|Year : 2022 | Volume
| Issue : 2 | Page : 85-88
Coronary artery ectasia and giant coronary aneurysms presenting with recurrent acute coronary syndrome and heart failure
Sunil Roy Narayanan1, Rashid Khan2, Muhammad Adnan Raufi2
1 Department of Cardiology, Aster Medcity, Kochi, Kerala, India
2 Department of Cardiology, Belhoul Speciality Hospital, Dubai, UAE
|Date of Submission||08-Jul-2022|
|Date of Decision||28-Aug-2022|
|Date of Acceptance||30-Aug-2022|
|Date of Web Publication||12-Oct-2022|
Dr. Sunil Roy Narayanan
Department of Cardiology, Aster Medcity, Kochi, Kerala
Source of Support: None, Conflict of Interest: None
Coronary artery ectasia (CAE) or aneurysm (CAA) is characterized by inappropriate dilatation of the coronary vasculature. While our understanding of CAAs has developed over the last few years, the exact mechanism of Kawasaki disease (KD) and coronary artery complications is unknown. There is a scarcity of data addressing the management and prognosis of patients with CAE. KD is the most common cause of coronary aneurysms in children, whereas atherosclerosis is the most common cause in adulthood, and may present as missed KD. While infection, trauma, vasculitis, KD, cocaine use, and iatrogenic causes form the other etiologies of CAE. Here, we report a young patient who presented with recurrent acute coronary syndromes and heart failure in whom angiography showed severely ectatic coronary arteries with giant CAAs containing a heavy burden of thrombus, diagnosed as probable missed KD, especially with morphological findings from the coronary angiogram.
Keywords: Acute coronary syndrome, coronary artery aneurysm, coronary artery ectasia, heart failure
|How to cite this article:|
Narayanan SR, Khan R, Raufi MA. Coronary artery ectasia and giant coronary aneurysms presenting with recurrent acute coronary syndrome and heart failure. Ann Clin Cardiol 2022;4:85-8
|How to cite this URL:|
Narayanan SR, Khan R, Raufi MA. Coronary artery ectasia and giant coronary aneurysms presenting with recurrent acute coronary syndrome and heart failure. Ann Clin Cardiol [serial online] 2022 [cited 2023 May 29];4:85-8. Available from: http://www.onlineacc.org/text.asp?2022/4/2/85/358388
| Introduction|| |
Coronary artery ectasia (CAE) or aneurism (CAA) is characterized by inappropriate dilatation of the coronary vasculature, and it is diagnosed in 1%–5% of coronary angiographies. The exact mechanism of CAE is unknown and thought to be due to a combination of genetic predisposition, common risk factors for coronary artery disease (CAD), and abnormal vessel wall metabolism. Atherosclerosis is the most common cause, while infection, trauma, vasculitis, Kawasaki disease (KD), cocaine use, and iatrogenic causes form the other etiologies. The flow disturbances associated with CAE can lead to thrombus formation and can cause ischemic complications without stenotic lesions. There is a scarcity of data addressing the management of these patients. Based on the significant flow disturbances within the ectatic segments, chronic anticoagulation is considered primary therapy. The prognosis of patients with CAE is unknown and prospective studies are needed. Here, we report a young patient who presented with recurrent acute coronary syndromes (ACSs) and heart failure in whom angiography showed severely ectatic coronary arteries with giant CAAs containing a heavy burden of thrombus.
| Case Report|| |
A 39-year-old male presented with ACS. He was treated medically with dual antiplatelets, low-molecular-weight heparins, statins, beta-blockers, and ACE inhibitors. He does not have conventional risk factors for CAD. His echocardiography showed inferior wall hypokinesia with mild left ventricular systolic dysfunction. His chest pain subsided with medical treatment. He has undergone a coronary angiography after 6 weeks, which showed chronic total occlusion of proximal right coronary artery (RCA) [Figure 1] and severe ectasia with aneurysmal dilatations of left anterior descending (LAD) [Figure 2] and left circumflex (LCX) coronary arteries [Figure 3]. A stress test was done, which was negative for inducible myocardial ischemia at 13.5 METS. He was advised of medical treatment and started on oral anticoagulation with warfarin along with aspirin and was advised to keep INR in the therapeutic range.
|Figure 1: Coronary angiography 6 weeks following myocardial infarction. Right coronary angiogram showing total occlusion of long segment of the right coronary artery (arrow) from the proximal segment with bridging collaterals in the left anterior oblique view|
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|Figure 2: Left coronary angiogram reveals an aneurysmal dilatation of the proximal left anterior descending artery until the middle segment with distal TMI III flow (A). An aneurysmal dilatation of the obtuse marginal branch is also seen (B)|
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|Figure 3: Left coronary angiogram in the left anterior oblique caudal (Spider) view showing aneurysmal dilatation of the proximal and distal circumflex artery coronary artery (A). Aneurysmal dilatation of the first marginal branch (B) and Ramus intermedius branch (C) is also seen|
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He was lost to follow-up. He presented again after 2 years with heart failure symptoms for 1 week and acute severe pulmonary edema. On questioning, he revealed that he had discontinued medications for the last 1 month. His electrocardiogram showed diffuse ST and T changes and echocardiography showed severe hypokinesia of the RCA and LAD territory with severe left ventricular systolic dysfunction and severe mitral regurgitation. He was started on intravenous diuretics and nitroglycerine along with dual antiplatelet and low-molecular-weight heparin. His cardiac enzymes showed a rise and fall suggestive of acute myocardial infarction. He was medically stabilized and subsequently taken up for coronary angiography. Coronary angiography demonstrated severe ectatic disease involving the LAD and circumflex coronary arteries with chronic total occlusion of RCA. LAD showed an organized thrombus in the aneurysmal segment with almost poor distal flow [[Figure 4] and Video 1] [Additional file 1]. LCX was diffusely ectatic with large aneurysmal dilatations, including the obtuse marginal branches [Figure 5]. However, after the first few injections, there was distal migration of thrombus in LAD with the restoration of TIMI 3 flow in LAD [[Figure 6] and Video 2] [Additional file 2]. RCA showed a chronic total occlusion of the proximal segment as seen earlier. He was medically managed, and his heart failure symptoms improved. His left ventricular systolic function marginally improved, mitral regurgitation reduced to grade 2, and pulmonary edema subsided. He was advised coronary artery bypass graft surgery with ligation of the proximal aneurysm after consultation with the cardiac surgeon. However, the patient refused surgery and decided to continue on medical therapy despite explaining the risks without revascularization. Six months later, he presented to another hospital with severe heart failure and pulmonary edema from which he could not be revived.
