A Rare and Lethal Ostial Left Main Trunk Lesion: A Case Report : Cardiology Discovery

Journal Logo

Case Report

A Rare and Lethal Ostial Left Main Trunk Lesion: A Case Report

Huang, Baotao1; Zhang, Ran2; Li, Chen1,∗

Editor(s): Xu, Tianyu; Fu, Xiaoxia

Author Information
Cardiology Discovery 2(1):p 58-61, March 2022. | DOI: 10.1097/CD9.0000000000000043
  • Open



Acute coronary syndrome may be the initial manifestation of patients with syphilitic aortitis; however, this etiology is likely to be ignored in those with latent syphilis. We present the following case in accordance with the CARE reporting checklist. A legally authorized representative of the patient has given his consent to publish the clinical information and figures in the journal.

Case presentation

A 63-year-old man was admitted to hospital because of chest tightness, shortness of breath, and fatigue for about 10 days. The patient did not complain of headache or intermittent claudication and did not have a weak pulse or loss of pulse in the limbs. The patient had a medical history of hypertension. He had no history of angina pectoris or stroke, prolonged unexplained fever, and/or mucocutaneous changes in childhood. Chest auscultation revealed bibasilar crackles. The patient was nonobese with a BMI of 25.6 kg/m2. No rash, subcutaneous nodules, or gangrene in the limbs was found. Bilateral blood pressure was symmetrical, no vascular murmur was heard, and no neurological abnormalities were detected. Electrocardiogram (ECG) showed ST-segment elevation in the aVR and V1 leads, with widespread ST-segment depression in the anterior and lateral leads and pathological Q wave in leads III and aVF [Figure 1A]. The peak high-sensitivity (hs)-troponin T was 740 ng/L, and the peak N-terminal pro-brain natriuretic peptide was 5303 ng/L. Invasive coronary angiography revealed severe ostial narrowing of the left main trunk, a relatively normal segment of the distal vessel, and an obscure left-to-right collateral circulation; the right coronary orifice was occluded [Figure 1B and 1C]. Contrast-enhanced computed tomography excluded aortic dissection, but revealed a descending aortic ectasia of 5.3 cm and moderate-to-severe stenosis of the ostia of the celiac trunk and superior mesenteric artery [Figure 1D–J]. Echocardiography showed non-dilated left ventricle (LV, end-diastolic dimension 48 mm); worsened LV function (left ventricular ejection fraction (LVEF), 44%); and segmental wall motion abnormality in the interventricular septum and the inferior free wall. Mild aortic regurgitation was detected [Figure 1K]. Both syphilis screening and confirmatory test results were positive, and the toluidine red unheated serum test showed a high titer (1:128). Cardiovascular syphilis was considered. Other tests such as erythrocyte sedimentation rate, C-reactive protein, and arterial biopsy were not performed because of rapid progressive deterioration of clinical conditions. From the guidelines’ point of view, coronary artery bypass grafting (CABG) should have been the first-line revascularization choice for this patient; however, the patient developed sudden onset of acute heart failure and needed emergency revascularization. Considering the relatively high requirement of the patient's pre-surgical health status and longer time for recovery, the patient and his family members refused to undergo CABG and chose percutaneous coronary intervention (PCI) instead. Initially, we planned to insert an appropriate hemodynamic support device as an adjunct to PCI, however, an intra-aortic balloon pump was not used because the abdominal aortic ectasia was a contraindication. Before stenting, the lesion was pre-dilated with a 2.5 mm × 15 mm pre-dilation balloon (Terumo, Tokyo, Japan) and treated with a 3.0 mm × 10 mm Flextome cutting balloon (Boston Scientific, Marlborough, Massachusetts, USA). Then, a 3.5 mm × 12 mm everolimus-eluting platinum-chromium stent (Promus PREMIER, Boston Scientific, Marlborough, Massachusetts, USA) was successfully implanted in the ostium of the left main coronary artery. Finally, post-dilation of stent deployment was performed with a 3.5 mm × 12 mm and 4.0 mm × 12 mm post-dilation balloon (Quantum Maverick balloon, Boston Scientific, Marlborough, Massachusetts, USA) [Figure 1L–N]. The procedure was uneventful, and an intravascular ultrasound (IVUS) imaging test after stent deployment showed excellent stent expansion and good apposition; however, tissue prolapse remained in the proximal segment of the stent [Figure 1O and 1P]. The patient was administered dual antiplatelet therapy with aspirin and ticagrelor after PCI, and his symptoms of heart failure were under control. However, on day 3 after the PCI, the patient died suddenly when cardiac arrest occurred probably because of stent thrombosis; cardiac resuscitation was unsuccessful. The patient's relatives did not consent to an autopsy; thus, pathological analysis was not possible.

