Kawasaki syndrome

Coronary artery ectasia (CAE) is an uncommon disorder diagnosed in 1 to 4% of patients undergoing coronary arteriography.[1-3] CAE usually is considered a variant of atherosclerotic coronary artery disease.[1-3] Coronary artery disease (CAD) in young adults (age [is less than] 40 years) usually occurs in patients with multiple predisposing factors, such as hyperlipidemia, cigarette smoking, type 1 diabetes mellitus, hypertension, strong family history, and others. In the absence of predisposing factors, other causes should be considered, particularly mucocutaneous lymph node syndrome (Kawasaki's disease [KD]).[4-6] We describe a young patient with three-vessel aneurysmal CAD presenting with multiple myocardial infarctions (MIs). The clinical, differential diagnosis and therapeutic features of this rare disorder are discussed.

CASE REPORT

A 46-year-old white man had a first MI at the age of 32 years. At that time, coronary arteriography demonstrated diffuse three-vessel coronary artery aneurysmal disease. He was treated medically, but over the subsequent 14 years he had 4 more non-Q-wave MIs. Thallium 201 stress tests performed after each episode were inconclusive. Following a recent non-Q-wave MI, he presented with postinfarction angina. His past medical history was significant for severe asthma and less than a pack per day of cigarette smoking. There was no history of hypertension, diabetes mellitus, or hyperlipidemia. His parents had CAD at a late age ([is greater than] 65 years). The patient was unable to recall any definite symptoms of acute KD in childhood. However, he had experimental exertional chest pain since he was a teenager. Physical examination and laboratory data disclosed no abnormalities. An ECG was entirely normal without Q waves or ST-T segment changes. A two-dimensional Doppler echocardiogram showed a left ventricular ejection fraction of 40% with inferoposterior hypokinesis and 2+ mitral regurgitation. The origins of the coronary arteries on the short axis view of the aortic root were not well visualized. A thallium 201 scintigraphy and a dobutamine echocardiographic stress test both showed anterior and inferoposterior reversible ischemia. Coronary arteriography (Fig 1) demonstrated severe disease involving the left anterior descending, the circumflex and the right coronary arteries. The proximal segments of the arteries were very ectatic. Multiple aneurysms alternating with severe stenoses were seen along the entire lengths of the vessels, an appearance typically seen in KD.[7-9] He underwent a four-vessel coronary artery bypass grafting with a left internal mammary artery to the left anterior descending artery, a right internal mammary artery to the right coronary artery, and a sequential graft from the proximal aorta to the first diagonal branch and to the first obtuse marginal branch using the left radial artery. Six months after his operation, the patient was in functional class I and had no further cardiac events; a thallium 201 scintigraphy showed no myocardial ischemia.

DISCUSSION

Coronary artery ectasia is an uncommon disorder, which is diagnosed in 1 to 4% of coronary arteriographies.[1-3] Most cases are considered a variant of atherosclerotic CAD.[1-3] KD is a febrile disorder of unknown cause with multiple systemic manifestations, affecting primarily infants and young children.[5,6] First described in Japan by Kawasaki in 1967[10] as acute mucocutaneous lymph node syndrome, it has been reported with increasing frequency around the world and is now considered the leading cause of acquired heart disease in children in the United States.[5,6] Involvement of the heart with acute myocarditis and coronary angiitis occurs in 25 to 50% of the patients during the acute phase, accounting for most of the mortality.[5,6] However, 50% of the aneurysms regress spontaneously over a 1- to 2-year period, and therefore adult ischemic heart disease secondary to KD is infrequent and occurs mostly in young adults.[7,8]

In many patients, history of childhood KD cannot be obtained. This is because the diagnosis of acute KD is based on clinical criteria only, without specific laboratory testing, and therefore requires a high index of suspicion.[6] Also, similar to rheumatic fever, in many patients there is a latent period of several years between childhood KD and onset of symptoms of ischemic heart disease.[7,8] In young adults, diagnosis is based on typical features in two-dimensional echocardiography and coronary arteriography. The former consists of local wall motion abnormalities as a result of prior MIs and ectasia or frank aneurysms of the proximal coronary arteries.[11] Echocardiography is particularly helpful in the pediatric population, both for initial diagnosis and for long-term follow-up.[11] Coronary arteriography typically reveals multivessel aneurysmal disease alternating with segmental stenoses, coronary ectasia, calcifications, rich collateral circulation, and varied degrees of left ventricular dysfunction as a sequela of multiple MIs or myocarditis or both.[7-9] We believe that our patient had KD, rather than CAE, for several reasons: (a) The patient developed extensive three-vessel disease at a very early age. He was a mild cigarette smoker without any other risk factors for atherosclerotic CAD. This would favor KD because patients with CAE have the typical risk profile of atherosclerotic CAD.[1-6] (b) The patient had extensive three-vessel disease. This is typical for KD but would be unusual for CAE, which tends to involve one or two vessels only.[1-3,7-9] It is possible that many patients previously diagnosed with CAE had actually had KD, since awareness to the latter entity has increased only recently. In our patient, echocardiography failed to reveal features of KD because the very proximal left main and right coronary arteries were spared. This is not uncommon in KD.[5-9] However, coronary arteriography clearly demonstrated the typical findings.

