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. 2018 Jan 21;23(5):e12531. doi: 10.1111/anec.12531

Acute coronary syndrome of very unusual etiology

Raimundo Barbosa‐Barros 1, Andrés R Pérez‐Riera 2,✉, Kimmo Koivula 3, Jairo de Carvalho Santos 1, Luiz C de Abreu 2, Kjell Nikus 4
PMCID: PMC6931839  PMID: 29356195

Abstract

Aortitis is one of many possible manifestations of tertiary syphilis. Aortic disease is the most common of all cardiovascular syphilitic lesions. Aortic diseases caused by tertiary syphilis include aortitis, aortic root dilation, aneurysm formation, aortic regurgitation and coronary ostial stenosis. A less common manifestation of syphilitic aortitis is coronary artery ostial narrowing related to aortic wall thickening. We report a case of a 40‐year‐old male patient admitted with a clinical picture of acute coronary syndrome (unstable angina). He had no risk factors for coronary artery disease. The physical examination revealed nothing remarkable. The admission electrocardiogram (ECG) showed ST segment depression in the anterolateral and inferior leads (Figure 1). The coronary angiogram showed critical ostial stenosis of the right (RCA) and left main coronary artery (Figure 2a, b). Cardiac‐computed tomography showed aortic wall thickening with involvement of bilateral coronary ostia (Figure 2b, c). The patient was referred for coronary bypass surgery after treatment with two doses of penicillin G. The laboratory test was strongly positive for syphilitic infection. Postoperative treatment with benzathine penicillin, in doses recommended for tertiary syphilis, was implemented.

Keywords: acute coronary syndrome, coronary artery disease, syphilitic aortitis

1. INTRODUCTION

Syphilis is an infectious disease transmitted mainly through sexual intercourse with several clinical presentations at various stages. Cardiovascular manifestations of tertiary syphilis include aortitis of ascending aortic aneurysm, aortic root dilation, aneurysm formation, aortic regurgitation, and ostial coronary stenosis (Kennedy, Barnard, & Prahlow, 2006; Tewari & Moorthy, 2014). Globally, syphilis remains a major cause of ascending aortic aneurysm (Roberts, Barbin, Weissenborn, Ko, & Henry, 2015). A less common manifestation of syphilitic aortitis is ostial coronary artery narrowing related to aortic wall thickening (Kennedy et al., 2006). Ostial coronary lesions have been detected in as many as 26% of patients with syphilitic aortitis. Among the aortic manifestations, the most common is syphilitic aortitis. In untreated syphilis, aortitis may manifest after 10 to 40 years after the initial sexual contact. The ascending aorta is affected in 50% of the cases, followed, in descending order, by the aortic arch, the descending and abdominal aorta, with possible impairment of coronary ostia and aortic valve (Figures 1 and 2).

Figure 1.

Figure 1

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Electrocardiographic diagnosis: Diffuse ST segment depression in anterolateral leads from V2 to V6, I, aVL and inferior leads II and aVF with concomitant ST segment elevation in aVR. Conclusion: circumferential (global) subendocardial ischemia

Figure 2.

Figure 2

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Coronary angiogram showing critical ostial stenosis of the right (a) and left (c) main coronary artery. CT angiography showing aortic wall thickening with bilateral involvement of coronary ostia (b,d)

After the primary infection, the Treponema pallidum bacteria can be isolated from the aortic wall, where it is initially located in the adventitia and soon thereafter in the lymphatic vessels. The fact that the ascending aorta is rich in lymphatic tissue is one of the main reasons for the tropism of Treponema spirochetes.

Syphilitic aortic aneurysm is a rare occurrence in the antibiotic era, making the diagnostic assumption even more challenging (Paulo, Cascarejo, & Vouga, 2012). The vasa vasorum undergoes a process of endarteritis obliterans, necrosis of the medial layer (mesoarteritis), and infiltration of plasma cells. The disease process in the vasa vasorum of the aortic arch is the cause of “aortic arch syndrome” (Valentin, Karnik, Anzbock, & Slany, 1991). The elastic tissue of the vessel is destroyed and replaced by scar tissue. The inflammatory process may continue for a long time and the diagnosis may be delayed up to 25 years after the initial infection.

