Consult the medical resource doctors trust

UpToDate is one of the most respected medical information resources in the world, used by over 360,000 doctors and thousands of patients to find answers to medical questions.

  • Content written by a faculty of over 4,000 physicians from leading medical institutions
  • Unbiased: free of advertising or pharmaceutical funding
  • Evidence-based treatment recommendations
  • Continuously updated to incorporate new medical findings

Cardiovascular sequelae of Kawasaki disease

INTRODUCTION

Kawasaki disease (KD) is a vasculitis of infancy and early childhood. It was first described by Dr Tomisaku Kawasaki in 1967, and initially designated as the mucocutaneous lymph node syndrome [1]. KD is an immune-mediated vasculitis with self-limited clinical course and unknown etiology.

Fever and manifestations of acute inflammation last for an average of 12 days without therapy. However, the disease may be complicated by the development of aneurysms in coronary arteries, which can lead to myocardial ischemia or infarction (MI) (movie 1 and movie 2 and movie 3). In untreated patients, the incidence of coronary artery aneurysms is about 20 to 25 percent with a mortality rate of approximately 2 percent.

Early detection of the disease and prompt initiation of therapy with aspirin and intravenous immune globulin (IVIG) have reduced the death rate to well below 1 percent and the prevalence of coronary artery aneurysms to approximately 5 percent.

An overview of the clinical features, natural history, treatment and follow-up of cardiovascular sequelae of Kawasaki disease will be presented here. The etiology, epidemiology, clinical manifestations, diagnosis, and treatment of Kawasaki disease are discussed separately. (See "Epidemiology and etiology of Kawasaki disease" and "Clinical manifestations and diagnosis of Kawasaki disease" and "Initial treatment and prognosis of Kawasaki disease".)

CARDIAC MANIFESTATIONS IN THE ACUTE PHASE

After a brief nonspecific prodrome of respiratory or gastrointestinal symptoms, the patient with KD typically experiences a combination of the following signs and symptoms.

To continue reading this article you need to subscribe.

