Ann Thorac Surg 2008;85:1081-1083. doi:10.1016/j.athoracsur.2007.08.032
© 2008 The Society of Thoracic Surgeons
Case Reports
Symptomatic Coronary Obstruction Due to Kawasaki Disease in an Adult
Cindy Codispoti, DOa,
Sheri Boyd, MDa,
David Sees, MDb,
William Conner, MDb,*
a Department of Medicine, Brooke Army Medical Center, Fort Sam Houston, Texas
b Department of Surgery, Brooke Army Medical Center, Fort Sam Houston, Texas
Accepted for publication August 14, 2007.
* Address correspondence to Dr Conner, Department of Surgery, Cardiothoracic Surgery Service, 3851 Roger Brooke Dr, Fort Sam Houston, TX 78234 (Email: william.conner{at}amedd.army.mil).
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Abstract
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Kawasaki disease is the most common cause of pediatric ischemic heart disease in the world, but it is unusual in adults. We present a case of Kawasaki disease in a young adult. This 20-year-old man presented with angina. Coronary angiography revealed aneurysmal obstructive lesions consistent with remote Kawasaki disease. The patient underwent coronary artery bypass grafting with arterial conduits. Postoperative echocardiography was normal and stress myocardial perfusion imaging showed no ischemia. He remained asymptomatic 12 months after surgery. There is controversy regarding optimal therapy, choice of conduit, treatment of proximal aneurysms, and surveillance in adults with Kawasaki disease.
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Introduction
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Kawasaki disease is an acute febrile illness of unknown cause that affects infants and children. It presents as a self-limited vasculitis, most notably affecting the proximal coronary arteries. Treatment with intravenous gamma globulin is stressed early in the clinical course to prevent or limit the development of proximal coronary aneurysms, which are associated with thrombosis or distal thromboembolism. This is rarely evident in adults because the coronary abnormalities often regress after the acute illness [1]. However, there are scattered reports of adults who present with myocardial infarction or sudden cardiac death attributed to persistent coronary aneurysms from remote Kawasaki disease [2]. There remains a paucity of literature regarding optimal therapy in these patients. In 2004, the American Heart Association published new guidelines for the care of patients with Kawasaki disease [1]. However, the recommendations do not adequately address the management of adults who present with symptomatic ischemic heart disease resulting from chronic aneurysms after childhood Kawasaki disease. Questions remain regarding revascularization, choice of conduit, treatment of aneurysms, and long-term surveillance.
A 20-year-old man who is a soldier was referred to us with new onset exertional angina. Diagnostic testing included positive exercise stress test and transthoracic echocardiogram demonstrating mildly depressed left ventricular function. Coronary angiography revealed severe multivessel coronary ectasia, giant coronary aneurysms with calcification, and extensive proximal coronary stenosis (Fig 1). After reporting the diagnostic results to the patient he related a history of Kawasaki disease as an infant. The patient was referred for coronary artery bypass grafting, which was performed with all arterial conduits (right internal mammary artery–distal right coronary artery, left internal mammary artery–left anterior descending coronary artery, left radial artery–obtuse marginal branch). At surgery the proximal coronary segments were noted to be aneurysmal and densely calcified (Fig 2). The aneurysmal segments were not treated. Transthoracic echocardiography 2 months after surgery demonstrated normal left ventricular function. A stress myocardial perfusion study revealed excellent exercise tolerance and no ischemia. Medical treatment included antiplatelet therapy, oral anticoagulation, beta-blocker, and a statin. Routine clinical surveillance for symptoms, positive electrocardiogram, or abnormal echocardiography requiring stress testing and cardiac catheterization was established. The patient remained asymptomatic 1 year post-revascularization.
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Fig 2. Surgical photograph demonstrating calcified, porcelain coronary aneurysms precluding ligation. (Ca2+ = calcium; CX = circumflex coronary artery; L = left; LAD = left anterior descending artery; R = right.)
