left main coronary artery thrombos”>Case Report

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American Journal of Emergency Medicine

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American Journal of Emergency Medicine 32 (2014) 197.e3-197.e5

Acute left main coronary Artery thrombosis as the first manifestation of systemic lupus erythematosus and catastrophic Antiphospholipid syndrome

Abstract

Most coronary events in young adults are related to atheroscle- rosis; however, approximately 20% of coronary heart disease in young adults is related to nonatherosclerotic factors such as coronary abnormalities, connective tissue disorders, and autoimmune diseases. Different initial manifestations of systemic lupus erythematosus and antiphospholipid syndrome (APS) have been reported. Myocar- dial infarction is observed in patients with SLE in all age groups; it appears during the course of the disease; and it is unusual in the APS. We present a unique case of a 28-year-old young man previously healthy who has an ST-elevation myocardial infarction by total acute thrombosis of the left main coronary artery. Laboratory studies demonstrated the presence of antibodies for SLE and APS. The patient was treated successfully with percutaneous coronary intervention. He developed catastrophic APS despite an adequate anticoagulation and was treated with intravenous steroids and plasmapheresis. Clinical evolution was satisfactory, and he discharged from the hospital. This case highlights the importance of considering in the emergency department, the prothrombotic states such as SLE and APS in young patients presenting with acute myocardial infarction caused by an unexplained intraCoronary thrombosis. Early diagnosis of catastrophic APS and aggressive therapies are essential to help such patients from succumbing to this potentially fatal condition.

Most coronary events in young adults are related to atheroscle- rosis; however, approximately 20% of coronary heart disease in young adults is related to nonatherosclerotic factors such as coronary abnormalities, connective tissue disorders, and autoimmune diseases [1]. Herein, we present a patient who has an ST-elevation myocardial infarction (MI) caused by acute total thrombosis of the left main coronary artery (LMCA) as the initial manifestation of systemic lupus erythematosus and catastrophic antiphospholipid syndrome (APS).

A previously healthy 28-year-old man with no Cardiovascular risk factors who denied cocaine use was admitted to the emergency department of our hospital with a 24-hour evolution of symptoms of chest pain and progressive shortness of breath. At admission, the clinical examination revealed a heart rate of 90 beats/min, blood pressure of 100/60 mm Hg, respiration of 20 times/min, and signs of acute heart failure. The electrocardiogram was remarkable for its ST- segment elevation and the symmetric T inversion in leads V₂ to V₆.

The cardiac biomarkers (creatine kinase, creatine kinase-MB, troponin I, and N-terminal pro-B type natriuretic peptide) were elevated. Immunological tests demonstrated the presence of anti- bodies for SLE and APS.

The chest x-ray showed acute pulmonary edema. Coronary computed tomographic angiography showed an aneurysm and total occlusion of the LMCA (Fig. 1A). Therapy with 300 mg of aspirin, a

Fig. 1. A, Computed tomographic coronary angiography shows distal enlargement (11 mm) and LMCA thrombosis. B, Left coronary angiography revealed the total occlusion of the LMCA for thrombus. C, Final angiographic result after successful PCI and stenting, with restoration of antegrade flow through the LMCA. D, Burden thrombus aspirated.

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Fig. 2. A, Magnetic resonance imaging (MRI) study shows the ischemic stroke with secondary Hemorrhagic transformation in the occipital lobe. B, MRI study shows Acute ischemic strokes in pons. C, MRI shows infarction in the left kidney. D, MRI shows infarction in the spleen.

loading dose of clopidogrel 600 mg, and intravenous (IV) infusion of unfractionated heparin was started. Coronary angiography showed total occlusion of the LMCA (Fig. 1B).The left coronary system had retrograde filling from the right coronary artery. An intra-aortic balloon pump was installed; percutaneous coronary intervention (PCI) of the LMCA was performed, with predilatation and Thrombus aspiration. (Fig. 1D). A bare-metal stent was successfully implanted in the LMCA (Fig. 1C).

