ABSTRACT: We present a case of a patient with an acute coronary syndrome who had the exceptionally rare finding of multivessel giant coronary artery aneurysms (>8 mm) involving the left main and ostial right coronary arteries with concomitant atherosclerotic disease. A work-up to diagnose medical conditions that would predispose the patient to giant coronary aneurysms was negative; however, a detailed history recalled a childhood hospitalization with a constellation of signs and symptoms consistent with Kawasaki disease. This demonstrates an unusual anatomy underlying a common cardiac presentation in adults that has implications for the management of the associated atherosclerotic coronary artery disease.
J INVASIVE CARDIOL: 2014;26(9):E127-E129
KEY WORDS: giant coronary artery aneurysms, Kawasaki disease
Coronary artery aneurysms (CAAs) are identified by angiography in 0.3%-5.3% of the general population.1 CAAs are considered “giant” when they have a diameter >8 mm or exceed the diameter of the reference vessel by 4-fold.2 Giant CAAs are extremely rare, with an incidence of ~0.02%, and usually involve a single coronary vessel, typically the right coronary artery.3 These giant aneurysms may be congenital or acquired in association with atherosclerosis, inflammatory vasculitis, and connective tissue disease or following trauma or exposure to drugs such as cocaine.1,4 The majority of patients remain asymptomatic and the giant CAA is detected incidentally; however, some patients may present with an acute coronary syndrome, congestive heart failure, or sudden death.4 Here, we describe a case of a patient who was admitted to our institution with acute coronary syndrome and found to have multivessel giant CAAs and a clinical history consistent with antecedent Kawasaki disease.
Case Report. A 54-year-old man with history of hypertension, end-stage renal disease requiring hemodialysis, left ventricular dysfunction (left ventricular ejection fraction [EF] ~30% with global hypokinesis), and recent admission for congestive heart failure was admitted to an outside hospital for new onset of substernal chest pain and shortness of breath. He had no prior history of diabetes mellitus, dyslipidemia, or tobacco use. An exercise-MIBI stress test performed 1 year earlier as part of a diagnostic work-up for his low EF was negative for ischemia. His pain subsided after admission although his electrocardiogram showed new T-wave inversions in leads V2-V5 (Figure 1) and mild elevation of cardiac enzymes with a troponin T of 0.161 ng/mL (reference range, 0-10 ng/mL). He was transferred to our hospital for urgent cardiac catheterization as part of his management for a complicated non-ST segment elevation myocardial infarction.
Coronary angiography demonstrated giant (>15 mm) ostial aneurysms of the left and right coronary arteries (Figure 2, Video 1). He also had triple-vessel coronary artery disease (CAD) with a 99% stenosis in the mid-left anterior descending artery, an 80% stenosis in the left circumflex artery, and a 99% stenosis in the mid-right coronary artery. A 64-row multidetector computed tomography scan with breath hold and three-dimensional reconstruction also showed the coronary aneurysms in the left (15 mm) and right (21 mm) coronary arteries as well as the concomitant coronary artery disease (Figure 3) and circumferential thickening of both the left and right coronary arteries (Figure 4). A myocardial perfusion positron emission tomography (PET) scan was performed to assess myocardial viability and all regions of the left ventricle, with the exception of a small area at the apex, were found to be viable. Based on this anatomy, atorvastatin 80 mg daily was added to his medical regimen and he was referred for coronary artery bypass graft surgery. As presentation with left main and/or triple-vessel coronary artery giant aneurysms is rare, diagnostic studies were performed preoperatively to identify an underlying etiology for the patient’s vasculopathy. The cause was presumed to be an inflammatory type of vasculitis on the basis of an elevated ESR of 108 mm/hr (reference range, 0-13 mm/hr) and a C-reactive protein of 100.1 mg/L (reference range, 0-3 mg/L). The antineutrophil cytoplasmic antibody (ANCA) was negative and did not reveal a perinuclear pattern, anti-Ro and anti-ssDNA antibodies were negative, and infectious and drug-related causes of the CAAs were excluded on the basis of serologies and history of negative toxicology screens, respectively. Giant cell arteritis as the etiology of the giant coronary artery aneurysms was excluded on the basis of a temporal artery biopsy. Thus, suspicion was raised for antecedent Kawasaki disease, which can be associated with persistent coronary arterial inflammation years after the onset of the disease.5 Upon further questioning, the patient’s parents confirmed that he was hospitalized as a child for a severe rash and fever. Although this is not conclusive, the rash, fever, and multivessel giant coronary aneurysms make the diagnosis of Kawasaki disease very likely.
