Ann Thorac Surg 2004;78:693-695
© 2004 The Society of Thoracic Surgeons
Case report
Rupture of a giant coronary artery aneurysm due to Kawasaki disease
Thomas M.T. Hwong, FRCSa,
Ahmed A. Arifi, MDa*,
Innes Y.P. Wan, FRCSa,
K.H. Thung, FRCSa,
Song Wan, MD, PhDa,
Rita Y.T. Sung, MDb,
Anthony P.C. Yim, MDa
a Division of Cardiothoracic Surgery, Department of Surgery, The Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, Hong Kong
b Division of Pediatric Cardiology, Department of Pediatrics, The Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, Hong Kong
Accepted for publication June 6, 2003.
* Address reprint requests to Dr Arifi, Division of Cardiothoracic Surgery, The Department of Surgery, The Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, Hong Kong
e-mail: arifiahmed{at}hotmail.com
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Abstract
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Coronary artery aneurysm requiring surgery is rare. We report a case of a ruptured giant coronary artery aneurysm due to Kawasaki vasculitis which presented with cardiac arrest and was successfully treated by emergency coronary artery bypass grafting. The controversies surrounding the management of this disease are also discussed.
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Introduction
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Kawasaki disease is an acute febrile illness that affects children younger than 4 years of age. This acute vasculitis syndrome of unknown etiology involves small and medium-sized arteries, particularly the coronary artery. The affected arteries develop aneurysmal formation, thrombotic occlusion, premature atherosclerosis, and progression to ischemic heart disease [1].
A 3-year-old boy was admitted to our hospital with a 10-day history of fever, malaise, and poor oral intake. His physical examination revealed an erythematous oropharynx and cervical lymph node enlargement. Laboratory findings included a white cell count of 7,000 cells/mL, and hematocrit of 27%, a platelet count of 1,110,000 cells/mL, and a C reactive protein of 39 mg/L. The electrocardiogram was normal, and the chest radiograph demonstrated cardiomegaly. On the day of admission, a two-dimensional echocardiogram revealed an 18-mm aneurysm of the right coronary artery (RCA) and a 4.7-mm aneurysm of the left anterior descending artery (Fig 1A). A diagnosis of Kawasaki disease was established and he was started on intravenous (IV) gamma immunoglobulin, oral aspirin, and IV heparin for anticoagulation. Repeated echocardiograms on day 2 and day 4 after admission showed progressive enlargement of the RCA aneurysm to 21.6 mm; platelet receptor blocker was started and further IV gamma immunoglobulin given. A follow-up echocardiogram on day 6 showed a further enlargement of the RCA aneurysm to 24.7 mm. A magnetic resonance angiogram was performed on day 8 that demonstrated a giant aneurysm of 30 mm in diameter arising from the proximal RCA with eccentric thrombus (Fig 1B).
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Fig 1. (A) An echocardiogram showing a giant aneurysm arising from the origin of the right coronary artery (RCA) measures 18.0 mm (long white line). The origin of the left coronary artery (LCA), also dilated, measures 4.7 mm (short white line). The aortic root (Ao), right ventricle (RV), and the left atrium (LA) are shown. (B) A sagittal section of the magnetic resonance angiogram demonstrating a giant coronary aneurysm (white arrows) arising from the RCA. The Ao, RV, LA, and the diaphragm (Dia) are shown.
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While preparations for operation were being made, the patient went into cardiac arrest while still in the ward (electromechanical dissociation type). Full resuscitation was started immediately and a cardiac tamponade was confirmed by bedside echocardiogram with a presumptive diagnosis of a rupture of the giant aneurysm. Emergency bedside relief of the cardiac tamponade through a partial sternotomy was performed. The patient regained his cardiac output soon afterwards and he was transferred to the operating room for immediate surgery.
After median sternotomy was performed, RCA aneurysm rupture was confirmed (Fig 2A). Cardiopulmonary bypass was established and saphenous vein graft was harvested from the left leg. Myocardial protection was achieved with cold blood cardioplegia. Examination of the coronary arterial systems showed a giant aneurysm involving the main RCA to the origin of the posterior descending artery. Multiple small aneurysms were noted that involved the left anterior descending artery and the circumflex artery. The RCA aneurysm was resected and the mural thrombus removed. The origin of the RCA was plicated using 6-0 Prolene (Ethicon, Somerville, NJ) suture. The origin of the posterior descending artery, which was 1.0 mm in diameter, was carefully dissected and arteriotomy was performed to match the size of the venous graft, which was 1.5 mm in diameter. The anastomosis was completed using 8-0 Prolene continuous suture with the aid of the intracoronary shunt in placed to protect the lumen (Fig 2B). The patient was weaned off the cardiopulmonary bypass with inotropic support. He was extubated on postoperative day 2 and returned to the pediatric ward soon afterwards. His subsequent recovery was uneventful and he was eventually discharged from the hospital 12 days after the operation. The patient has been well since discharge and at 2 months follow-up has returned to full activities as expected for his age. Echocardiogram and Doppler ultrasounds at 1 and 2 months after the operation showed functional and patent graft with no progressive dilatation of the left coronary artery aneurysm.
