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 Table of Contents  
CASE REPORT
Year : 2016  |  Volume : 4  |  Issue : 4  |  Page : 149-152

Coronary artery ectasia, an enigma in cardiology: A case report with review of literature


Department of Cardiology, North Eastern Indira Gandhi Regional Institute of Health and Medical Sciences, Shillong, Meghalaya, India

Date of Web Publication20-Dec-2016

Correspondence Address:
Animesh Mishra
Department of Cardiology, North Eastern Indira Gandhi Regional Institute of Health and Medical Sciences, Mawdiangdiang, Shillong, Meghalaya
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2321-449x.196286

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  Abstract 

Coronary artery ectasia (CAE) is the diffuse affection of artery which is defined as a dilation 1.5 fold the diameter of the normal adjacent segments of the same artery. Usually, it affects male, and the cause is multifactorial. It is considered as a variant of atherosclerosis. CAE is associated with connective tissue diseases such as Takayasu arteritis and polyarteritis nodosa. It may be primary or secondary to the presence of other diseases. Hence, the presence of CAE supports search to look for the presence of aneurysms involving other major vessels. Involvement of artery in CAE may be focal or diffuse. Prevalence of CAE varies between 0.3% and 5.3% in different studies. Clinically, it may present with features of angina and myocardial infarction. Coronary angiography is the mainstay of diagnosing CAE. Other measures to diagnose are computerized tomography coronary angiography and magnetic resonance angiography. CAE can be managed both medically through anticoagulants, antiplatelet therapy, and invasive interventional procedures such as percutaneous transluminal coronary angioplasty and coronary artery bypass grafting, if indicated.

Keywords: Coronary artery, ectasia, unstable angina


How to cite this article:
Ete T, Kavi G, Mishra A, Jha PK, Malviya A, Megeji RD. Coronary artery ectasia, an enigma in cardiology: A case report with review of literature. Heart India 2016;4:149-52

How to cite this URL:
Ete T, Kavi G, Mishra A, Jha PK, Malviya A, Megeji RD. Coronary artery ectasia, an enigma in cardiology: A case report with review of literature. Heart India [serial online] 2016 [cited 2021 Dec 2];4:149-52. Available from: https://www.heartindia.net/text.asp?2016/4/4/149/196286


  Introduction Top


Coronary artery ectasia (CAE) is usually considered a variant of atherosclerosis with varied clinical manifestations. Here, we are reporting a case who presented to us with features of unstable angina.


  Case Report Top


A Fifty-four-year-old male nondiabetic, nonhypertensive got admitted following complaints of chest pain at rest. Patient had a similar history of rest pain for 6 months. He also had a history of relief of chest pain on taking nitrates intermittently. There was no history suggestive of any loss of consciousness or palpitation. On examination, pulse was 86/min, regular and blood pressure - 136/84 mmHg. Cardiovascular system revealed normal heart sounds and no murmur was present. Other systems revealed no significant abnormality on examination. Electrocardiogram revealed normal sinus rhythm with no ST-T wave changes. Echocardiogram showed structurally normal heart with normal left ventricular function with no evidence of any regional wall motion abnormality. Blood report revealed hemoglobin (Hb) - 13.6 g%, total leukocyte count - 8500/cumm, platelet count - 2 lakhs/cumm, and erythrocyte sedimentation rate - 34 mm/h. Renal function test and liver function test were within normal limit. Markers for hepatitis B surface antigen, anti-hepatitis C virus, and HIV-1 and 2 were normal. Cardiac enzymes such as troponin-I, troponin-T, and creatine kinase-MB were within normal limit even after serial evaluation. Lipid profile showed a triglyceride level of 239 mg/dl, cholesterol - 219 mg/dl, low-density lipoprotein - 173 mg/dl, and high-density lipoprotein - 45 mg/dl. Patient underwent coronary angiography that revealed a normal left main coronary artery, ectatic segment involving the proximal left anterior descending artery with coronary slow flow, normal left circumflex artery, and right coronary artery (RCA) [Figure 1] and [Figure 2]. Patient was treated with subcutaneous low molecular weight heparin and dual antiplatelet (DAPT) therapy along with statin. Patient was followed up regularly with a significant improvement in subjective symptoms. Patient was diagnosed as a case of unstable angina due to coronary ectasia involving proximal left anterior descending artery which on being treated with heparin and DAPT therapy showed a significant improvement.
Figure1: Coronary angiography(right anterior oblique caudal) view showing ectatic segment in proximal part of left anterior descending artery