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Comment
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Symptomatic ischemic heart disease in adults as a consequence of childhood Kawasaki disease is rare. There are no established recommendations regarding therapy, choice of conduit, treatment of aneurysms, or surveillance in this population. In an autopsy series of 6 patients older than 15 years, Takahashi and colleagues [3] demonstrated significant proximal coronary aneurysm with recanalization and obstruction in 60% of coronary arteries examined [3]. He did not report any evidence of coronary aneurysm rupture. Sugao and colleagues [2] reported a case series of 5 adult patients with symptomatic coronary artery obstruction due to childhood Kawasaki disease complicated by myocardial infarction, arrhythmia, or poor left ventricular function [2]. All 5 patients underwent coronary artery bypass grafting with favorable outcomes. Although the authors recommend arterial conduits, they do not address treatment of proximal coronary aneurysms.
Our patient similarly presented with symptomatic ischemia. His coronary anatomy was consistent with long-term sequelae of Kawasaki disease, primarily chronic proximal coronary aneurysms with fibrotic remodeling and obstruction [3]. Given the lack of data regarding the prognosis of adult patients with coronary artery obstruction due to childhood Kawasaki disease, recommendations for revascularization were based on expert opinion and well-established evidence in adults with multivessel obstructive coronary atherosclerosis.
In 2004, Tsuda and Kitamura [4] reported the results of a national survey in Japan and demonstrated that patients receiving an internal thoracic artery graft after the age of 12 had patency rates comparable with adults who received an internal thoracic artery graft for atherosclerotic disease. In the same study, only 70% of children who underwent coronary artery bypass grafting with venous conduits were free of cardiac events at 10 years. Therefore, the superior patency and survival rates associated with internal thoracic artery grafts found in adults with obstructive atherosclerosis seem to apply to the symptomatic adult Kawasaki population. Maximal arterial grafting is strongly recommended [5, 6].
Intuition would suggest a long-term risk of proximal coronary aneurysm rupture, but there are no published reports of this complication in any of the series or guidelines reviewed. Fibrotic obstruction within the aneurysmal segment and myocardial ischemia seem to dominate the clinical presentation in adults, and risk of aneurysm rupture is minimal. Furthermore, it is reported that attempts at coronary aneurysm ligation or exclusion is associated with a prohibitive mortality risk [1, 7]. Given this evidence, an attempt at aneurysm ligation or exclusion is unnecessary and potentially harmful.
Although the long-term prognosis of symptomatic adults with remote Kawasaki disease is yet unknown, it is most likely related to left ventricular function [4]. Therefore, routine surveillance after surgical therapy seems reasonable so that early reintervention can preserve ejection fraction and positively effect survival. As supported by the American Heart Association’s scientific statement on Kawasaki disease, surgical patients should have bi-annual clinic visits with electrocardiogram and echocardiography. Medical therapy should include anti-platelet therapy, beta-blocker, statin, and anticoagulation. Stress testing and cardiac catheterization should be performed when confronted with clinical symptoms or abnormal diagnostic tests.
In conclusion, symptomatic adults with ischemic heart disease as sequelae of childhood Kawasaki disease should be revascularized using arterial conduits, especially an internal thoracic artery graft. Addressing the proximal coronary aneurysms prohibitively increases operative risk and is not required. Because the long-term prognosis of these patients is unknown, close clinical follow-up, aggressive medical therapy, and appropriate diagnostic testing are recommended.
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References
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- Newburger JW, Masato T, Gerber MA, et al. Diagnosis, treatment, and long-term management of Kawasaki disease Circulation 2004;110:2747-2771.[Abstract/Free Full Text]
- Sugao I, Katsuo F, Shinichiro N, et al. Adult coronary artery disease secondary to Kawasaki disease in childhood Am J Cardiol 1992;23:692-694.
- Takahashi K, Oharaseki S. Pathological study of post coronary arteritis in adolescents and young adults: with reference to the relationship between sequelae of Kawasaki disease and atherosclerosis Pediatr Cardiol 2001;22:138-142.[Medline]
- Tsuda E, Kitamura S. National survey of coronary artery bypasses grafting for coronary stenosis caused by Kawasaki disease in Japan Circulation 2004;110:II61-II66.[Medline]
- Yoshiro Y, Yagihara T, Kameda Y, et al. Result of surgical treatments in patients with coronary-arterial obstructive disease after Kawasaki disease Eur J Cardiothorac Surg 2000;17:515-519.[Abstract/Free Full Text]
- Kitamura 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]
- Kitamura S. The role of coronary bypass operation on children with Kawasaki disease Coron Artery Dis 2002;13:437-447.[Medline]
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Abstract
Introduction
