Takayasu Arteritis with Coronary Aneurysms Causing Acute Myocardial Infarction in a Young Man

Abstract

Takayasu arteritis is an inflammatory condition that involves the large cardiac vessels, predominantly the aorta and its main branches. It typically affects young women (age, ≤40 yr), most often Asians and Latin Americans. Herein, we describe a rare manifestation of Takayasu arteritis in a 19-year-old black Tunisian man who presented with acute inferior myocardial infarction and complete atrioventricular block after occlusion from a giant aneurysm in the right coronary artery. The coronary artery disease was associated with aneurysmal dilations in the carotid, vertebral, and right renal arteries. Medical therapy improved Thrombolysis in Myocardial Infarction flow in the area of the giant aneurysm from grade 1 to grade 3. Upon the diagnosis of Takayasu arteritis, intravenous methylprednisolone and oral prednisone therapy was started. After 10 days of hospitalization, the patient was discharged on a medical regimen. Renovascular hypertension due to renal artery stenosis was suspected, so he underwent successful percutaneous transluminal angioplasty of the inferior segmental artery of the right renal artery. During 12 months of close postprocedural monitoring, he experienced lower blood pressure, no chest pain, and no cardiovascular complications.

This association of conditions has not been previously reported. Besides presenting this very rare combination of findings, we discuss the differential diagnosis of Takayasu arteritis in our patient.

Primary systemic vasculitis is a group of autoimmune syndromes characterized by stenosis, occlusion, or aneurysmal dilation of blood vessels consequent to intramural inflammation. Takayasu arteritis (TA) is an inflammatory condition that involves the large cardiac vessels, chiefly the aorta and its main branches. It typically affects young women (age, ≤40 yr), and it occurs quite frequently in Asians and Latin Americans.¹

The inflammatory process associated with TA involves the coronary arteries in less than 10% of patients, mostly in the form of stenotic lesions.² Coronary aneurysms that result in an acute myocardial infarction (MI) as a presentation of the process are extremely rare.³ Herein, we report the case of a young man who had TA with a primarily aneurysmal manifestation in the carotid, vertebral, right renal, and right coronary arteries that resulted in an acute MI.

Case Report

In July 2009, a 19-year-old black Tunisian man was admitted to the emergency department for persistent median chest pain of 30 minutes' duration. The pain occurred during rest, radiated to the left arm, and was accompanied by profuse sweating. The patient's medical history included arterial hypertension. He did not drink alcohol or smoke, did not have diabetes mellitus, had no history of cerebrovascular accidents or peripheral vascular disease, and had no family history of coronary artery disease (CAD). His blood pressure was 140/80 mmHg. The pulse rate was 50 beats/min and was symmetrical over the carotid and upper- and lower-extremity arteries. There was no cervical, supraclavicular, axillary, or femoral adenopathy. Auscultation revealed normal breath sounds bilaterally and bradycardia without murmurs. The abdomen was normal. An electrocardiogram showed ST-segment elevation in the inferior leads and complete atrioventricular block (Fig. 1).

Fig. 1 Electrocardiogram shows ST-segment elevation in the inferior leads and complete atrioventricular block.

The patient was admitted to intensive care and was started on aspirin, clopidogrel, and unfractionated heparin. Coronary angiography revealed wall irregularities and aneurysmal coronary arteries without significant narrowing (Figs. 2 and 3). A saccular aneurysm in the right coronary artery resulted in Thrombolysis in Myocardial Infarction (TIMI) grade 1 flow (Fig. 3).

Fig. 2 Right coronary angiograms (right anterior oblique projection) show a giant aneurysm. Thrombolysis in Myocardial Infarction (TIMI) flow was grade 1 on day 1 (A), and grade 3 on day 3 (B).

Fig. 3 Left coronary angiogram (left anterior oblique projection) on day 1 shows aneurysmal dilation of the coronary arteries, with wall irregularities in the left circumflex and left anterior descending coronary arteries.

Pharmacologic therapy also included captopril and amlodipine. Because coronary vasculitis with intracoronary thrombi was suspected, intravenous abciximab was initiated. This improved the patient's general condition and resolved the electrocardiographic ST-segment changes and atrioventricular block within 2 hours.

A 2-dimensional echocardiogram showed normal left ventricular function with an ejection fraction of 0.56 and no regional wall-motion abnormalities. Mild aortic and mitral regurgitation was observed, but the aortic root was not dilated. Chest radiography revealed a normal cardiac silhouette and no infiltrates. Laboratory test results were total leukocytes, 7.5 ×10⁹/L; hemoglobin level, 13.2 g/dL, platelet count, 180 ×10⁹/L; erythrocyte sedimentation rate, 55 mm/hr; C-reactive protein, 40 mg/L; total plasma creatine kinase, 1,524 U/L; myoglobin concentration, >1,000 μg/L; and troponin I concentration, 9.33 μg/L.

Test results of renal and liver function were normal, and those for other inflammatory markers, rheumatoid factor, venereal disease, cryoglobulin, complement activity, antiphospholipid antibodies, antinuclear antibodies, extractable nuclear antigens, and antineutrophil cytoplasmic antibodies were negative. Results for hepatitis B and C and human immunodeficiency virus were negative. Function of the coagulation inhibitor system (including antithrombin III, protein C, and protein S) was also normal. All blood and urine samples were negative.