Read the rest of this article and others like it

The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use (click here) ©2009 UpToDate, Inc.
References Top
  1. Kawasaki, T, Kosaki, F, Okawa, S, et al. A new infantile acute febrile mucocutaneous lymph node syndrome (MLNS) prevailing in Japan. Pediatrics 1974; 54:271.
  2. 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. Circulation 2004; 110:2747.
  3. Towbin, JA, Bricker, JT, Garson, Jr A. Electrocardiographic criteria for diagnosis of acute myocardial infarction in childhood. Am J Cardiol 1992; 69:1545.
  4. Nakanishi, T, Takao, A, Kondoh, C, et al. ECG findings after myocardial infarction in children after Kawasaki disease. Am Heart J 1988; 116:1028.
  5. Garson, Jr A. The Electrocardiogram in Infants and Children. A Systematic Approach, Lea & Febiger, Philadelphia, 1983.
  6. Moran, AM, Newburger, JW, Sanders, SP, et al. Abnormal myocardial mechanics in kawasaki disease: rapid response to gamma-globulin. Am Heart J 2000; 139:217.
  7. Ravekes, WJ, Colan, SD, Gauvreau, K, et al. Aortic root dilation in Kawasaki disease. Am J Cardiol 2001; 87:919.
  8. Suzuki, A, Kamiya, T, Tsuda, E, et al. Natural history of coronary artery lesions in Kawasaki disease. Prog Pediatr Cardiol 1997; 6:211.
  9. McMorrow Tuohy, AM, Tani, LY, Cetta, F, et al. How many echocardiograms are necessary for follow-up evaluation of patients with Kawasaki disease?. Am J Cardiol 2001; 88:328.
  10. 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.
  11. Takahashi, M, Mason, W. Can echocardiogram predict coronary angiographic findings: Size, thrombosis, and stenosis In: Proceedings of the Fourth International Symposium on Kawasaki Disease, Takahashi, M, Taubert, K (Eds), American Heart Association, Dallas 1993. p.336.
  12. Beiser, AS, Takahashi. M, Baker, AJ, et al, for the US Multicenter Kawasaki Disease Study Group. A predictive instrument for coronary artery aneurysms in Kawasaki disease. Am J Cardiol 1998; 81:1116.
  13. Burns, JC, Glode, MP. Kawasaki syndrome. Lancet 2004; 364:533.
  14. Senzaki, H, Masutani, S, Kobayashi, J, et al. Circulating matrix metalloproteinases and their inhibitors in patients with Kawasaki disease. Circulation 2001; 104:860.
  15. Ikeda, K, Ihara, K, Yamaguchi, K, et al. Genetic analysis of MMP gene polymorphisms in patients with Kawasaki disease. Pediatr Res 2008; 63:182.
  16. Biezeveld, MH, Kuipers, IM, Geissler, J, et al. Association of mannose-binding lectin genotype with cardiovascular abnormalities in Kawasaki disease. Lancet 2003; 361:1268.
  17. Kariyazono, H, Ohno, T, Khajoee, V, et al. Association of vascular endothelial growth factor (VEGF) and VEGF receptor gene polymorphisms with coronary artery lesions of Kawasaki disease. Pediatr Res 2004; 56:953.
  18. Yasukawa, K, Terai, M, Shulman, ST, et al. Systemic production of vascular endothelial growth factor and fms-like tyrosine kinase-1 receptor in acute Kawasaki disease. Circulation 2002; 105:766.
  19. Takahashi, M, Mason, W, Lewis, AB. Regression of coronary aneurysms in children with Kawasaki syndrome. Circulation 1987; 75:387.
  20. Akagi, T, Rose, V, Benson, LN, et al. Outcome of coronary artery aneurysms after Kawasaki disease. J Pediatr 1992; 121:689.
  21. Kato, H, Ichinose, E, Yoshioka, F, et al. Fate of coronary aneurysms in Kawasaki disease: Serial coronary angiography and long-term follow-up study. Am J Cardiol 1982; 49:1758.
  22. Suzuki, A, Arakaki, Y, Sugiyama, H, et al. Observation of coronary arterial lesion due to Kawasaki disease by intravascular ultrasound. In: Kawasaki disease: Proceedings of the 5th International Kawasaki Disease Symposium, Kato, H (Ed), Elsevier, Amsterdam, 1995, p. 451.
  23. Iemura, M, Ishii, M, Sugimura, T, et al. Long term consequences of regressed coronary aneurysms after Kawasaki disease: vascular wall morphology and function. Heart 2000; 83:307.
  24. Sugimura, T, Kato, H, Inoue, O, et al. Vasodilator response of coronary arteries after Kawasaki disease: Evaluation by intracoronary injection of isosorbide dinitrate. J Pediatr 1992; 121:684.
  25. Mitani, Y, Okuda, Y, Shimpo, H, et al. Impaired endothelial dysfunction in epicardial arteries after Kawasaki disease. Circulation 1997; 96:454.
  26. Koutlas, TC, Wernovsky, G, Bridges, ND, et al. Orthotopic heart transplantation for Kawasaki disease after rupture of a giant coronary artery aneurysm. J Thorac Cardiovasc Surg 1997; 113:217.