The clinical presentation may be of angina when there is obstruction of the coronary ostia, or dyspnea, when there is aortic valve regurgitation. However, the most common clinical symptom is chest pain secondary to rapid expansion of the luetic aneurysm.

2. DISCUSSION

Syphilis is a millenary disease and currently, tertiary syphilis has been virtually eliminated in the developed countries in the Western world (Frank, Mehlman, Tsai, Lomasney, & Joob, 1999). Unfortunately, in Brazil, the disease still remains rather prevalent, especially in some high‐risk populations (Avelleira & Bottino, 2006). There is a direct relationship between the patient's socioeconomic conditions and development of the disease. The basic disease manifestation of cardiovascular syphilis is aortitis. Myocarditis and myocardial syphilitic gums are very unusual. There is, not infrequently, an association of the aortic lesions with coronary artery disease due to the presence of anatomic contiguity (Hu, Liu, Tang, & Peng, 2014). Although our patient complained of chest pain, there was no such association, as confirmed by cardiac catheterization and surgery.

Valvular involvement in the present case is due to enlargement to the aortic annulus followed by dilation of the aorta. There is often a direct involvement of the aortic valve; in this case, surgery was indicated because of severe regurgitation.

ST depression during angina present in ≥6 leads accompanied by inverted T waves, has been associated with autopsy‐proven extensive subendocardial myocardial infarction, left main coronary artery subocclusion, left main equivalent, or severe 3‐vessel disease (circumferential or global subendocardial ischemia) (Nikus et al., 2010). In our case, tight ostial stenosis of both coronary arteries induced global ischemia. We are not aware of any previously published case report of concomitant global ischemia induced by syphilitic bilateral ostial coronary artery stenosis. In a case report by Chadwick et al. (2016), lateral ST depression with T‐wave inversion is mentioned, but there is no detailed description of the ECG pattern. Recommended diagnostic tests in case of a suspicion of arteritis include computed tomography.

Differential diagnostic alternatives include giant cell arteritis, Behçet's disease, temporal arteritis, and Takayasu's arteritis (in cases with involvement of the aorta). Giant cell arteritis involves large‐caliber arteries, such as temporal, carotid and vertebral arteries and the aorta, but it most commonly affects the elderly.

Behçet's disease is an inflammatory disorder of unknown etiology, characterized by recurrent oral aphthous ulcers, genital ulcers, uveitis, and skin lesions. Aortic root dilation, aortic valve regurgitation, intracardiac thrombi, and pulmonary artery aneurysm may precede other manifestations of the disease and occasionally require surgical intervention.

Takayasu's arteritis is most frequent in young females (female/male ratio 3.8 to 1) and is characterized by fever, dizziness, and hypertension. It involves large arteries, mainly through stenotic inflammatory lesions of the aorta and its primary branches and the pulmonary artery.

Postoperative treatment with benzathine penicillin, in doses recommended for tertiary syphilis, is regularly implemented to decrease the probability of relapse. However, it is known that even with the elimination of Treponema Pallidum in the system, the chance of recurrence of the disease still persists. Because of this, there is a need for a long‐term follow‐up of the patient (Tramont, 1976).

The diagnosis of syphilitic aortitis is not currently contemplated because of the rarity of the disease and the widespread use of antibiotics, which are extremely effective when used properly. In this case, the surgeon's experience was crucial for the diagnosis, since syphilitic aortitis was not considered as a diagnostic alternative preoperatively. In Brazil, we still have important sociocultural conflicts, reflected in public health. Therefore, different manifestations of syphilis have to be taken into account in the diagnostic work‐up.

CONFLICTS OF INTEREST

The authors declare that they have no conflicts of interest.

Barbosa‐Barros R, Pérez‐Riera AR, Koivula K, de Carvalho Santos J, de Abreu LC, Nikus K. Acute coronary syndrome of very unusual etiology. Ann Noninvasive Electrocardiol. 2018;23:e12531 10.1111/anec.12531

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