The echocardiogram revealed akinesia of the anterior wall, a reduced ejection fraction of 30%, and undocumented intracardiac thrombi or valvular abnormalities. Heart failure was treated with IV diuretics, nitroglycerin, and dobutamine. The intra-aortic balloon pump was removed at 48 hours for Hemodynamic improvements. The maintenance treatment was aspirin 100 mg/d, clopidogrel 75 mg/d, infusion of unfractionated heparin, diuretics, and angiotensin-con- verting enzyme inhibitors.

On the fifth day of admission, the patient suddenly presented dysarthria and right-sided hemiparesis. A brain magnetic resonance imaging showed 2 ischemic strokes, one with hemorrhagic transfor- mation. (Fig. 2A, B). Acute infarctions on the left kidney and spleen were also demonstrated (Fig. 2C, D).

The suspicion of catastrophic APS (CAPS) was raised, and therapy with IV methylprednisolone for 3 days and 5 sessions of plasmaphe- resis were added to the therapy. The patient had significant clinical improvement. The patient was discharged with prednisone, aspirin, clopidogrel, anticoagulant vitamin K antagonist, angiotensin-converting enzyme inhibitors, diuretics, and spironolactone.

Myocardial infarction is observed in patients with SLE in all age groups; it appears during the course of the disease and it is unusual in the APS [2,3]. We report an unusual case of a previously healthy young individual in which the first manifestation of SLE and APS was an ST- elevation MI caused by total acute thrombosis of the LMCA who, despite antithrombotic therapy, developed CAPS (infarcts in the brain, kidney, and spleen). Our patient had an aneurysm of the LMCA observed on coronary computed tomographic angiography. Coronary artery aneurysms are rare in patients with SLE and are considered a complication of lupus itself or secondary to Steroid therapy [4].

When the SLE is associated with APS, there is a high prevalence of thrombosis, especially venous thrombosis [5]. When arterial throm- bosis happens, it occurs in the brain in 50% of cases and coronary occlusions in 23% [6].

H. Gonzalez-Pacheco et al. / American Journal of Emergency Medicine 32 (2014) 197.e3-197.e5

There is evidence that APS increases the risk of MI, which was the first manifestation in 2.8% and appeared during the evolution of the disease in 5.5% of the patients [3,7]. We believe that acute in situ thrombosis is the mechanism of coronary thrombosis and other territories, ruling out the possibility of embolism in the absence of intracardiac thrombi and valvular involvement. The mechanism of

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Luis M. Amezcua-Guerra MD Department of Immunology National Institute of Cardiology

Mexico City, Mexico

Natalia Aldana-Sepulveda MD

thrombosis in APS is believed to relate to a prothrombotic states [8]. The management of these patients with MI is similar to any other patient, which includes thrombolysis or PCI [9,10]. Catastrophic APS was confirmed by thrombosis and demonstrated by advanced Imaging techniques in 4 arterial territories (acute MI and stroke, as well as spleen and renal infarcts) in the presence of persistent lupus anticoagulant activity. Catastrophic APS is a rare and Severe form of APS, characterized by widespread systemic thrombotic disease, sometimes despite an adequate anticoagulation, and it is associated with a mortality rate of 50% and diagnosed when there is thrombosis in 3 or more organs [7,11]. Early diagnosis and aggressive therapies with anticoagulation plus steroids plus plasmapheresis are the most common therapy and seem to be the main explanation for the significant reduction in mortality rate [11].

This case highlights the importance of considering in the emergency department the prothrombotic states such as SLE and APS in young patients presenting with acute MI caused by an unexplained intracoronary thrombosis. Early diagnosis of CAPS and aggressive therapies are essential to help such patients from succumbing to this potentially fatal condition.

Hector Gonzalez-Pacheco MD

Coronary Care Unit National Institute of Cardiology

Mexico City, Mexico E-mail address: [email protected]

Guering Eid-Lidt MD Yigal Pina-Reyna MD Catheterization Laboratory National Institute of Cardiology

Mexico City, Mexico

Department of Radiology and Imaging National Institute of Cardiology

Mexico City, Mexico

Carlos Martinez-Sanchez MD

Coronary Care Unit National Institute of Cardiology

Mexico City, Mexico

http://dx.doi.org/10.1016/j.ajem.2013.09.032

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