  27. Naoe, S, Takahashi, K. Morphological aspects of past coronary arteritis in Kawasaki disease with particular emphasis on recanalization of thrombus occluded arteries. In: Proceedings of the Fourth International Symposium on Kawasaki Disease, Takahashi, M, Taubert, K (Eds), American Heart Association, Dallas 1993. p.231.
  28. Suzuki, A, Miyagawa-Tomita, S, Komatsu, K, et al. Active remodeling of the coronary arterial lesions in the late phase of kawasaki disease: immunohistochemical study. Circulation 2000; 101:2935.
  29. Ogawa, S, Fukazawa, R, Ohkubo, T, et al. Silent myocardial ischemia in Kawasaki Disease. Evaluation of percutaneous transluminal coronary angioplasty by dobutamine stress testing. Circulation 1997; 96:3384.
  30. Cardiovascular risk reduction in high-risk pediatric populations. Pediatrics 2007; 119:618.
  31. Dalla Pozza, R, Bechtold, S, Urschel, S, et al. Subclinical atherosclerosis, but normal autonomic function after Kawasaki disease. J Pediatr 2007; 151:239.
  32. Noto, N, Okada, T, Yamasuge, M, et al. Noninvasive assessment of the early progression of atherosclerosis in adolescents with Kawasaki disease and coronary artery lesions. Pediatrics 2001; 107:1095.
  33. Cheung, YF, Wong, SJ, Ho, MH. Relationship between carotid intima-media thickness and arterial stiffness in children after Kawasaki disease. Arch Dis Child 2007; 92:43.
  34. Fujiwara, H, Hamashima, Y. Pathology of the heart in Kawasaki disease. Pediatrics 1978; 61:100.
  35. Sasaguri, Y, Kato, H. Regression of aneurysms in Kawasaki disease: a pathological study. J Pediatr 1982; 100:225.
  36. Sugimura, T, Kato, H, Inoue, O, et al. Intravascular ultrasound of coronary arteries in children. Assessment of the wall morphology and the lumen after Kawasaki disease. Circulation 1994; 89:258.
  37. Kurisu, Y, Azumi, T, Sugahara, T, et al. Variation in coronary arterial dimension (distensible abnormality) after disappearing aneurysm in Kawasaki disease. Am Heart J 1987; 114:532.
  38. Cheung, YF, Yung, TC, Tam, SC, et al. Novel and traditional cardiovascular risk factors in children after Kawasaki disease: implications for premature atherosclerosis. J Am Coll Cardiol 2004; 43:120.
  39. Dhillon, R, Clarkson, P, Donald, AE, et al. Endothelial dysfunction late after Kawasaki disease. Circulation 1996; 94:2103.
  40. Niboshi, A, Hamaoka, K, Sakata, K, Yamaguchi, N. Endothelial dysfunction in adult patients with a history of Kawasaki disease. Eur J Pediatr 2008; 167:189.
  41. Mitani, Y, Ohashi, H, Sawada, H, et al. In vivo plaque composition and morphology in coronary artery lesions in adolescents and young adults long after kawasaki disease: a virtual histology-intravascular ultrasound study. Circulation 2009; 119:2829.
  42. McCrindle, BW, McIntyre, S, Kim, C, et al. Are patients after Kawasaki disease at increased risk for accelerated atherosclerosis?. J Pediatr 2007; 151:244.
  43. Gupta-Malhotra, M, Gruber, D, Abraham, SS, et al. Atherosclerosis in survivors of Kawasaki disease. J Pediatr 2009; 155:572.
  44. Kato, H, Ichinose, E, Kawasaki, T. Myocardial infarction in Kawasaki disease: Clinical analysis of 195 cases. J Pediatr 1986; 108:923.
  45. Scott, JS, Ettedgui, JA, Neches, WH. Cost-effective use of echocardiography in children with Kawasaki disease. Pediatrics 1999; 104:E57.
  46. Hamaoka, K, Onouchi, Z, Kamiya, Y, et al. Evaluation of coronary flow velocity dynamics and flow reserve in patients with Kawasaki Disease by means of a Doppler guide wire. J Am Coll Cardiol 1998; 31:833.
  47. Kanamaru, H, Sato, Y, Takayama, T, et al. Assessment of coronary artery abnormalities by multislice spiral computed tomography in adolescents and young adults with Kawasaki disease. Am J Cardiol 2005; 95:522.
  48. Rhodes, J, Hijazi, ZM, Marx, GR, et al. Aerobic exercise function of patients with persistent coronary artery aneurysms secondary to Kawasaki disease. Pediatr Cardiol 1996; 17:226.
  49. Beller, GA. Pharmacological stress imaging. JAMA 1991; 265:633.
  50. Fukuda, T, Akagi, T, Ishibashi, M, et al. Noninvasive evaluation of myocardial ischemia in Kawasaki disease: Comparison between dipyridamole stress thallium imaging and exercise stress testing. Am Heart J 1998; 135:482.
