Kawasaki's disease

[Andrea-Damon]

  My boyfriend was over last night, and it has been diagnosed that he has Kawasaki Disease. He is 17 yr. old.  He told me he felt nauseated and I told him to get up and I'd get him some pepto bismo.  He said he did'nt want to get up cause his heart was racing.   I pulled him up and he fell into my face, then fell backwards and landed.  I thought he was just playing till I realized he did'nt hear me and was just staring straight ahead. This lasted about 9 seconds.  I slapped his face to get him to respond.  When he came back he had no recollection of falling or being out of it.  He says his heart races, thats normal for him. And his heart always beats fast. He said he has an enlarged heart.  Please tell me the association between this and the disease. Do you think in your opinion this incident is assoc. with the disease.  Please advise, I'm scared for him.  He was supposed to go to a heart specialist 5 months ago but missed his appointment.  
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Dear Posted by Andrea Damon on October 21, 1998 at 09:01:41:
Topic Area: Heart Disease
My boyfriend was over last night, and it has been diagnosed that he has Kawasaki Disease. He is 17 yr. old. He told me he felt
nauseated and I told him to get up and I'd get him some pepto bismo. He said he did'nt want to get up cause his heart was
racing. I pulled him up and he fell into my face, then fell backwards and landed. I thought he was just playing till I realized he
did'nt hear me and was just staring straight ahead. This lasted about 9 seconds. I slapped his face to get him to respond. When
he came back he had no recollection of falling or being out of it. He says his heart races, thats normal for him. And his heart
always beats fast. He said he has an enlarged heart. Please tell me the association between this and the disease. Do you think in
your opinion this incident is assoc. with the disease. Please advise, I'm scared for him. He was supposed to go to a heart
specialist 5 months ago but missed his appointment.
First let me say that I would highly recommend your boyfriend calling his doctor today and discussing this episode.  This could be serious and if it happens again I would recommend calling 911.
What follows is some general information concerning Kawasaki disease and it's treatment.
Kawasaki's Disease
In 1967, Tomisaku Kawasaki described a new acute illness that had been affecting infants and young children in Japan. The principal manifestations of this disease were fever, injection of the conjunctivae, a reddening of the lips and oral cavity, reddening of the palms and soles, a polymorphous exanthema of the body trunk, and swelling of the cervical lymph nodes . The fever lasted for approximately 1 to 2 weeks. Desquamation began at the nails during the second week of the illness and then spread to the finger tips and toes. He termed this illness mucocutaneous lymph node syndrome, which has come to be known as Kawasaki disease. A prominent feature of this illness was the 1% to 2% of patients with sudden death due to cardiac failure. These infants were subsequently found at autopsy to have coronary arteritis with associated thrombosis and aneurysm formation. Kawasaki disease is recognized as an important cause of acquired heart disease in children.
Most cases of Kawasaki disease occur in children between the ages of 6 months and 8 years, with a median age of 2 years. Boys are affected more commonly than girls in a ratio of 1.5:1. The annual incidence is highest in children from Japan and Korea where the annual attack rate is 40 to 150 cases per 100,000 children younger than 5 years of age. In contrast, the annual incidence for this age group in other geographic areas, including the United States, is approximately 6 cases per 100,000 children.
Kawasaki disease is believed to be an infectious illness based on its acute and self-limited course, its occurrence in young children, and the geographic clustering of cases. However, no specific infectious agent has been consistently isolated from patients with this disease
The diagnosis of Kawasaki disease requires fever and the presence of at least four of the five principal clinical features. If the patient has fever and fewer than four principal features, then a diagnosis of Kawasaki disease can be made by documenting the presence of coronary artery disease using two-dimensional echocardiography or coronary angiography. Measles, scarlet fever, and Stevens-Johnson syndrome are other acute febrile illnesses that may mimic Kawasaki disease and should be carefully considered in each case to avoid misdiagnosis.

The cardiac manifestations of Kawasaki disease in the acute stages of the illness are diverse and may potentially include pericarditis, myocarditis, endocarditis, inflammation of the conduction system, and coronary artery involvement. Coronary involvement has received the most attention because of its short- and long-term implications. In a large Japanese study, 146 (25%) of 594 patients with acute Kawasaki disease were shown by coronary angiography to have coronary aneurysms. Myocardial infarction is a possible complication of coronary aneurysm that usually occurs in the first year. Many such events are asymptomatic and take place during sleep. Others are symptomatic and heralded by shock, increased irritability, abdominal pain, and chest pain. Mitral regurgitation is relatively uncommon, having been detected in only about 1% of patients over the acute and subacute stages of the disease.
The long-term complications of coronary lesions in Kawasaki disease relate to the persistence of coronary aneurysms, the development of thrombotic occlusion, the progression to ischemic heart disease, and a theoretical risk for premature atherosclerosis. The patients with normal coronary arteries at the outset apparently have an excellent prognosis. Coronary aneurysms detected after the acute stage regress in almost one half of the cases. Small and moderate-sized coronary aneurysms have the highest likelihood of regression. In contrast, giant coronary aneurysms (> 8 mm in diameter) have the lowest rates of regression, the highest risk for stenosis, and the strongest association with myocardial infarction. Systemic artery aneurysms develop in about 2% of patients. They occur most commonly in the axillary and iliac arteries, and rarely produce ischemic manifestations.

Initial treatment with high doses of salicylates shortens the duration of fever and lowers the frequency of coronary aneurysms.
Children suspected of having Kawasaki disease should be examined as soon as possible by two-dimensional echocardiography to evaluate the coronary arteries. A 12 lead electrocardiogram is also recommended to look for evidence of ischemia and any conduction system abnormalities. Initial therapy consists of aspirin 80 to 100 mg per kg per day in four divided doses and intravenous gammaglobulin in a single 2 g dose287. The high doses of aspirin are continued until the child has become afebrile. The aspirin is then given in a reduced single daily dose (3 to 5 mg per kg) for the next 6 to 8 weeks and then discontinued if the child has no coronary abnormalities. The lower doses of aspirin are continued for their antiplatelet effects for an indefinite period in children with coronary lesions. All children with Kawasaki disease should have repeat two-dimensional echocardiography 10 to 14 days after the onset of illness to identify any new coronary lesions.
The American Heart Association has developed guidelines for the long-term management of patients with Kawasaki disease. Any coronary abnormalities are followed by repeat two-dimensional echocardiography at appropriate intervals. Stress testing may be employed in children with known coronary artery disease. A positive stress test warrants consideration of coronary angiography. Children with critical coronary stenoses are treated by percutaneous angioplasty or bypass surgery, depending on the age of the child and extent of the lesions. Whether children who have had Kawasaki disease are predisposed in adulthood to atherosclerosis is an important question for future studies.
I hope you find this information useful.  Information provided in the heart forum is for general purposes only.  Only your physician can provide specific diagnoses and therapies.  Please feel free to write back with additional questions.
If you would like to make an appointment at the Cleveland Clinic Heart Center, please call 1-800-CCF-CARE or inquire online by using the Heart Center website at www.ccf.org/heartcenter.  The Heart Center website contains a directory of the cardiology staff that can be used to select the physician best suited to address your cardiac problem.