Chronic total occlusions of the right coronary and left anterior descending coronary arteries in a young adult patient with antiphospholipid syndrome

Abstract

A 36-year-old male appeared to have an old myocardial infarction on electrocardiogram, and coronary angiography (CAG) was performed. The CAG showed total occlusions of the right coronary artery and left anterior descending artery. He was successfully treated with drug-eluting stent implantation for both occluded coronary arteries. Such serious coronary lesions are uncommon for his young age. The patient was diagnosed as having antiphospholipid syndrome (APS) based on elevation of anticardiolipin antibody and anti-β2 glycoprotein I antibody. Two years after stent implantation, the patient was well without ischemia or thrombosis. APS should be considered a potential cause of serious coronary disease in young adults.

<Learning objective: Antiphospholipid syndrome (APS) should be considered a potential cause of serious coronary disease in young adults. Although there is a high risk of acute stent thrombosis and restenosis after multiple stents implantation, percutaneous coronary intervention with drug-eluting stent implantation could be an appropriate therapy for chronic total occlusion in APS patients.>

Introduction

Acute myocardial infarction (AMI) is relatively uncommon in patients under 40 years of age [1]. Some clinicopathological conditions such as nephritic syndrome, antiphospholipid syndrome (APS), coronary artery spasm, coronary embolization, accelerated atherosclerosis, spontaneous coronary artery dissection, or Kawasaki disease may be involved [1], [2]. APS is an autoimmune disorder associated with vascular thrombosis and/or fetal loss [3]. This syndrome can cause AMI by thrombus, as well as by accelerated atherosclerosis [4], [5]. The treatment of these patients is a clinical challenge because of the high rate of restenosis after percutaneous coronary intervention (PCI) or coronary bypass failure [6].

The case of a patient with APS who showed severe chronic total occlusion (CTO) legions on angiography and was successfully treated with drug-eluting stent (DES) implantation is reported.

Case report

A 36-year-old male had experienced occasional chest pain with cold sweats about 18 months prior to admission to our hospital. He consulted the respiratory department of our hospital with persistent exertional dyspnea, but he was referred to our department for a suspected old myocardial infarction on electrocardiogram (ECG). Therefore, he was admitted to our hospital for coronary angiography (CAG). He had no history of any disease including coronary artery disease (CAD) and thrombosis, but he had some risk factors of CAD such as smoking (1 pack per day smoking history over 16 years), dyslipidemia [total cholesterol of 227 mg/dl, low-density lipoprotein (LDL)-cholesterol of 136 mg/dl, and triglycerides of 358 mg/dl], and obesity. He had no family history of familial hypercholesterolemia, CAD, or thrombosis. The levels of cardiac enzymes and troponin T were within normal limits. The ECG showed Q waves in leads II, III, and aVF, and the QS pattern in leads V1 and V2. The echocardiogram showed a reduced left ventricular ejection fraction of 44%, and akinesis of the apex and inferior wall, with hypokinesis of the antero-septal wall, suggesting old myocardial infarction in the territory of the right coronary artery (RCA) and left anterior descending artery (LAD). He was treated with aspirin 200 mg daily and clopidogrel 75 mg daily on admission. He underwent CAG on the fourth hospital day. The CAG showed a double barrel-like appearance of the RCA from the ostia to the distal portion and a subsequent occlusive lesion with retrograde collateral filling from the left circumflex artery (Fig. 1A), and total occlusion at the proximal LAD with retrograde collateral filling from the RCA (Fig. 1B). At that time, PCI was performed at the RCA. Intravascular ultrasound (IVUS) imaging showed a continuous helical dissection-like lumen from the posterior descending artery (PDA) with relative hard plaque and partial calcification, but there was no thrombus (Fig. 1C–E). Three overlapping DESs were successfully implanted from the PDA to the ostial lesion of the RCA. The final angiogram showed a good result and good collateral flow from the PDA to the LAD. The patient had some coronary risk factors, but his coronary artery lesions were severe for his young age. Therefore, other risk factors for CAD, especially in a young adult, were examined. Immunological tests showed that antinuclear antigen was positive, the anti-dsDNA antibody level was 2.9 IU/ml, the anticardiolipin (aCL) antibody level was 52 U/ml (normal <10 U/ml), the anti-β2 glycoprotein I (GPI) antibody level was 11.2 U/ml (normal <3.5 U/ml), and lupus anticoagulant was negative. At that time, APS was suspected, but he was not diagnosed as having systemic lupus erythematosus (SLE), because he did not fulfill the criteria for SLE at our rheumatology clinic. He was then started on long-term warfarin therapy. One week after the procedure, another PCI was performed at the total occlusive lesion of the LAD. The IVUS imaging showed a hard plaque and partial calcification near the entry point of the occlusive lesion and eccentric soft plaque at the distal portion of the LAD. Three overlapping DESs were successfully implanted from the distal to the proximal portions of the LAD. The final CAG showed a good result. He was treated with warfarin, aspirin, clopidogrel, atrovastatin, and amlodipine during follow-up. The follow-up CAG done 3 months later showed no restenosis of the implanted stents. At that time, the aCL antibody and anti-β2 GPI antibody levels were 33 U/ml and 32.8 U/ml, respectively. APS is defined by clinical events and laboratory confirmation of antiphospholipid antibody on 2 or more occasions at least 12 weeks apart [4]. Therefore, this patient was diagnosed as having APS. The CAG follow-ups done six months and two years later did not show significant stenotic legions (Fig. 2A and B), and the patient has remained well without ischemia or thrombosis. Therefore, because of the high risk of major bleeding associated with triple therapy, clopidogrel was discontinued after the final CAG.