  51. Miyagawa, M, Mochizuki, T, Murase, K, et al. Prognostic value of dipyridamole-thallium myocardial scintigraphy in patients with Kawasaki disease. Circulation 1998; 98:990.
  52. Noto, N, Ayusawa, M, Karasawa, H, et al. Combination of dobutamine stress and myocardial contrast echocardiography. In: Kawasaki disease: Proceedings of the 5th International Kawasaki Disease Symposium, Kato, H (Ed), Elsevier, Amsterdam, 1995, p. 466.
  53. Fukuda, T. Myocardial ischemia in Kawasaki disease: Evaluation by dipyridamole stress thallium-201 myocardial imaging and exercise test. Kurume Med J 1992; 39:245.
  54. Hijazi, ZM, Udelson, JE, Snapper, H, et al. Physiologic significance of chronic coronary aneurysms in patients with Kawasaki disease. J Am Coll Cardiol 1994; 25:1633.
  55. Paridon, SM, Galioto, FM, Vincent, JA, et al. Exercise capacity and incidence of myocardial perfusion defects after Kawasaki disease in children and adolescents. J Am Coll Cardiol 1995; 25:1420.
  56. Mavrogeni, S, Papadopoulos, G, Douskou, M, et al. Magnetic resonance angiography is equivalent to X-ray coronary angiography for the evaluation of coronary arteries in Kawasaki disease. J Am Coll Cardiol 2004; 43:649.
  57. Greil, GF, Stuber, M, Botnar, RM, et al. Coronary magnetic resonance angiography in adolescents and young adults with Kawasaki disease. Circulation 2002; 105:908.
  58. Sugimura, T, Kato, H, Inoue, O, et al. Intravascular ultrasound of coronary arteries in children: Assessment of the wall morphology and the lumen after Kawasaki disease. Circulation 1994; 84:258.
  59. Williams, RV, Wilke, VM, Tani, LY, Minich, LL. Does abciximab enhance regression of coronary aneurysms resulting from Kawasaki disease?. Pediatrics 2002; 109:E4.
  60. Suzuki, A, Kamiya, T, Ono, Y, et al. Follow-up study of coronary arterial lesions caused by Kawasaki disease. In: Proceedings of the Fourth International Symposium on Kawasaki Disease, Takahashi, M, Taubert, K (Eds), American Heart Association, Dallas 1993, p. 317.
  61. Levy, DM, Silverman, ED, Massicotte, MP, et al. Longterm outcomes in patients with giant aneurysms secondary to Kawasaki disease. J Rheumatol 2005; 32:928.
  62. Burtt, DM, Pollack, P, Bianco, JA. Intravenous streptokinase in an infant with Kawasaki disease complicated by acute myocardial infarction. Pediatr Cardiol 1986; 6:307.
  63. Kato, H, Ichinose, E, Inoue, O, et al. Intracoronary thrombolytic therapy in Kawasaki disease. In: Kawasaki disease, Shulman, ST (Ed), Alan Liss, New York, 1987, p. 445.
  64. Sonobe, T, Kataoka, T, Tsuchiya, K, et al. Intravenous coronary thrombolysis using recombinant tissue plasminogen activator for intra-aneurysmal thrombi in Kawasaki disease. In: Proceedings of the Fourth International Symposium on Kawasaki Disease, Takahashi, M, Taubert, K (Eds), American Heart Association, Dallas, 1993, p. 363.
  65. Ishii, M, Ueno, T, Akagi, T, et al. Guidelines for catheter intervention in coronary artery lesion in Kawasaki disease. Pediatr Int 2001; 43:558.
  66. Sugimura, T, Yokoi, H, Sata, N, et al. Interventional treatment for children with severe coronary artery stenosis with calcification after long-term Kawasaki disease. Circulation 1997; 96:3928.
  67. Ishii, M, Ueno, T, Ikeda, H, et al. Sequential follow-up results of catheter intervention for coronary artery lesions after Kawasaki disease: quantitative coronary artery angiography and intravascular ultrasound imaging study. Circulation 2002; 105:3004.
  68. Li, SS, Cheng, BC, Lee, SH. Images in cardiovascular medicine. Giant coronary aneurysm formation after sirolimus-eluting stent implantation in Kawasaki disease. Circulation 2005; 112:e105.
  69. Kitamura, S, Kameda, Y, Seki, T, et al. Long-term outcome of myocardial revascularization in patients with Kawasaki coronary artery disease. A multicenter cooperative study. J Thorac Cardiovasc Surg 1994; 107:663.
  70. Kitamura, S, Tsuda, E, Kobayashi, J, et al. Twenty-five-year outcome of pediatric coronary artery bypass surgery for Kawasaki disease. Circulation 2009; 120:60.
  71. Tsuda, E, Kitamura, S. National survey of coronary artery bypass grafting for coronary stenosis caused by Kawasaki disease in Japan. Circulation 2004; 110:II61.
  72. Checchia, PA, Pahl, E, Shaddy, RE, Shulman, ST. Cardiac transplantation for Kawasaki disease. Pediatrics 1997; 100:695.
white circle LOG IN
white circle DEMO