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Figure2: Coronary angiography(right anterior oblique cranial) view showing (a-d) ectasia in proximal segment of left anterior descending artery

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  Discussion Top


CAE is the dilatation of 1.5 fold the diameter of normal adjacent segments of the same artery.[1] It is usually a diffuse affection of the artery which must be differentiated from aneurysm.[2] Half of CAE is usually due to atherosclerosis, and hence it is considered as a variant of atherosclerotic coronary artery disease. CAE can be either localized or diffuse affecting the entire length of coronary artery. Due to impaired coronary blood flow, it may result in angina, even in myocardial infarction.[3] Coronary ectasia can be found in up to 5% of angiographic series and in autopsy series up to 0.22%–1.4%.[4] It is seen more commonly in males. In year 1812, Bourgon originally described the condition.[5] The term ectasia was given by Björk.[6] The first classification of CAE was done by Markis in 1976 based on vessel involvement: type I – diffuse ectasia of two or more major vessels, Type II – diffuse ectasia in one vessel and localized disease in another, Type III – diffuse ectasia in one vessel only, and Type IV – localized involvement only. Etiology of coronary ectasia is an open field where research is still going; however, several possible causes have been recognized such as chronic exposure to herbicides, connective tissue diseases such as Takayasu arteritis, polyarteritis nodosa, Kawasaki disease, and Ehlers–Danlos, and also including diseases such as Marfan syndrome, rheumatic fever, and syphilis.[7],[8] Prolonged anabolic steroid use has also been considered as a probable cause of CAE as it produces structural damage in blood vessels. Chronic exposure to vasodilator substance such as nitric oxide (NO) is a potential cause of coronary ectasia.[9] Diabetes mellitus and alterations of angiotensin-converting enzyme gene constitute a potent risk factor for coronary ectasia formation.[10] Coronary arterial wall pathology in CAE shows the lack of uniformity. In patients with mixed CAE, similar histological appearance to that of atherosclerosis with marked destruction and reduction of the medial elastic fibers with disruption of the internal and external elastic laminate is seen. Pure form of CAE shows smooth muscle hyalinization of the coronary fibromuscular media leaving an intact intima.[11] The process of hyalinization process has been related to excessive NO production.[12] This finding was supported by reports in the early 1980s that investigated the association between excessive herbicide exposure and CAE due to increase NO production that leads to hyalinization by indirect acetylcholine production. Another pathological explanation for CAE is remodeling. Remodeling is based on the finding that early compensatory enlargement of human atherosclerotic coronary arteries occurs even before noticeable luminal narrowing. Enlargement of artery occurs mainly in the external elastic lamina with positive adaptation of the internal elastic lamina giving rise to a false impression of patent coronary lumens with or without minimal atherosclerosis.[13] This mechanism is the reason for the risk of arterial wall vulnerability with potential progress to acute coronary syndrome. However, negative remodeling of coronary inner elastic lamina is expected to have a more benign and stable prognosis. Negative remodeling appears earlier in the lumen, alarming for a better disease control.