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Fig 2. (A) The anesthesiologist's view during the operation showing aneurysmal dilation of the coronary artery (A) and the point of rupture (arrows). (B) The anesthesiologist's view showing the resected coronary aneurysm (black arrows) and the patent venous graft (white arrows).
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Comment
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Kawasaki disease is a generalized vasculitis of unknown etiology generally found in infancy and early childhood with characteristic clinical features and specific diagnostic criteria (Table 1). The generalized vasculitis also affects the coronary artery resulting in weakness of the arterial wall, which leads to dilatation and aneurysm formation. The incidence is highest in Japan at 100 per 100,000 children in the pediatric age group [2]. The corresponding incidence in Hong Kong is reported to be 30 per 100,000 children [3].
In the untreated group, coronary aneurysm occurs in about 20% of all cases, whereas coronary aneurysm develops in only 4% to 8% of children who receive IV gamma immunoglobulin [2]. Based on autopsy data, the incidence of coronary aneurysm rupture is reported to occur in 5% of cases [4]. Thrombosis of such coronary aneurysm results in myocardial infarction, the leading cause of death for these children [1].
Despite the generally accepted standard approach to the management of this disease, controversies persist regarding certain aspects of treatment. How should patients with giant coronary aneurysm be managed? Giant coronary aneurysms (defined as aneurysms with an internal luminal diameter of 8 mm or more) are much less likely to reduce in size and will often thrombose or rupture [5]. To us, pending aneurysmal rupture in spite of maximal medical therapy, as in this case, is a clear indication for surgery as ruptured giant coronary aneurysms carries very high mortality. Coronary artery bypass grafting for coronary artery stenosis, ventricular aneurysmectomy, valve replacement, and cardiac transplantation has all been described for the treatment of coronary ischemic sequelae of Kawasaki disease, but no clear recommendation is available for the optimal timing of surgical intervention during the acute phase. We believe, as highlighted in this case, that patients with enlarging coronary aneurysms should be monitored closely and early surgical intervention should be considered when repeated assessments demonstrate a progression of the size of the aneurysm despite maximal medical therapy. In this case we successfully saved a child's life with coronary bypass grafting using venous graft alone. Although the published data suggest that an arterial graft carries a better long-term patency rate and grows with the child [6], we chose the long saphenous vein because the anastomosis site for the posterior descending artery was very distal and required a long graft that could only be provided safely by the long saphenous vein in such an emergency situation.
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References
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- 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;94:1379-1385.[Abstract/Free Full Text]
- Yanagawa H., Yashiro M., Nakamura Y., Kawasaki T., Kato H. Results of 12 nationwide epidemiological incidence surveys of Kawasaki disease in Japan. Arch Pediatr Adolesc Med 1995;149:779-783.[Abstract/Free Full Text]
- Ng YM, Sung R, So LY, et al. A 5 years surveillance of Kawasaki Disease in Hong Kong-1999. Hong Kong Kawasaki Disease study group. Presented at the 10th Asian Congress of Pediatrics, March 26–30, 1994, Taipai, Taiwan
- Takahashi K, Oharaseki T, Wakayama M, Shibuya K, Naoe S. Pathological study of ruptured coronary artery aneurysms of Kawasaki disease. Abstract presented at the 2nd World Congress of Pediatric cardiology and Cardiac Surgery, May 11–15, 1997, Honolulu, HI. Abstract 202
- Nakano H., Ueda K., Saito A., Nojima K. Repeated quantitative angiograms in coronary arterial aneurysm in Kawasaki Disease. Am J Cardiol 1985;56:846-851.[Medline]
- Kitamuran S., Kameda Y., Seki T., et al. Long-term outcome of myocardial revascularization in patients with Kawasaki coronary artery disease. J Thorac Cardiovasc Surg 1994;107:663-674.[Abstract/Free Full Text]
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Abstract
Introduction
