262 Part VI / Other Cardiac Conditions
dren <5 years of age in Japan (10), and about one-tenth of this figure in the United States
(116). Coronary artery aneurysms are the major cardiac problem, occurring in 15–25%
of untreated patients and in about 2.3% in those treated with aspirin and immunoglobulin
during the acute phase (117,118). Myocardial involvement is evident in the acute phase
as demonstrated by elevated cardiac troponin I (119) and brain natriuretic peptide (120).
It may or may not accompany coronary disease as shown by discordance between thal-
lium-201 scintigraphy and coronary angiography (121). Cardiogenic shock and death are
most commonly a result of myocardial infarction. Kato et al. (122) reviewed 195 chil-
dren with Kawasaki’s disease who had myocardial infarction. In their study, myocardial
infarction usually occurred within the first year of illness, and 22% of patients died. The
common presenting symptoms were shock, crying, chest pain (which was more common
in older children), abdominal pain, and vomiting. ECG findings included abnormal Q-
waves, usually in leads II, III, and aVF. Rhythm problems were reported as well. Cardiac
enzymes are elevated in most patients (121).
Treatment of the acute episode of Kawasaki’s disease includes intravenous gamma-
globulin and aspirin. Intravenous gamma globulin infusion reduces the risk of coronary
artery aneurysm formation (116,123). The recommended dose is 1–2 g/kg over 10–12 h
(117,124). High-dose aspirin (80–100 mg/kg/d) is given until the fever subsides and is
subsequently reduced to 3–5 mg/kg/d for inhibition of platelet aggregation. This regi-
men is continued for 8 wk if no or transient coronary artery abnormalities are present,
and long term if abnormalities persist. Warfarin may be added to aspirin in the latter
case (117). Some centers add corticosteroids to the treatment in severe unresponsive
cases (125). Treatment of myocardial infarction includes optimization of oxygenation,
correction of metabolic disturbances, analgesia, inotropic support, and treatment of sig-
nificant arrhythmia. Anticoagulation with heparin or warfarin is not used routinely for
children with myocardial infarction (126). Percutaneous transluminal coronary angio-
plasty (PTCA), percutaneous transluminal coronary rotational ablation (PTCRA), and
stent implantation (127) have been used in patients with Kawasaki’s disease. In one
report (127), PTCA resulted in 74% immediate success and 24% restenosis, whereas
PTCRA resulted in 100% immediate success and no restenosis. Stenting was used in a
few patients with 86% immediate success. New aneurysm formation is a worrisome
complication. Surgical revascularization has been used with good results (128).
RHEUMATIC FEVER
Rheumatic fever is precipitated by group A streptococcal pharyngitis (129) and is
rare before 5 yr of age. Its incidence has decreased markedly in the last decades in the
developed world (130), but it is still a major cause for acquired heart disease in devel-
oping countries. Richmond et al. (8) reported an incidence of 375/100,000 school chil-
dren-years among the aborigine population in Western Australia. Rheumatic carditis is
the major cause of morbidity (131). Echocardiographic evidence of valvulitis is present
in most patients (132); however, the rare involvement of the myocardium in the inflam-
matory process may be the cause of acute congestive heart failure and shock. Bitar et al.
(9) reported death from congestive heart failure in 6 of 91 patient with rheumatic fever;
all presented with heart failure. Others report a much better prognosis (133).
In addition to antistreptococcal and heart failure treatment, anti-inflammatory ther-
apy should be administered. Although the superiority of corticosteroids over salicilates
for severe rheumatic carditis has not been proven (24), it is advocated by many (25).
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