The indications for myocardial revascularization in CAE are similar to those in other patients with atherosclerotic CAD.[1-3] In contrast, the indications in children with KD have been a matter of controversy because of the technical difficulties in performing bypass grafting, unclear natural history of the disease, and questions of long-term patency and growth of the grafts.[12] However, since the first coronary artery bypass grafting performed by Kitamura et al[13] in 1976, it has become clear that coronary artery bypass grafting in these patients can be performed with low early morbidity and improved late outcome.[14-17] Internal mammary arteries are the conduit of choice because they have better long-term patency and have the potential to grow when used in young children.[14-17] The excellent results recently reported with the radial artery in atherosclerotic CAD[18] and the unsatisfactory long-term results with saphenous vein grafts in KD[14,15] support our decision to utilize the radial artery as adjunct to the internal mammary arteries to achieve complete arterial revascularization. However, to the best of our knowledge this is the first time it has been used in patients with KD, and longer follow-up on more patients is needed to support the use of the radial artery in this disease.

[Figure 1 ILLUSTRATION OMITTED]

REFERENCES

[1] Hartnell GG, Parnell BM, Pridie RB. Coronary artery ectasia: its prevalence and clinical significance in 4,993 patients. Br Heart J 1985; 54:392-95

[2] Swaye PS, Fisher LD, Litwin P, et al. Aneurysmal coronary artery disease. Circulation 1983; 67:134-38

[3] Swanton RH, Thomas ML, Coltart DJ, et al. Coronary artery ectasia--a variant of occlusive coronary arteriosclerosis. Br Heart J 1978; 40:393-400

[4] Farmer JA, Gotto AM Jr. Risk factors for coronary artery disease. In: Braunwald E, ed. Heart disease--a textbook of cardiovascular medicine. Philadelphia: WB Saunders, 1992; 1125-60

[5] Leung DYM. Kawasaki disease. Curr Opin Rheumatol 1993; 5:41-50

[6] D'Amico TA. Kawasaki's disease. In: Sabiston DC, Spencer FC, eds. Surgery of the chest. Philadelphia: WB Saunders, 1995; 1990-95

[7] Ishiwata S, Katsuo F, Nishiyama S, et al. Adult coronary artery disease secondary to Kawasaki disease in childhood. Am J Cardiol 1992; 69:692-94

[8] Kato H, Kawasaki T, Fujiwara H, et al. Adult coronary artery disease probably due to childhood Kawasaki disease. Lancet 1992; 340:1127-29

[9] Nakano H, Ueda K, Saito A, et al. Repeated quantitative angiograms in coronary arterial aneurysm in Kawasaki disease. Am J Cardiol 1985; 56:846-51

[10] Kawasaki T. Acute febrile mucocutaneous syndrome with lymphoid involvement with specific desquamation of the fingers and toes in children. Jpn J Allergy 1967; 16:178-222

[11] Akagi T, Rose V, Benson LN, et al. Outcome of coronary artery aneurysms after Kawasaki disease. J Pediatr 1992; 121:689-94

[12] Suzuki A, Kamiya T, Ono Y, et al. Indication of aortocoronary bypass for coronary arterial obstruction due to Kawasaki disease. Heart Vessels 1985; 1:94-100

[13] Kitamura S, Kawashima Y, Fujita T, et al. Aortocoronary bypass grafting in a child with coronary artery obstruction due to mucocutaneous lymph node syndrome: report of a case. Circulation 1976; 53:1035-40

[14] Hirose H, Kawashima Y, Nakano S, et al. Long-term results in surgical treatment of children 4 years old or younger with coronary involvement due to Kawasaki disease. Circulation 1986; 74(suppl I):I-77-81

[15] Suzuki A, Kamiya T, Ono Y, et al. Aortocoronary bypass surgery for coronary arterial lesions resulting from Kawasaki disease. J Pediatr 1990; 116:567-73

[16] Kitamura S, Seki T, Kawachi K, et al. Excellent patency and growth potential of internal mammary artery grafts in pediatric coronary artery bypass surgery: new evidence for a "live" conduit. Circulation 1988; 78(suppl I):I-129-39

[17] Kawachi K, Kitamura S, Seki T, et al. Hemodynamics and coronary blood flow during exercise after coronary artery bypass grafting with internal mammary arteries in children with Kawasaki disease. Circulation 1991; 84:618-24

[18] Dietl CA, Benoit CH. Radial artery graft for coronary revascularization. Ann Thorac Surg 1995; 60:102-09

(*) From the Department of Cardiothoracic Surgery, Boston University Medical Center, Boston.

Manuscript received July 17, 1996; revision accepted August 23. Reprint requests: Dr. Shapira, Department of Cardiothoracic Surgery, Boston University Medical Center, 88 East Newton Street, Boston, MA 02118

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