Discussion. Giant left main and/or multivessel CAAs are rare, comprising only 6.6% of all cases of giant coronary aneurysms (incidence of 0.02%).4 Clinically, the majority of patients with giant CAAs are asymptomatic, but ~33% of patients do present with an acute coronary syndrome. The most important predictor of developing an acute coronary syndrome is the aneurysm size, which is not surprising given the propensity for large aneurysms to thrombose or cause myocardial ischemia.6-9
The most recognized etiology of acquired CAA is Kawasaki disease, which is a syndrome of fever, rash, conjunctivitis, and mucosal changes associated with vasculitis that occurs in infants and young children.10 The major sequelae of Kawasaki disease are seen in the coronary artery vascular tree. CAAs are observed in up to 25% of untreated children and have been associated with an increased risk of thrombosis or progression of atherosclerotic CAD in 1%-2% of affected patients.10,11
Approximately 5% of patients under age 40 years who had a coronary artery angiogram for suspected myocardial ischemia were found to have CAAs attributable to Kawasaki disease.12 The high incidence of ischemic disease may be explained by the subacute vasculitis causing proliferation of the myofibroblasts that leads to progressive stenosis of the coronary artery.13 One study of adult patients with Kawasaki disease or suspected Kawasaki disease that presented with an acute coronary syndrome found that 90% were male, with a median age of 29 years (range, 18-69 years) at presentation, and ~90% were found to have giant coronary aneurysms.14 Another report of 76 patients with giant coronary aneurysms (>8 mm) found that 16% of patients had a myocardial infarction after 25 years of follow-up and 59% required a coronary revascularization procedure.15 This is not surprising given that PET and intravascular ultrasound have demonstrated persistent coronary inflammation that is associated with vascular thickening more than 30 years after the index diagnosis.5
The risk of giant CAA rupture increases with age of the patient, antecedent angina, congestive heart failure, or distal embolization. Coronary artery bypass graft surgery and percutaneous coronary intervention are both selected as revascularization strategies, although the data to guide procedure selection in adults and clinical outcomes are limited.
Conclusion. Unusual presentations of giant CAAs involving the left main coronary artery and/or multivessel giant CAAs warrant consideration of inflammatory, connective tissue disease, infectious, or drug-related causes in addition to atherosclerotic coronary artery disease as the etiology of this rare vascular pathology.
- Cohen P, O’Gara PT. Coronary artery aneurysms: a review of the natural history, pathophysiology, and management. Cardiol Rev. 2008;16(6):301-304.
- Kato H, Sugimura T, Akagi T, et al. Long-term consequences of Kawasaki disease. A 10- to 21-year follow-up study of 594 patients. Circulation. 1996;94(6):1379-1385.
- Nichols L, Lagana S, Parwani A. Coronary artery aneurysm: a review and hypothesis regarding etiology. Arch Pathol Lab Med. 2008;132(5):823-828.
- Halapas A, Lausberg H, Gehrig T, Friedrich I, Hauptmann KE. Giant right coronary artery aneurysm in an adult male patient with non-ST myocardial infarction. Hellenic J Cardiol. 2013;54(1):69-76.
- Suda K, Tahara N, Kudo Y et al. Persistent coronary arterial inflammation in a patient long after the onset of Kawasaki disease. Int J Cardiol. 2012;154(2):193-194
- Yeu BK, Menahem S, Goldstein J. Giant coronary artery aneurysms in Kawasaki disease-the need for coronary artery bypass. Heart Lung Circ. 2008;17(5):404-406.
- McGlinchey PG, Maynard SJ, Graham AN, Roberts MJ, Khan MM. Images in cardiovascular medicine. Giant aneurysm of the right coronary artery compressing the right heart. Circulation. 2005;112(4):e66-e67.
- Augustin N, Wessely R Porner M, Schomig A, Lange R. Giant coronary aneurysm obstructing the right heart. Lancet. 2006;368(9533):386.
- Konen E, Feinberg MS, Morag B et al. Giant right coronary aneurysm: CT angiographic and echocardiographic findings. AJR Am J Roentgenol. 2001;177(3):689-661.
- Newburger JW, Takahashi M, Gerber MA, et al. Diagnosis, treatment, and long-term management of Kawasaki disease: a statement for health professionals from the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association. Pediatrics. 2004;114(6):1708-1733.
- Suzuki A, Kamiya T, Tsuda E, Tsukano S. Natural history of coronary artery lesions in Kawasaki disease. Prog Ped Cardiol. 1997;6(3):211-218.
- Daniels LB, Tjajadi MS, Walford HH, et al. Prevalence of Kawasaki disease in young adults with suspected myocardial ischemia. Circulation. 2012;125(20):2447-2453.
- Orenstein JM, Shulman ST, Fox LM, et al. Three linked vasculopathic processes characterize Kawasaki disease: a light and transmission electron microscopic study. PLoS One. 2012;7(6):e389-e98.
- Tsuda E, Abe T, Tamaki W. Acute coronary syndrome in adult patients with coronary artery lesions caused by Kawasaki disease: review of case reports. Cardiol Young. 2011:21(1);74-82.
- Suda K, Iemura M, Nishiono H, et al. Long-term prognosis of patients with Kawasaki disease complicated by giant coronary aneurysms: a single-institution experience. Circulation. 2011;123(17):1836-1842.
From the 1Division of Cardiac Surgery, Brigham and Women’s Hospital, Harvard Medical School, Boston, Massachusetts;2Department of Cardiology, Boston Children’s Hospital, and Department of Pediatrics, Harvard Medical School, Boston, Massachusetts; and 3Cardiology Division, Brigham and Women’s Hospital, Harvard Medical School, Boston, Massachusetts.
Disclosure: The authors have completed and returned the ICMJE Form for Disclosure of Potential Conflicts of Interest. The authors report no conflicts of interest regarding the content herein.
Manuscript submitted February 3, 2014, provisional acceptance given February 4, 2014, final version accepted February 26, 2014.
Address for correspondence: Jane A. Leopold, MD, 77 Avenue Louis Pasteur, NRB0630K, Boston MA 02115. Email: firstname.lastname@example.org