|Figure 4: Coronary angiography at the current presentation. Left coronary injection in the LAO view showing huge thrombus burden in the aneurysmal segment of the left anterior descending (A) with almost no antegrade flow poor distal flow. Aneurysmal dilatation of left circumflex coronary artery is also seen (B). LAO: Left anterior oblique, LAD: Left anterior descending|
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|Figure 5: Left coronary angiogram in the RAO caudal view showing aneurysmal dilatation of the proximal and distal circumflex artery coronary artery (B). Aneurysmal dilatation of the left anterior descending coronary artery with no antegrade flow is also seen (A) . RAO: Right anterior oblique|
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|Figure 6: Left coronary angiogram in the RA oblique cranial view showing opening of the LAD with distal migration of thrombus with the force of previous injections and TIMI 3 flow in epicardial vessels. RA: Right anterior, LAD: Left anterior descending|
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| Discussion|| |
CAE is defined as the dilatation of an arterial segment to a diameter of at least 1.5 times that of the adjacent coronary artery. When the dilatation involves the entire vessel, the word “ectasia” is used, and when it is localized, it is called CAA. CAA is termed giant if its diameter exceeds the reference vessel by more than four times or measures more than 8 mm. CAAs are rare and “giant” CAAs are very rare (prevalence: about 0.02%). Pathologists and cardiologists have observed CAE for more than 2 centuries. The largest series from the CASS registry found CAE in 4.9% of more than 20,000 coronary angiograms they reviewed. CAE incidence in an Indian patient cohort with ischemic heart disease has been reported to exceed 10%.
CAE is attributed to atherosclerosis in 50% of cases, congenital in 20%–30% of cases, and association with inflammatory or connective tissue diseases in 10%–20% of cases. All three coronary vessels can be affected by CAE; however, this is rare. Proximal and middle segments of the RCA are the most common sites for CAE, followed by the proximal LAD and LCX. The presence of aneurismal segments produces sluggish or turbulent flow. Patients with CAE can present with ACS, caused by sluggish or turbulent blood flow in the ectasia leading to thrombus formation and distal embolization.
Giant and multiple coronary aneurysms are uncommon in adulthood. Possible etiologies are atherosclerosis, KD, and connective tissue disorders. CAAs develop in 15%–25% of untreated children with KD. CAAs were noted in 7.9% of patients younger than 40 years who underwent coronary angiography for ischemia symptoms, and in 85% of them, a history of KD was probable. On the other hand, CAA frequently remains clinically silent for decades. They carry a lifelong risk for coronary artery thrombosis and myocardial ischemia or infarction.
The specific causative mechanisms of abnormal luminal dilatation in CAE are essentially unknown. The origin of CAE revolves around the vascular endothelium and the biological properties of the arterial wall. The prognosis of CAE is controversial and prospective studies focusing on conservative or invasive strategies to prevent cardiac complications are needed. The indications for myocardial revascularization in CAE are similar to those in other patients with atherosclerotic CAD. High-grade CAE may predispose to thromboembolic-related ACS, formal anticoagulation has been proposed as a potential treatment strategy. However, there is a lack of quality data to support this recommendation. Polytetrafluoroethylene (PTFE)-covered, balloon-expandable stent has been shown to be an effective device for percutaneous management for the exclusion of coronary aneurysms. In the symptomatic patient not suitable for percutaneous coronary intervention, surgical excision or ligation of the CAE combined with bypass grafting of the affected coronary arteries can be the procedure of choice.
Our patient had neither clinical history nor laboratory test results supporting connective tissue disorders or atherosclerosis. Our patient's most probable cause was CAE caused by congenital anomalies or childhood KD, which remain undiagnosed. We believe our patient had KD, rather than CAD, for several reasons: the patient developed the extensive three-vessel disease at a very early age without risk factors for atherosclerosis. The patient had an extensive three-vessel disease. This is typical for KD and unusual for CAE with CAD, which tends to involve one or two vessels. It is possible that many patients previously diagnosed with CAE had KD since awareness of the entity has increased only recently. There are no studies examining the anatomical changes that may occur in CAE over time. From a small angiographic series of patients, the CAE diameter remained stable over a mean follow-up of 36 months.
In conclusion, giant CAA is a sporadic condition, and treatment depends on the clinical findings, location, and size of the CAA. We present this case due to its rarity, interesting clinical presentation, and follow-up showing natural history. While our understanding of CAAs has developed over the last few years, a great deal remains unknown. CAAs are rare, and it can result in fatal outcomes in some patients. Many unanswered questions remain regarding their etiology, prognosis, and therapy. The introduction of genetic studies, new noninvasive imaging modalities, and the systematic testing of antiplatelet, anticoagulation, and vasoactive medications may significantly improve their prognosis.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]