Clinical Case Vignette

A 56-year-old male patient with hypertension, hyperlipidemia, tobacco use, and obesity presented with acute anterior wall ST-segment elevation myocardial infarction. Emergent angiography revealed a total occlusion of the proximal left anterior descending (LAD) coronary artery with a large associated thrombus. There was also diffuse ectasia of the circumflex and right coronary arteries and a discrete fusiform aneurysm of the proximal right coronary artery (RCA) (Video 1). Primary percutaneous coronary intervention (PCI) was performed, aided with several runs of mechanical thrombectomy (AngioJet [Boston Scientific; Marlborough, MA]), restoring Thrombolysis in Myocardial Infarction 2 flow. Dual antiplatelet therapy was selected for post-PCI maintenance. Four years later, coronary angiography performed for unstable angina showed progression of the mid-LAD and mild circumflex stenoses and further enlargement of the RCA fusiform aneurysm (Video 2). The patient underwent three-vessel coronary artery bypass grafting surgery (internal mammary to the LAD and vein grafts to the obtuse marginal and posterior descending coronary arteries), and a ligation of the fusiform right coronary fistula. Unfortunately, on subsequent angiography, the mammary graft was found to be occluded (Video 3), and medical treatment was deemed to be the mainstay therapy.

Video 1

Video 2

Video 3

Introduction

The management of patients with coronary ectasia often poses a significant challenge due to the poorly understood mechanisms and variable presentations of coronary ectasia and the paucity of data supporting a specific treatment strategy. Here we provide a concise overview of the definitions, epidemiology, pathophysiology, clinical presentations, and management of aneurysmal coronary artery disease.

Definitions of Aneurysmal Coronary Disease

The terms coronary artery ectasia and coronary artery aneurysm have historically been used interchangeably to describe aneurysmal dilation of coronary arteries.¹ In contemporary literature, arbitrary definitions have been adopted to facilitate a standardized classification of aneurysmal coronary disease: the term coronary artery aneurysm is usually used to describe focal dilation of a coronary segment (≥1.5 times the adjacent normal segment), and the term coronary artery ectasia is used to define more diffuse aneurysmal lesions.² Coronary artery aneurysm is further sub-classified as saccular if the transverse diameter exceeds its longitudinal diameter and as fusiform if the longitudinal diameter exceeds its transverse diameter. Coronary aneurysms exceeding four times the size of the adjacent normal vessel are considered giant (Figure 1).

Figure 1: Illustration of the Variable Morphologies and Clinical Presentation of Aneurysmal Dilatation Involving Coronary Arteries¹

Epidemiology, Pathophysiology, and Presentation

The prevalence of coronary artery aneurysm and coronary artery ectasia in the general population is unknown. However, several studies have reported the rates of both phenotypes among patients who are referred for coronary angiography for various clinical indications. Although coronary artery ectasia is documented in up to 5% of coronary angiograms, true coronary artery aneurysms are less common (<1% of all angiograms).¹ In a prospective registry of 51,555 consecutive coronary angiograms, coronary artery aneurysm was present in 414 patients (0.8%).³

The underlying mechanism of coronary ectasia is not well-understood. In adult patients, most of coronary artery aneurysms and coronary artery ectasias are caused by atherosclerosis or vessel wall injury after a coronary intervention (balloon angioplasty, stenting, or atherectomy).^1,4 In children and young adults, vasculitis (e.g., Kawasaki disease) is a common cause of coronary artery aneurysm.⁵ Less common causes of coronary artery aneurysm include cocaine use and systemic infections.⁶ Genetic susceptibility may also play a role in the causation of coronary artery aneurysm and coronary artery ectasia.⁷

The vast majority of coronary artery aneurysm and coronary artery ectasia is detected incidentally during coronary angiography or computed tomography.¹ However, coronary artery aneurysms are often the culprit for acute or sub-acute clinical presentations (Figure. 1). Local thrombosis in proximal large coronary artery aneurysms can lead to distal embolization and acute myocardial infarction.^1,7 Progressive enlargement of coronary artery aneurysm may result in symptomatic local compression of adjacent structure (pulmonary artery, tricuspid valve, etc.) and can also eventually rupture, producing life-threatening cardiac tamponade or fistulous communications.^8,9 Stress-induced ischemia due to microvascular dysfunction in dilated coronary arteries (dilated coronopathy) has also been documented in patients who have coronary artery ectasia or coronary artery aneurysm without associated obstructive coronary lesions.¹⁰

Management of Aneurysmal Coronary Disease

Treatment of coronary ectasia should be individualized based on the disease phenotype (coronary artery aneurysm vs. coronary artery ectasia), the patient's characteristics, and whether the aneurysm/ectasia is a culprit for the clinical presentation versus an incidental finding. Treatment decisions should also take into account the technical challenges associated with both percutaneous and surgical revascularizations.

