showing severe spasm of the proximal left anterior descending (LAD) coronary artery ( Fig. 1 C), resolved after intracoronary nitrates infusion ( Fig. 1 D). Distally, the LAD artery presented with a tortuous path. Intravascular ultrasound virtual histology (IVUS/VH) was performed confirming the absence of dissections or vulnerable atherosclerotic plaque. In addition, a hypo-echogenic plaque was detected compatible with pathological fibrous intimal thickening (800 µm) at VH analysis, and a plaque burden of 42% ( Fig. 1 E).