[14] Although the concept of remodeling is reasonably sound, it is unable to explain many features that distinguish CAE from atherosclerosis. Conventional inflammatory markers such as cytokines, tumor necrosis factor, and interleukin-6 have been found to be elevated in 50% of CAE patients, particularly in those with evidence for atherosclerosis. Although inflammatory markers have failed to differentiate between atherosclerosis and CAE. Parameters such as total leukocytes count, monocyte count, and C-reactive protein are closely linked to the presence of CAE although not specific.[15] The most commonly involved artery in CAE is the RCA although for unknown reasons.[16] The least affected artery appears to be the left main artery (LMA) as in coronary artery disease, the LMA is usually least affected. Coronary angiography is the main diagnostic technique for the identification of CAE. Intravascular ultrasound is an excellent tool for assessing luminal size and characterizing arterial wall changes. Three dimensional, noncontrast-enhanced, free-breathing coronary magnetic resonance angiography (MRA) facilitates visualization of majority of the proximal and middle segments of the left main, left anterior descending, and RCAs.[17] Coronary artery computed tomography (CACT) is being used in the evaluation of CAE. However, compared with CT, MRA has the advantage of requiring no exposure to ionizing radiation or injection of a contrast agent. Optical coherence tomography has a role to monitor struts apposition once a patient of CAE undergoes percutaneous coronary intervention and also to assess minimal luminal area. The prevalence of CAE in a population who underwent CACT is 8%. For management, heparin as well as fibrinolysis has been successfully used in CAE for re-canalization in isolated cases of acute thrombotic occlusions, occasionally revealing an absence of flow-limiting stenoses.[18] In isolated cases of CAE, the prognosis is better and antiplatelet drugs are usually the mainstay of treatment. Using additional clinical, pathophysiological, and histopathological investigations, it is important to clarify the mechanism underlying CAE for better management of the patient. In patients with coexisting obstructive lesions and symptoms and signs of significant ischemia in spite of medical therapy, percutaneous or surgical coronary vascularization can safely and effectively restore normal myocardial perfusion in patients with coronary ectasia. Excellent short-term and long-term results of angioplasty have been reported in patients lesions adjacent to coronary ectasias.[19] Our patient presented with clinical features of unstable angina who on undergoing angiography had CAE involving the proximal left anterior descending artery. Patient was treated successfully with subcutaneous low molecular heparin and DAPT therapy with a significant improvement in clinical symptoms. To conclude, in contrast to atherosclerostic coronary artery disease, medical management of patients with CAE has not been adequately addressed. Antiplatelet drugs are the mainstay of treatment and prognosis is usually better in isolated cases of CAE. The observed incidence of myocardial infarction even in patients with isolated CAE suggests the generalized administration of antiplatelets in all patients with CAE.[20]