Figure 1:
(A) Electrocardiogram showing ST-segment elevation in lead aVR and V1, with widespread ST-segment depression in the anterior and lateral leads and pathological Q wave in leads III and aVF. (B, C) Invasive coronary angiogram showing a severe ostial narrowing of the left main trunk with a relatively smooth distal vessel region, and an occluded right coronary orifice. (D–J) Contrast computed tomography aortogram showing ostial diseases of coronary arteries, an ectasia at the descending aorta, and moderate-to-severe stenosis of the ostium of the celiac trunk and superior mesenteric artery. (K) Echocardiogram showing mild aortic regurgitation. (L–N) Invasive coronary angiogram showing an opened left main coronary artery after PCI and stent implantation. (O, P) Intravascular ultrasound imaging test after stent deployment showing tissue prolapse (yellow star) in the proximal segment of the stent with a minimum lumen area (MLA) of 9.71 mm2 and minimum stent area (MSA) of 13.92 mm2. LM: Left main coronary artery; PCI: Percutaneous coronary intervention; RCA: Right coronary artery.


Severe coronary lesions confined to the ostial segments suggest rheumatologic large-vessel vasculitis or infectious aortitis.[1] About 10% of patients with untreated syphilis develop cardiovascular syphilis after 10 to 30 years of the initial infection. Syphilitic aortitis is characterized by necrotic medial vasorum and wrinkled intima and usually manifests as coronary stenosis, aortic aneurysm, or aortic insufficiency.[1,2] However, if the early symptoms of patients with syphilis are related to the cardiovascular system, especially in those with latent syphilis, the underlying etiology may go unnoticed by physicians.

There are no universally accepted diagnostic criteria for cardiovascular syphilis. The pathological features of cardiovascular syphilis are vasculitis of the vasa vasorum with a multifocal lymphoplasmacytic infiltrate and weakened vessel wall of the aortic root.[3] Imaging tests such as positron emission tomography-computed tomography provide key information to assess the presence and activity of arterial inflammation and suggest the diagnosis of syphilis-associated vasculitis. The differential diagnosis of syphilis-associated vasculitis includes infectious arteritis due to other causes, arteritis associated with rheumatic disease, and isolated arteritis. Herein, we have made a preliminary differential diagnosis based on the clinical and laboratory criteria of the patient. Factually, older age and hypertension were the only risk factors for atherosclerosis in this patient. Coronary artery lesions mainly involved the ostia, while the distal coronary vascular bed was relatively normal, and multiple ostial stenoses were found in the major branches of the aorta. Patients with these characteristics fulfill diagnostic criteria of non-atherosclerotic coronary artery disease. The differential diagnosis should include Takayasu arteritis, giant cell arteritis, Kawasaki disease, Behcet disease, and eosinophilic granulomatosis with polyangiitis, besides syphilitic arteritis. However, because the patient's symptoms rapidly worsened, only selected point-of-care testing could be employed to improve the rescue efficacy. This case is different from others in that previous studies showed that the ascending aorta or aortic arch was the primary location of syphilitic arteritis.[4] However, the present case showed aneurysmal dilatation of the abdominal aorta and severe ostial narrowing of its main branches. Furthermore, we performed IVUS for this patient, which showed the pathologic changes of syphilitic-related coronary artery disease. The IVUS-derived image characteristics of coronary artery vasculitis related to cardiovascular syphilis are as follows: (1) The fibrous plaque is dominant, without a necrotic core; (2) the ostia of coronary arteries were most frequently involved; and (3) the media-adventitia interface of some lesions was not clear. By contrast, atherosclerotic plaques evaluated by IVUS are characterized by diffuse lesions that rarely involve the coronary artery ostia alone and may be presented as fibrous, fibrofatty, and/or calcified plaques with clear media-adventitia boundaries. Unfortunately, in this case, because of the patient's unprotected left main lesion, a detailed IVUS examination was not possible prior to stent implantation. We speculate that necrotic medial vasorum and wrinkled intima of the left main trunk are responsible for tissue prolapse after stent implantation, posing a risk to stent thrombosis.