Coronary angiographic follow-up on day 3 showed that flow through the right coronary artery had improved to TIMI grade 3 (Fig. 2B). An aortogram showed normal arch vessels and no lesions in the thoracic or abdominal aorta or in its main branches, except for a stenosis above an aneurysmal dilation of the inferior segmental artery of the right renal artery (Fig. 4).

Fig. 4 Follow-up angiogram (right anterior oblique projection) on day 3 shows stenosis above an aneurysmal dilation of the inferior segmental artery of the right renal artery.

Cerebral angiography, performed to detect associated brain vasculitides, only showed moderate dilation of the carotid arteries and an enlarged left vertebral artery. Narrowing of the right internal carotid artery was also seen. Takayasu arteritis was suspected, so pulse therapy with intravenous methylprednisolone (1 g/d for 3 d) followed by orally administered prednisone (1 mg/kg/d) was added to the medical regimen.

Because the patient was asymptomatic and hemodynamically stable, he was discharged after 10 days of hospitalization with instructions to take aspirin, captopril, clopidogrel, amlodipine, acebutolol, and steroids. Upon analysis of his case, renovascular hypertension due to the renal artery stenosis was suspected. Therefore, 3 months later, the patient underwent percutaneous transluminal angioplasty of the inferior segmental artery of the right renal artery. The procedure was performed with use of a Monorail® catheter and deployment of a 3.5 × 12-mm TAXUS® Liberté® stent (Boston Scientific Corporation; Natick, Mass).

During the 12-month follow-up period, the patient had no chest pain or cardiovascular complications. His blood pressure decreased, which reduced the need for antihypertensive medication.

Discussion

In our patient, primary systemic large-vessel vasculitis (a chiefly aneurysmal condition of the carotid, vertebral, right renal, and right coronary arteries) resulted in an acute MI. Although the patient's systemic hypertension, young age, and angiographically confirmed arterial lesions matched some clinical features of TA, the diagnostic criteria for the disease were not fully met. The criteria of the American College of Rheumatology include age, symptoms, signs, and angiographic findings related to the involvement of the aorta and its major branches.⁴ The presence of more than 3 criteria suggests a high probability of TA. Our patient had only 2 of 6 criteria.

In a report by Sharma and colleagues,² a patient presented with 4 required minor criteria for the diagnosis of TA: high erythrocyte sedimentation rate, aortic regurgitation, a documented lesion in a coronary artery, and hypertension. In our patient's case, the main differential diagnosis was Kawasaki disease, which is distinguished from TA by the involvement of medium-sized vessels, mucocutaneous lymph node syndrome, and a very distinct age distribution in young children (usually less than 5 years old).⁵ The caliber and size of the predominant vessels involved strongly influence the clinical features of the different forms of vasculitis and are therefore a major criterion for classification.

Other differential diagnoses, such as Behçet disease, giant-cell arteritis, polyarteritis nodosa, and syphilitic arteritis, were less likely and were not supported by our patient's medical history, physical findings, or laboratory data.

Takayasu arteritis occurs predominantly in young Asian women. It has been reported worldwide, with the highest prevalence in Asia.⁴ The vascular territories affected by TA vary markedly by geographical location: in Japan and Europe, the thoracic aorta and its branches are typically involved, but in India, the abdominal aorta is reportedly affected more often.^6,7

Coronary arterial involvement is observed in approximately 10% of TA cases and can be divided into 3 distinct morphologic types: stenosis or occlusion of the coronary ostia, diffuse or focal coronary arteritis, and coronary aneurysm formation.^8,9 Most of the lesions cause luminal narrowing that can lead to occlusion; however, coronary aneurysms are extremely rare. They can develop as vascular walls weaken because of arterial hypertension and the extensive destruction of elastic fibers in the media. Coronary aneurysms often cause stasis of blood flow and result in mural thrombus and MI. In rare instances, symptomatic CAD may be the initial sign of TA.¹⁰ There is no consensus on how coronary lesions related to TA should be treated. Steroids alone may improve stenotic lesions that involve the carotid arteries.¹¹ However, it is unclear whether this also applies to coronary lesions. The effect of steroids on aneurysmal lesions is also unknown.

Like Al-Hulaimi and colleagues,¹² Araszkiewicz and associates¹³ reported an atypical presentation of TA in a young woman who presented with acute MI and had mixed, aneurysmal, and obstructive CAD that involved the left anterior descending coronary artery. Acute management consisted of thrombolytic therapy in one patient¹² and intravenous abciximab in the other.¹³ Long-term management of the CAD was surgical in the 1st report¹² and medical in the 2nd.¹³ Suzuki and co-authors¹⁴ described the long-term survival of a patient who had a giant aneurysm of the left main coronary artery due to TA but no major cardiac complications.

Cerebrovascular symptoms associated with TA are not common and chiefly involve the carotid arteries; involvement of the vertebral arteries is rare.¹⁵ The cerebrovascular complications of TA are primarily related to the presence of occlusive lesions. Our patient's carotid and left vertebral arteries were enlarged without associated cerebrovascular symptoms.

We did not perform coronary angioplasty initially. Instead, we started the patient on therapy with abciximab, in addition to clopidogrel, aspirin, and heparin. The subsequent percutaneous transluminal angioplasty was successful. Close follow-up and repeated angiographic evaluations were planned in order to monitor the possible progression of aneurysms. We believe that this is the 1st report of a very rare combination of findings in a patient with TA.