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Mavrogeni SI, Manginas A, Papadakis E, Foussas S, Douskou M, Baras P, et al. Correlation between magnetic resonance angiography (MRA) and quantitative coronary angiography (QCA) in ectatic coronary vessels. J Cardiovasc Magn Reson 2004;6:17-23.  Back to cited text no. 1
    
2.
Lekuona I, Laraudogoitia E, Palomar S, Salcedo A, Zumalde J, Morillas M. Atherosclerotic coronary ectasia or a lymphomucocutaneous syndrome in an adult (Kawasaki's disease)? Rev Esp Cardiol 1999;52:63-6.  Back to cited text no. 2
    
3.
Hartnell GG, Parnell BM, Pridie RB. Coronary artery ectasia. Its prevalence and clinical significance in 4993 patients. Br Heart J 1985;54:392-5.  Back to cited text no. 3
    
4.
Mavrogeni S. Coronary artery ectasia: From diagnosis to treatment. Hellenic J Cardiol 2010;51:158-63.  Back to cited text no. 4
    
5.
Bourgon A in Biblioth Med 1812;37:183, cited in Packard M, Wechsler HF. Aneurysm of the coronary arteries. Arch Intern Med 1929;43:1-14.  Back to cited text no. 5
    
6.
Björk L. Ectasia of the coronary arteries. Radiology 1966;87:33-4.  Back to cited text no. 6
    
7.
Posnik-Urbanska A, Szymanowska Z, Kawecka-Jaszcz K. The probability of Kawasaki diseases in young patients with cardiac disorders – Retrospective studies. Przegl Lek 2003;60:792-6.  Back to cited text no. 7
    
8.
Dieter RS, Murtaugh T, Black J, Russell DC. Coronary arteriomegaly in a patient with Ehlers-Danlos syndrome and multiple aneurysms – A case report. Angiology 2003;54:733-6.  Back to cited text no. 8
    
9.
Sorrell VL, Davis MJ, Bove AA. Current knowledge and significance of coronary artery ectasia: A chronologic review of the literature, recommendations for treatment, possible etiologies, and future considerations. Clin Cardiol 1998;21:157-60.  Back to cited text no. 9
    
10.
Androulakis AE, Andrikopoulos GK, Kartalis AN, Stougiannos PN, Katsaros AA, Syrogiannidis DN, et al. Relation of coronary artery ectasia to diabetes mellitus. Am J Cardiol 2004;93:1165-7.  Back to cited text no. 10
    
11.
Mattern AL, Baker WP, McHale JJ, Lee DE. Congenital coronary aneurysms with angina pectoris and myocardial infarction treated with saphenous vein bypass graft. Am J Cardiol 1972;30:906-9.  Back to cited text no. 11
    
12.
Johanning JM, Franklin DP, Han DC, Carey DJ, Elmore JR. Inhibition of inducible nitric oxide synthase limits nitric oxide production and experimental aneurysm expansion. J Vasc Surg 2001;33:579-86.  Back to cited text no. 12
    
13.
Balin M, Celik A, Kobat MA. The association between soluble lectin-like oxidized low-density lipoprotein receptor-1 levels and patients with isolated coronary artery ectasia. J Thromb Thrombolysis 2012;33:239-45.  Back to cited text no. 13
    
14.
Schoenhagen P, Ziada KM, Kapadia SR, Crowe TD, Nissen SE, Tuzcu EM. Extent and direction of arterial remodeling in stable versus unstable coronary syndromes: An intravascular ultrasound study. Circulation 2000;101:598-603.  Back to cited text no. 14
    
15.
Li JJ, Nie SP, Qian XW, Zeng HS, Zhang CY. Chronic inflammatory status in patients with coronary artery ectasia. Cytokine 2009;46:61-4.  Back to cited text no. 15
    
16.
Befeler B, Aranda MJ, Embi A, Mullin FL, El-Sherif N, Lazzara R. Coronary artery aneurysms: Study of the etiology, clinical course and effect on left ventricular function and prognosis. Am J Med 1977;62:597-607.  Back to cited text no. 16
    
17.
Bluemke DA, Achenbach S, Budoff M, Gerber TC, Gersh B, Hillis LD, et al. Noninvasive coronary artery imaging: Magnetic resonance angiography and multidetector computed tomography angiography: A scientific statement from the American Heart Association committee on cardiovascular imaging and intervention of the council on cardiovascular radiology and intervention, and the councils on clinical cardiology and cardiovascular disease in the young. Circulation 2008;118:586-606.  Back to cited text no. 17
    
18.
Yetkin E, Waltenberger J. Novel insights into an old controversy: Is coronary artery ectasia a variant of coronary atherosclerosis? Clin Res Cardiol 2007;96:331-9.AQ  Back to cited text no. 18
    
19.
Ochiai M, Yamaguchi T, Taguchi J, Ohno M, Yoshimura H, Kashida M, et al. Angioplasty of stenoses adjacent to aneurysmal coronary artery disease. Jpn Heart J 1990;31:749-57.  Back to cited text no. 19
    
20.
Devabhaktuni S, Mercedes A, Diep J, Ahsan C. Coronary artery ectasia - A review of current literature. Curr Cardiol Rev 2016;12:318-23.  Back to cited text no. 20
    


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