Medical Therapy

Because atherosclerosis is implicated in the causation of most coronary artery aneurysms and coronary artery ectasias, aggressive risk factor modification should be the cornerstone of their management.¹ A common dilemma clinicians face routinely is the choice of antiplatelet and/or antithrombotic regimen in patients with incidental non-giant coronary artery aneurysm or diffuse ectasia. The rational for an intensified antithrombotic regimen in patients with coronary artery aneurysm or coronary artery ectasia stems from data suggesting higher rates of ischemic events in these patients. In one study, patients with coronary artery aneurysm had higher 5-year mortality compared with those without coronary artery aneurysm (adjusted hazard ratio 1.56; 95% confidence interval, 1.01-2.41).¹¹ In another study, 54% of patient who were found to have a coronary artery aneurysm on computed tomography suffered a major cardiovascular adverse event at a median of 4-year follow-up.¹² Similarly, in a cohort of 78 patients who developed coronary artery aneurysm after drug-eluting stent implantation, major adverse events were observed in 27% at a median follow up of 3 years.¹³ Doi et al. also found coronary artery ectasia to be independently associated with threefold and fivefold increases in cardiac death (95% confidence interval, 1.37-5.37; p = 0.004) and non-fatal myocardial infarction (95% confidence interval, 2.20-11.0; p < 0.001).¹⁴ The latter study also showed that patients who were treated with oral anticoagulation and were able to achieve a time-in-therapeutic range of >60% had 0% occurrence of major adverse events compared with 33% in those who were not treated with effective anticoagulation. Given the limited supporting evidence, we reserve chronic anticoagulation for selected high-risk patients (e.g., multivessel ectasia or recurrent events despite dual antiplatelet therapy) (Figure 2).

Percutaneous Coronary Intervention

Outcomes data on PCI for incidental coronary artery aneurysm are limited to small case series.^13,15 In the setting of acute coronary syndrome, PCI of an aneurysmal culprit vessel has been consistently associated with lower procedural success, higher incidence of no-reflow and distal embolization, and higher rates of subsequent stent thrombosis, repeat revascularization, and long-term mortality.^1,3,13,16,17 This is partially due to the considerable technical challenges associated with PCI in patients with coronary artery aneurysm or diffuse ectasia. These challenges include the substantial thrombus burden in aneurysmal vessels, the lack of approved purpose-specific devices for coronary artery aneurysm closure in the United States, and the difficulties associated with landing zone sizing and delivery of stiff high-profile devices. Nonetheless, a variety of interventional techniques have been described to overcome these challenges, and the choice of technique should be tailored to the specific anatomy (Figure 2). For example, in patients with a proximal aneurysmal disease of the RCA, coverage of the neck of the aneurysm can be effectively achieved either with a covered stent or double open cell stent implantation.^1,15 On the other hand, patients with a large side branch originating near the aneurysm may benefit from advanced PCI techniques (e.g., stent-assisted coiling) to preserve the side branch (Figure 3).¹⁸

Figure 2: Suggested Algorithm for Management of Patients With Coronary Artery Aneurysm¹

Figure 3: Cine Angiography Illustrating a Moderate-Sized Saccular Aneurysm in the Mid-LAD Treated With a Modified Stent-Assisted Coil Embolization¹

Surgical Interventions

A variety of surgical techniques has been described to treat coronary artery aneurysm, including aneurysm resection, ligation, or marsupialization with interposition graft.^19,20 However, the most common surgical practice is to suture ligate the vessel proximal and distal to the coronary artery aneurysm and implant a bypass grafting distally. Surgical treatment is considered a first-line therapy for coronary artery aneurysm involving the left main and giant aneurysms, although long-term data remain scarce (Figure 2).

Conclusions

There is a clear unmet need for further investigations to understand the pathophysiology, natural history, phenotypic features, and optimal treatment of incidental and symptomatic coronary artery aneurysm and coronary artery ectasia. Recognizing the limitations of our current knowledge of aneurysmal coronary disease, an individualized approach accounting for the patient's anatomical and clinical risk factors is warranted.

