A case of vascular Behçet’s disease complicated with acute myocardial infarction due to spontaneous coronary artery dissection

Abstract

Spontaneous coronary artery dissection (SCAD) is a rare cause of acute myocardial infarction and occurs predominantly in young women without conventional cardiovascular risk factors. Emotional or physical stress and hormone imbalance are associated with the occurrence of SCAD, but the precise mechanisms have not been sufficiently elucidated. Furthermore, there is a paucity of data regarding the underlying disease in patients with SCAD. We herein report a case involving a 48-year-old man who developed acute myocardial infarction due to SCAD in the distal segment of the left anterior descending artery and was successfully treated with conservative medical therapy. A repeat coronary angiography examination performed 2 weeks later revealed complete angiographic healing. In addition, he was treated for suspected Behçet’s disease because he had a history of recurrent aphthous stomatitis, nodular erythema, and genital ulcers. After thorough assessment, he was finally diagnosed with vascular Behçet’s disease based on additional evidence of bilateral deep vein thrombosis. This case report highlights a rare case of vascular Behçet’s disease complicated with SCAD in a male patient. Physicians should be aware that SCAD may occur in association with systemic inflammatory disease including Behçet’s disease, even in male patients.

<Learning objective: Spontaneous coronary artery dissection (SCAD) is a rare disease entity that can cause acute myocardial infarction especially in young individuals without significant cardiovascular risk factors. We herein describe a 48-year-old man who was diagnosed with vascular Behçet’s disease after undergoing conservative medical therapy for acute myocardial infarction due to SCAD. This case report highlights that SCAD may occur in association with systemic inflammatory disease including Behçet’s disease, even in male patients.>

Introduction

Spontaneous coronary artery dissection (SCAD) is a rare disease entity that can cause acute myocardial infarction by tearing of the arterial media of coronary arteries, especially in young individuals without significant cardiovascular risk factors [1], [2]. Approximately 90% of patients with SCAD are women [1], [3], [4]. Emotional or physical stress and hormone imbalance are associated with the occurrence of SCAD [1], [2], but the precise mechanisms have not been thoroughly clarified. Furthermore, scarce data exist regarding the underlying disease in patients with SCAD. Previous studies have shown that fibromuscular dysplasia is the most common underlying disease in patients with SCAD [1], [4]. However, there is a paucity of data regarding the relationship between systemic inflammatory diseases, including Behçet’s disease, and SCAD. Only one case report to date has described a female patient with Behçet’s disease complicated with SCAD [5].

We herein describe a 48-year-old man who was diagnosed with vascular Behçet’s disease after undergoing conservative medical therapy for acute myocardial infarction due to SCAD. This case report highlights that SCAD may occur in association with systemic inflammatory disease including Behçet’s disease, even in male patients.

Case report

A 48-year-old man was admitted to the emergency department because of persistent chest pain. Aside from his current smoking habit, he had no cardiovascular risk factors. Although he had also been experiencing recurrent aphthous stomatitis, nodular erythema, and genital ulcers, he had not been diagnosed with Behçet’s disease at that time. Thus, he was only being treated with topical medication; he was taking no oral medications. He had experienced no physical or emotional stress preceding the chest pain. On arrival, an electrocardiogram showed ST-segment elevation in leads V2 to V6 (Fig. 1). Laboratory testing showed initial negative cardiac troponin T (0.006 ng/mL) and C-reactive protein (0.1 mg/dL) levels and a slightly elevated white blood cell count (10,800 cells/μL). Transthoracic echocardiography revealed impaired contractility in the apical segment of the left ventricle, although the estimated ejection fraction was broadly preserved (59%). The patient was diagnosed with ST-elevation acute myocardial infarction. Emergency coronary angiography (CAG) revealed a non-iatrogenic and non-traumatic dissection in the distal segment of the left anterior descending artery (LAD) with delayed washout of contrast media, even after intracoronary injection of isosorbide dinitrate (Fig. 2A). No significant stenosis was present in the left circumflex artery or right coronary artery. According to the CAG findings, the patient was diagnosed with acute myocardial infarction due to type 2 SCAD based on the currently established angiographic classification [6]. Because the culprit lesion was present in the distal segment of the LAD and the vessel diameter was small, we decided to perform conservative medical therapy including aspirin, nicorandil, enalapril, and atorvastatin. He was managed without a β-blocker because of the tendency for development of sinus bradycardia. The peak levels of creatine phosphokinase and its MB isozyme were 709 and 76 U/L, respectively. Two weeks later, we performed a second CAG examination, which showed natural healing in the distal segment of the LAD (Fig. 2B).

Fig. 1.

Electrocardiogram on arrival revealed ST-segment elevation in leads V2 to V6.

Fig. 2.

Coronary angiography. (A) Emergency coronary angiography revealed spontaneous (non-iatrogenic and non-traumatic) coronary artery dissection in the distal segment of the left anterior descending artery (yellow arrows). (B) Repeated coronary angiography performed 2 weeks later revealed natural healing of the dissected segment.

In contrast to his uneventful cardiac course, the patient complained of painful aphthous stomatitis in addition to the history of nodular erythema and genital ulcers (Fig. 3A–C). These findings led to a differential diagnosis of Behçet’s disease. Additional screening tests for Behçet’s disease were then performed, including investigation of ocular lesions, vascular complications, and serum markers. Although no clear evidence of ocular lesions was found, an ultrasound examination of the lower extremities revealed evidence of bilateral deep vein thrombosis. The patient was finally diagnosed with vascular Behçet’s disease based on the presence of aphthous stomatitis, nodular erythema, genital ulcers, and vascular lesions. Oral administration of colchicine and rivaroxaban was initiated, and his clinical course was uneventful during a 12-month follow-up.

