Abstract
Coronary artery ectasia (CAE) is defined as a localised or diffuse dilatation of coronary artery lumen more than 1.5 times that of an adjacent normal segment. CAE may present with or without functionally significant stenosis. Such patients may manifest with stable angina or with acute coronary syndrome. Ectasia may serve as a nidus for thrombus formation with likelihood of distal embolisation or it may lead to dissection or spasm. This condition presents a challenge for an interventionist. Should we intervene or manage it medically. We describe a case of CAE where all the vessels were diffusely ectatic with variable degree of stenosis. In brief we discuss the causes and management issues in ectasia.
Keywords: cardiovascular medicine, interventional cardiology, ischaemic heart disease
Background
Coronary artery ectasia (CAE) was first described by Morgagni,1 and remains a challenge to clinicians regarding its aetiology, clinical sequelae and management. The incidence of CAE is reported to be between 1.4% and 5.3%.2 In adults, 50% of the cases are attributed to atherosclerosis.3 While in children or young adults it is due to Kawasaki disease.4 Other causes that are described include connective tissue diseases such as Ehler-Danlos syndrome, polyarteritis nodosa and scleroderma. The incidence of CAE in Indian studies has been reported to exceed 10%.5 CAE is not innocuous and around 50% may present with myocardial infarction or angina.
Case presentation
A 40-year-old male patient presented to our emergency department with typical anginal pain from 1 hour. The only risk factor was smoking. Examination revealed a pulse rate of 68 bpm and blood pressure of 100/60 mm Hg. Systemic examination was unremarkable except for left ventricular fourth heart sound.
Investigations
A standard ECG revealed a normal sinus rhythm with ST segment elevation in inferior leads with ST depression in leads V1–V4 (figure 1). Transthoracic echocardiogram showed hypokinesia in the basal and mid-inferior and inferoseptal wall. The patient received thrombolytic therapy. An angiogram performed subsequently revealed diffuse CAE. Left anterior descending coronary artery (LAD) had ectasia in proximal to distal segment with mild stenosis in proximal segment and slow flow in distal segment (figure 2, Video 1). Left circumflex coronary artery (LCX) and major obtuse marginal had ectasia from the ostia (figure 3, Video 2). Right coronary artery (RCA) was diffusely ectatic with severe stenosis in the proximal segment with evidence of thrombus, mild stenosis in mid-segment and severe stenosis at the origin of posterior descending artery and proximal posterolateral artery (posterior left ventricular artery) (figure 4, Videos 3 and 4).
Figure 1.
Twelve-lead ECG showing ST segment elevation in II, III, avF and ST segment depression in V1–V4.
Figure 2.
Right anterior oblique (RAO) cranial view showing ectasia in proximal to distal segment of left anterior descending coronary artery (LAD) with mild stenosis in proximal segment and slow flow in distal segment.
Video 1.
Right anterior oblique (RAO) cranial view showing ectasia in proximal to distal segment of left anterior descending coronary artery (LAD) with mild stenosis in proximal segment and slow flow in distal segment.
Figure 3.
Right anterior oblique (RAO) caudal view of the left system showing diffuse ectasia of left circumflex coronary artery (LCX). LAD, left anterior descending coronary artery.
Video 2.
Right anterior oblique (RAO) caudal view of the left system showing diffuse ectasia of left circumflex coronary artery (LCX).
Figure 4.
Left anterior oblique (LAO) cranial view of the right coronary artery (RCA) showing diffuse ectasia with severe stenosis in the proximal segment with evidence of thrombus, mild stenosis in mid-segment and severe stenosis at the origin of posterior descending artery (PDA) and proximal posterolateral artery (PLV, posterior left ventricular artery).
Video 3.
Left anterior oblique (LAO) cranial view of the right coronary artery showing diffuse ectasia with severe stenosis in the proximal segment with evidence of thrombus, mild stenosis in mid-segment and severe stenosis in the origin of posterior descending artery (PDA) and proximal posterolateral artery (PLV, posterior left ventricular artery).
Video 4.
Left anterior oblique (LAO) view showing thrombus in proximal right coronary artery (RCA) and severe stenosis with diffuse ectasia.
Treatment
After thrombolysis our patient was asymptomatic with an uneventful course during hospitalisation. Modified Bruce protocol was negative for inducible ischaemia with 10 metabolic equivalents. Standard protocol for acute coronary syndrome including dual antiplatelets and statins was initiated along with anticoagulation.
Outcome and follow-up
We decided on optimal medical management with anticoagulation and the patient is doing well on follow-up. Dual antiplatelets with anticoagulation were given for a period of 1 month following which clopidogrel was withdrawn. The patient was continued on ecosprin 75 mg and warfarin optimised to international normalised ratio (INR) with statins.
Discussion
Coronary ectasia is defined as localised or diffuse dilatation of a coronary artery that exceeds 1.5 times the adjacent normal arterial segment.6 Topographically, RCA is the most common vessel affected by ectasia followed by LAD and then LCX.7 Ectasia is usually diffuse in RCA while a discrete form of ectasia is seen in LAD. Atherosclerosis accounts for 50% of the cases of CAE. Based on the extent of ectasia, a classification was proposed by Markis et al. In decreasing order of severity, diffuse ectasia of two or three vessels was classified as type I; diffuse disease in one vessel and localised disease in another vessel as type II; diffuse ectasia of one vessel only as type III; and localised segmental ectasia as type IV.4
Our patient had type I topographical ectasia, in which there is diffuse ectasia in two or three vessels (all in our case). We can see variable vascular response to atherosclerosis simultaneously in our case, namely stenosis and ectasia. The possible mechanism leading to CAE is chronic inflammation along with degradation of extracellular matrix by matrix metalloproteinases and other catalytic enzymes leading to weakening of the tunica media.8 Investigation of choice is coronary angiography. Ideally, treatment of choice is coronary artery bypass grafting; however, few case reports exist on stenting of these ectatic vessels.9 Our patient was discharged on antiplatelets (clopidogrel and aspirin 75 mg each), atorvastatin 80 mg and warfarin titrated to INR of 2–3. Despite lack of randomised studies to approve anticoagulation, it is routinely used in these cases.9 Ours is an unusual case where stenosis and ectasia are seen simultaneously as a variable response to atherosclerosis.
Learning points.
Coronary artery ectasia (CAE) is seen in 1.4%–5.3% of patients undergoing coronary angiography.
CAE is usually a variable response to atherosclerosis which accounts for 50% of the cases.
The aetiology, prognosis and treatment are yet to be defined.
Anticoagulation is routinely used despite lack of randomised studies.
Coronary artery bypass grafting is a preferred mode of treatment.
Footnotes
Contributors: SKK and SB drafted the manuscipt and did literature search. AM performed the coronary angiogram. PSS edited and finalised the manuscript.
Funding: The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests: None declared.
Patient consent: Obtained.
Provenance and peer review: Not commissioned; externally peer reviewed.
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