References

1. Kawsara A, Núñez Gil IJ, Alqahtani F, Moreland J, Rihal CS, Alkhouli M. Management of Coronary Artery Aneurysms. JACC Cardiovasc Interv 2018;11:1211-23.
2. Luo Y, Tang J, Liu X, et al. Coronary Artery Aneurysm Differs From Coronary Artery Ectasia: Angiographic Characteristics and Cardiovascular Risk Factor Analysis in Patients Referred for Coronary Angiography. Angiology 2017;68:823-30.
3. Núñez-Gil IJ, Terol B, Feltes G, et al. Coronary aneurysms in the acute patient: Incidence, characterization and long-term management results. Cardiovasc Revasc Med 2018;19:589-96.
4. Bavry AA, Chiu JH, Jefferson BK, et al. Development of coronary aneurysm after drug-eluting stent implantation. Ann Intern Med 2007;146:230-2.
5. Friedman KG, Gauvreau K, Hamaoka-Okamoto A, et al. Coronary Artery Aneurysms in Kawasaki Disease: Risk Factors for Progressive Disease and Adverse Cardiac Events in the US Population. J Am Heart Assoc 2016;5:e003289.
6. Baker DW, Whitehead NJ, Barlow M. Mycotic Coronary Aneurysms. Heart Lung Circ 2018;Dec 19:[Epub ahead of print].
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9. Sugiyama K, Suzuki S, Kamiya K, Koizumi N, Ogino H. Ruptured coronary artery aneurysm with pulmonary artery fistulae. J Card Surg 2017;32:799-800.
10. Krüger D, Stierle U, Herrmann G, Simon R, Sheikhzadeh A. Exercise-induced myocardial ischemia in isolated coronary artery ectasias and aneurysms ("dilated coronopathy"). J Am Coll Cardiol 1999;34:1461-70.
11. Baman TS, Cole JH, Devireddy CM, Sperling LS. Risk factors and outcomes in patients with coronary artery aneurysms. Am J Cardiol 2004;93:1549-51.
12. Warisawa T, Naganuma T, Tomizawa N, et al. High prevalence of coronary artery events and non-coronary events in patients with coronary artery aneurysm in the observational group. Int J Cardiol Heart Vasc 2016;10:29-31.
13. Joo HJ, Woong Yu C, Choi R, et al. Clinical outcomes of patients with coronary artery aneurysm after the first generation drug-eluting stent implantation. Catheter Cardiovasc Interv 2018;92:E235-E245.
14. Doi T, Kataoka Y, Noguchi T, et al. Coronary Artery Ectasia Predicts Future Cardiac Events in Patients With Acute Myocardial Infarction. Arterioscler Thromb Vasc Biol 2017;37:2350-5.
15. Briguori C, Sarais C, Sivieri G, Takagi T, Di Mario C, Colombo A. Polytetrafluoroethylene-covered stent and coronary artery aneurysms. Catheter Cardiovasc Interv 2002;55:326-30.
16. Iannopollo G, Ferlini M, Koziński M, et al. Patient Outcomes With STEMI Caused by Aneurysmal Coronary Artery Disease and Treated With Primary PCI. J Am Coll Cardiol 2017;69:3006-7.
17. Ipek G, Gungor B, Karatas MB, et al. Risk factors and outcomes in patients with ectatic infarct-related artery who underwent primary percutaneous coronary intervention after ST elevated myocardial infarction. Catheter Cardiovasc Interv 2016;88:748-53.
18. Win HK, Polsani V, Chang SM, Kleiman NS. Stent-assisted coil embolization of a large fusiform aneurysm of proximal anterior descending artery: novel treatment for coronary aneurysms. Circ Cardiovasc Interv 2012;5:e3-5.
19. Singh SK, Goyal T, Sethi R, et al. Surgical treatment for coronary artery aneurysm: a single-centre experience. Interact Cardiovasc Thorac Surg 2013;17:632-6.
20. Izumi K, Hisata Y, Hazam S. Surgical repair for a coronary-pulmonary artery fistula with a saccular aneurysm of the coronary artery. Ann Thorac Cardiovasc Surg 2009;15:194-7.

Clinical Topics: Acute Coronary Syndromes, Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Invasive Cardiovascular Angiography and Intervention, Noninvasive Imaging, Pericardial Disease, Stable Ischemic Heart Disease, Vascular Medicine, Atherosclerotic Disease (CAD/PAD), Aortic Surgery, Cardiac Surgery and CHD and Pediatrics, Cardiac Surgery and SIHD, Congenital Heart Disease, CHD and Pediatrics and Imaging, CHD and Pediatrics and Interventions, CHD and Pediatrics and Quality Improvement, Interventions and ACS, Interventions and Coronary Artery Disease, Interventions and Imaging, Interventions and Vascular Medicine, Angiography, Nuclear Imaging, Chronic Angina

Keywords: Coronary Angiography, Coronary Artery Disease, Coronary Aneurysm, Mucocutaneous Lymph Node Syndrome, Risk Factors, Dilatation, Drug-Eluting Stents, Dilatation, Pathologic, Acute Coronary Syndrome, Pulmonary Artery, Constriction, Pathologic, Cardiac Tamponade, Tricuspid Valve, Genetic Predisposition to Disease, Myocardial Infarction, Intracranial Aneurysm, Percutaneous Coronary Intervention, Thrombosis, Atherosclerosis, Atherectomy, Registries, Angioplasty, Balloon, Tomography

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