It is difficult to accurately identify the culprit blood vessels of patients diagnosed with non-ST-segment elevation myocardial infarction (non-STEMI). The left main coronary artery stenosis was considered as a culprit lesion and the intervention was performed based on the following considerations. First, the ECG showed diffuse ST-segment depression, while the ST-segment was elevated in lead aVR, which was consistent with the ECG pattern of left main coronary artery disease. Second, at the time of initial coronary angiography, right coronary ostium was not found, except for a suspicious collateral circulation arising from the distal left anterior descending coronary artery supplying the right coronary artery, which is supportive of the characteristics of chronic coronary occlusive disease. Third, the rapid disease progression and severe left main coronary artery stenosis posed a serious and imminent threat to the safety of the patient, while the right coronary ostium was not clear. Therefore, it was challenging to directly perform PCI for the right coronary artery without retrograde collateral channel guidance, especially considering severe ostial stenosis of the left main trunk. For patients with acute myocardial infarction complicated with chronic total occlusion (CTO) lesions, it is unclear whether complete revascularization improves prognosis. The EXPLORE trial randomized STEMI patients and concurrent CTO to receive either early revascularization or conservative therapy within 1 week of their emergency PCI. There were no significant differences in the incidence of major adverse coronary events at 4 months and at 3.9 years of follow-up between the 2 groups.[5,6] Evidence for complete revascularization in non-STEMI patients with CTO is lacking in prospective randomized controlled trials. Coronary angiography of our patient showed severe stenosis of the left main coronary artery and total occlusion of the right coronary artery. Therefore, coronary artery bypass should be the preferred reperfusion strategy. However, the patient and his family refused to consent to CABG and opted for PCI instead, because the former is more invasive and has a slower recovery than the latter.

We acknowledge that this is not the first report of syphilitic arteritis, but by presenting this case, we hope to remind physicians that in patients with lesions mainly located in the ostia of the coronary arteries, non-atherosclerotic coronary artery disease should be suspected. The plaque characteristics among different etiologies of coronary artery disease should be evaluated, and systematic treatment should be taken into account. From the patients’ point of view, they should seek timely treatment to prevent damage to other vital organs once they become aware of their syphilis diagnosis. Penicillin is not only the main treatment for syphilis but also very important for the prevention of in-stent restenosis in patients with concomitant coronary artery disease.[7] Unfortunately, our patient suffered sudden cardiac death after PCI and had no chance to receive medical treatment for syphilis.

The lack of pathological examination is the main limitation of our case report. The diagnosis of cardiovascular syphilis can be more objective if autopsy results are available. Further, optical coherence tomography (OCT) has the advantages of identifying vulnerable plaques and evaluating the efficacy of interventional therapy. If OCT was performed, we could have obtained more comprehensive information about the coronary lesions.


Severe coronary lesions confined to the ostia suggest infectious aortitis or rheumatologic large-vessel vasculitis rather than atherosclerosis.


This work was supported by Sichuan Science and Technology Program (2019YFS0351).

Author Contributions

Baotao Huang and Ran Zhang drafted the manuscript. Chen Li prepared the figures and revised the manuscript.

Conflicts of Interest



[1]. Gornik HL, Creager MA. Aortitis. Circulation 2008;117(23):3039–3051. doi: 10.1161/CIRCULATIONAHA.107.760686.
[2]. Hook EW 3rd. Syphilis. Lancet 2017;389(10078):1550–1557. doi: 10.1016/S0140-6736(16)32411-4.
[3]. Ladich E, Butany J, Virmani R. Cardiovascular Pathology. 4th ed.Amsterdam, The Netherlands: Elsevier Inc; 2016. 169–211.
[4]. Roberts WC, Barbin CM, Weissenborn MR, et al. Syphilis as a cause of thoracic aortic aneurysm. Am J Cardiol 2015;116(8):1298–1303. doi: 10.1016/j.amjcard.2015.07.030.
[5]. Henriques JP, Hoebers LP, Råmunddal T, et al. Percutaneous intervention for concurrent chronic total occlusions in patients with STEMI: the EXPLORE trial. J Am Coll Cardiol 2016;68(15):1622–1632. doi: 10.1016/j.jacc.2016.07.744.
[6]. Elias J, van Dongen IM, Råmunddal T, et al. Long-term impact of chronic total occlusion recanalisation in patients with ST-elevation myocardial infarction. Heart 2018;104(17):1432–1438. doi: 10.1136/heartjnl-2017-312698.
[7]. Zhang L, Wang Y, Zhang Z, et al. Risk factors of in-stent restenosis among coronary artery disease patients with syphilis undergoing percutaneous coronary intervention: a retrospective study. BMC Cardiovasc Disord 2021;21(1):438. doi: 10.1186/s12872-021-02245-6.

Aortitis; Syphilis; Coronary artery disease; Case report

Copyright © 2022 The Chinese Medical Association, published by Wolters Kluwer Health, Inc.