Acute coronary syndrome due to suspected fibromuscular dysplasia – a case report

    Authors

    Keywords

    acute coronary syndrome, fibromuscular dysplasia, myocardial infarction with non-obstructive coronary arteries

    DOI

    https://doi.org/10.15836/ccar2022.154

    Full Text

    **Introduction**: Fibromuscular dysplasia (FMD) of coronary arteries is a rare disorder that can present as an acute coronary syndrome, left ventricular failure or even sudden cardiac death. The most common manifestation of FMD is spontaneous coronary dissection (SCAD) with intramural hematoma. Percutaneous coronary intervention (PCI) for SCAD carries a significant risk of adverse outcomes due to dissection propagation. (1-3) **Case report**: We present a 58-year-old woman, who was hospitalized in the Department of Cardiovascular diseases due to chest pain and high troponin values. The ECG was normal and after taking a fast-acting nitrate, the symptoms subsided. Coronary angiography showed normal epicardial arteries and the diagnosis of MINOCA (myocardial infarction with non-obstructive coronary arteries) was established. Echocardiography revealed a normal systolic function with mild hypocontractility of the inferior and inferolateral wall. The patient was discharged without antiplatelet therapy due to aspirin allergy. She was re-hospitalized for the same symptoms seven months later. A repeated coronary angiography revealed a severe stenosis of the distal first obtuse marginal artery (**Figure 1**) and SCAD was suspected. Therefore, coronary angiography of the initial hospitalization was revised and a significant stenosis of first diagonal artery (D1), which was overlooked during the initial coronary angiography, was found (**Figure 2**). Since the D1 was now completely normal, the diagnosis of possible fibromuscular dysplasia was made. She was treated conservatively and was discharged with clopidogrel and statin therapy. FIGURE 1. Comparison of coronary angiography findings showing stenosis of the distal first obtuse marginal artery (left) and normal findings (right). FIGURE 2. Comparison of coronary angiography findings showing stenosis of the first diagonal artery (left) and normal findings (right). **Conclusion**: 58-year-old female was hospitalized twice due to an acute coronary syndrome because of suspected FMD. Although the etiology is poorly known, attempts are still being made to clarify FMD’s genetic and molecular underpinnings. Since PCI has worse outcomes, a conservative therapy is typically preferred because lesions heal on their own.

    Literature

    1. Khoury MH, Gornik HL. Fibromuscular dysplasia (FMD). Vasc Med. 2017 June;22(3):248–52. https://doi.org/10.1177/1358863X17700716
    2. Kuzyk J, Boiko O, Stetsko T. Fibromuscular Dysplasia of the Coronary Arteries: A Case Report and Review of the Literature. Turk Patoloji Derg. 2018;34(3):269–73. https://doi.org/10.5146/tjpath.2015.01341
    3. Akiba Y, Endo A, Suzuki K. Fibromuscular Dysplasia of Coronary and Brachial Artery. Can J Cardiol. 2018 December;34(12):1689.e3–4. https://doi.org/10.1016/j.cjca.2018.09.002
    Cardiologia Croatica
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    Acute coronary syndrome due to suspected fibromuscular dysplasia – a case report

    Extended Abstract
    Issue9-10
    Published
    Pages154
    PDF via DOIhttps://doi.org/10.15836/ccar2022.154
    acute coronary syndrome
    fibromuscular dysplasia
    myocardial infarction with non-obstructive coronary arteries

    Authors

    Drago Baković*ORCIDUniversity of Zagreb, Zagreb, Croatia
    Matea Bilić PavlinovićORCIDUniversity of Zagreb, Zagreb, Croatia
    Tony RumoraORCIDUniversity of Zagreb, Zagreb, Croatia
    Denis DošenORCIDUniversity of Zagreb School of Medicine, Zagreb, Croatia
    Miroslav KrpanORCIDUniversity of Zagreb School of Medicine, Zagreb, Croatia
    Kristina Marić BešićORCIDUniversity of Zagreb School of Medicine, Zagreb, Croatia

    *Correspondence email: dragobakovic2@gmail.com

    Full Text

    Introduction: Fibromuscular dysplasia (FMD) of coronary arteries is a rare disorder that can present as an acute coronary syndrome, left ventricular failure or even sudden cardiac death. The most common manifestation of FMD is spontaneous coronary dissection (SCAD) with intramural hematoma. Percutaneous coronary intervention (PCI) for SCAD carries a significant risk of adverse outcomes due to dissection propagation. (1–3)

    Case report: We present a 58-year-old woman, who was hospitalized in the Department of Cardiovascular diseases due to chest pain and high troponin values. The ECG was normal and after taking a fast-acting nitrate, the symptoms subsided. Coronary angiography showed normal epicardial arteries and the diagnosis of MINOCA (myocardial infarction with non-obstructive coronary arteries) was established. Echocardiography revealed a normal systolic function with mild hypocontractility of the inferior and inferolateral wall. The patient was discharged without antiplatelet therapy due to aspirin allergy. She was re-hospitalized for the same symptoms seven months later. A repeated coronary angiography revealed a severe stenosis of the distal first obtuse marginal artery (Figure 1) and SCAD was suspected. Therefore, coronary angiography of the initial hospitalization was revised and a significant stenosis of first diagonal artery (D1), which was overlooked during the initial coronary angiography, was found (Figure 2). Since the D1 was now completely normal, the diagnosis of possible fibromuscular dysplasia was made. She was treated conservatively and was discharged with clopidogrel and statin therapy.

    FIGURE 1. Comparison of coronary angiography findings showing stenosis of the distal first obtuse marginal artery (left) and normal findings (right).

    FIGURE 2. Comparison of coronary angiography findings showing stenosis of the first diagonal artery (left) and normal findings (right).

    Conclusion: 58-year-old female was hospitalized twice due to an acute coronary syndrome because of suspected FMD. Although the etiology is poorly known, attempts are still being made to clarify FMD’s genetic and molecular underpinnings. Since PCI has worse outcomes, a conservative therapy is typically preferred because lesions heal on their own.

    Literature

    1. 1.
      Khoury MH, Gornik HL. Fibromuscular dysplasia (FMD). Vasc Med. 2017 June;22(3):248–52.DOI
    2. 2.
      Kuzyk J, Boiko O, Stetsko T. Fibromuscular Dysplasia of the Coronary Arteries: A Case Report and Review of the Literature. Turk Patoloji Derg. 2018;34(3):269–73.DOI
    3. 3.
      Akiba Y, Endo A, Suzuki K. Fibromuscular Dysplasia of Coronary and Brachial Artery. Can J Cardiol. 2018 December;34(12):1689.e3–4.DOI