Fig. 3.

Physical examination findings that led to the diagnosis of Behçet’s disease. (A) Aphthous stomatitis. (B and C) Skin lesions.

Discussion

SCAD is an infrequent but important cause of acute coronary syndromes and is caused by non-traumatic and non-iatrogenic separation of the coronary arterial wall [2]. Although the true prevalence of SCAD remains uncertain, it reportedly accounts for 0.1% to 4.0% of all acute coronary syndrome pathologies [2], [3]. Moreover, approximately 90% of patients with SCAD are women without significant cardiovascular risk factors [1], [3], [4].

The management of SCAD is controversial because of the lack of randomized clinical trials that have investigated an optimal treatment strategy. However, data from large-scale observational studies suggest that conservative medical therapy is generally recommended in hemodynamically stable patients with SCAD because of the reportedly favorable short-term outcomes and high rate of spontaneous angiographic healing of the dissected segment [1], [3], [4]. Consistent with these previous study results, our patient was conservatively managed with medical therapy because of his hemodynamic stability and the distal location of the culprit lesion. This conservative therapy resulted in natural healing of the dissection and an acceptable mid-term outcome. Despite favorable short-term clinical outcomes in hemodynamically stable patients with SCAD, long-term adverse cardiovascular events including recurrent myocardial infarction due to recurrent SCAD are relatively common. The reported rate of recurrent SCAD ranges from approximately 10% to 30% of patients [2], [4].

The underlying etiology and predisposing condition of SCAD are considered to be multifactorial. Previous studies have shown that fibromuscular dysplasia is the most common underlying disease in patients with SCAD [1], [4]. Furthermore, connective tissue disorders, vasculitis, and systemic inflammatory diseases can also be the underlying diseases of SCAD [1], [2], [3], [4]. These underlying arteriopathies may weaken the walls of vessels, including the coronary arteries, and may be associated with subsequent spontaneous dissections. In addition to these underlying diseases, other precipitating factors of SCAD include emotional stress, excessive physical exertion, illegal drug use, and hormonal imbalances during pregnancy, postpartum status, and hormonal therapy [1], [2], [3], [4]. All of these conditions may be associated with the occurrence of SCAD, but a paucity of information regarding the relationship between SCAD and Behçet’s disease is currently available.

Behçet’s disease is an autoimmune disorder of unknown origin and is generally diagnosed based on symptoms including aphthous stomatitis, ocular lesions, skin lesions, and genital ulcers [7]. It has a broad spectrum of clinical phenotypes, and vascular Behçet’s disease is a nonspecific vasculitis that involves various vessels of all sizes. The reported incidence of cardiovascular involvement in patients with Behçet’s disease ranges from 2% to 13% and is significantly higher in men than in women [7], [8]. Generally, venous lesions are more common than arterial lesions [8]. The most common type of vascular involvement in patients with Behçet’s disease is deep vein thrombosis in the lower extremities, but some patients have both venous and arterial lesions [8].

Arterial involvement in patients with Behçet’s disease includes thrombosis, stenosis, and aneurysmal changes, and such changes typically affect the aorta, pulmonary arteries, and peripheral arteries in both the upper and lower extremities [8]. Conversely, coronary involvement is uncommon in patients with Behçet’s disease, and acute myocardial infarction is generally caused by coronary artery aneurysm and/or thrombotic occlusion [9]. The present case highlights that SCAD is another type of coronary involvement in patients with Behçet’s disease in addition to the aneurysms and/or thrombotic occlusion [5]. The precise pathogenetic mechanism of arterial involvement in patients with Behçet’s disease remains unknown, but vasculitis due to infiltration of neutrophils into the vasa vasorum, which leads to fibrosis and/or necrosis of the arterial wall, may be a potential explanation [10].

SCAD has been described in patients with various systemic inflammatory and autoimmune diseases [2], [4], [5]. Although the precise mechanism remains uncertain, underlying coronary vasculitis or systemic inflammation may contribute to the occurrence of SCAD via rupture of the vasa vasorum, endothelial dysfunction, and dysfunction of the microvascular coronary circulation in these patients [2]. Behçet’s disease is not included in the list of underlying diseases of SCAD [2], [4], [5]. However, a case reported by Díez-Delhoyo et al. [5] and our case strongly suggest that Behçet’s disease should be included in the list of underlying causes of SCAD because the likelihood of coincidence of these two rare conditions is considered minimal.

Despite the increasing awareness of SCAD due to our enhanced understanding of its epidemiology and recent advances in technology, including intravascular ultrasound and optical coherence tomography, SCAD tends to be misdiagnosed or underdiagnosed [2]. Our case report highlights that SCAD should always be included among the differential diagnoses in patients with acute coronary syndrome, even male patients. Because many questions have not been answered, further investigations are warranted to clarify more precise mechanisms including the underlying conditions and optimal management of SCAD.

Conclusion

This case report suggests that patients who develop SCAD may also have systemic inflammatory diseases including vascular Behçet’s disease.

Conflict of interest

The authors declare that there is no conflict of interest.