Fig. 1.

Coronary angiogram and intravascular ultrasound images of right coronary artery (RCA) at the first percutaneous coronary intervention. (A) Coronary angiograms showing a double barrel-like appearance of the right RCA from the ostia to the distal portion, and (B) total occlusion at the proximal left anterior descending artery. The white arrow indicates the entry site of chronic total occlusion. (D,E,F) Intravascular ultrasound images from the mid portion of the RCA before stent implantation showing a continuous helical dissection-like lumen with relative hard plaque and partial calcification.

Fig. 2.

Coronary angiogram 2 years after percutaneous coronary intervention. Two-year follow-up coronary angiogram showing no significant restenosis in (A) the right coronary artery and (B) left anterior descending artery.

Discussion

A case of APS in a young adult patient with two severe vessel-occlusive coronary artery lesions was described. To the best of our knowledge, this is the first case showing two CTOs of major coronary arteries on CAG in a patient with APS.

The cause of severe CAD in the present case was not apparent. A young man with AMI and APS but no major coronary risk factors showed no significant coronary plaque on IVUS imaging after thrombus aspiration in the infarct-related artery [7], while a young woman with SLE and APS had diffuse coronary atherosclerosis progression for her age in the non-occluded coronary arteries on IVUS imaging [8]. APS is classified as primary APS without an underlying systemic autoimmune disease and secondary APS with the presence of another systemic autoimmune disease. APS-induced arterial events are the most pronounced in SLE-associated secondary APS, where traditional and non-traditional risk factors are multiplied, and atherosclerosis occurs more prematurely [5]. The present patient may have had secondary APS, because of his positive antinuclear antigen and elevation of anti-dsDNA antibody. In the present case, the IVUS imaging mainly showed progressive atherosclerotic lesions with focal calcification at both occlusive coronary artery lesions. It has been reported that aCL antibodies enhanced platelet aggregation and can potentially cross-react with antibodies against oxidized LDL, which is associated with accelerated atherosclerosis progression, and promote local intimal smooth muscle cell proliferation by enhancing the release of cytokines and growth factors [9]. Therefore, in the present patient with APS, some coronary risk factors, especially hypercholesterolemia, likely facilitated formation of atherosclerotic lesions. Based on the atherosclerotic lesions, the proximal LAD and RCA occlusions with subsequent CTO from thrombus formation after plaque rupture and/or hypercoagulable state might have occurred.

In the present case, the CAG revealed a double barrel appearance from the PDA to the ostial lesion of RCA and IVUS imaging also showed continuous helical dissection like lumen. This appearance was speculated as a result of spontaneous recanalization after thrombotic events due to APS or spontaneous coronary dissection.

Thrombectomy [7], stent implantation [4], or coronary bypass surgery [9] for the treatment of AMI in patients with APS has been reported. However, there are no reports of the treatment of CTO lesions in patients with APS. A CTO lesion is ordinarily treated by PCI with DES implantation or bypass surgery. PCI was chosen in the present case. The risk of acute stent thrombosis or the high rate of restenosis after stent implantation in AMI patients with APS has been reported [4], [6], [9], [10]. However, in the present patient, there have been no thrombosis and severe restenosis owing to the second generation DES, intense anticoagulation, and prevention of atherosclerosis therapy including lifestyle modification in at least two years after PCI, even though multiple stents were implanted. Therefore, PCI with DES implantation could be an appropriate therapy for the CTO lesions of APS patients. Careful follow-up is required in this case for possible thrombotic events and progression of atherosclerosis in the future.

Conflict of interest

None declared.