Long-term consequences of Kawasaki disease. A 10- to 21-year follow-up study of 594 patients.

BACKGROUND: The long-term consequences of the cardiovascular sequelae in Kawasaki disease remain uncertain. METHODS AND RESULTS: We identified 594 consecutive children with acute Kawasaki disease between 1973 and 1983, and this cohort was followed up for 10 to 21 years (mean, 13.6 years). In all patients, we evaluated coronary lesions by coronary angiography just after the acute stage. One hundred and forty-six patients (24.6%) were diagnosed as having coronary aneurysms. A second angiogram was performed 1 to 2 years later in all 146 patients who previously had coronary aneurysms, which demonstrated that 72 (49.3%) of these 146 had regression in the coronary aneurysm. A third angiogram was performed for 62 patients, a fourth for 29, and a fifth for 17. By 10 to 21 years after the onset of the illness, stenosis in the coronary aneurysm had developed in 28 patients. Myocardial infarction occurred in 11 patients, 5 of whom died. In the 26 patients with giant coronary aneurysms, stenotic lesions developed in 12, and no regression occurred. The 448 patients with normal findings at the first angiogram subsequently never developed any abnormal cardiac findings. Systemic artery aneurysms developed in 13 patients (2.2%), and valvular heart disease appeared in 7 (1.2%). CONCLUSIONS: The incidence of coronary aneurysm in acute Kawasaki disease was 25%, 55% of which showed regression. During follow-up, ischemic heart disease developed in 4.7% and myocardial infarction in 1.9%. Death occurred in 0.8%.

Aortic valve replacement for aortic regurgitation due to Kawasaki disease. Report of two cases.

Two cases of severe aortic regurgitation due to Kawasaki disease are reported. Both patients were diagnosed as having Kawasaki disease in their infancy, and were followed up by a pediatrician. Aortic regurgitation was detected 18 months in one case and 36 days in the other case after onset of the illness. With the passage of time, the aortic regurgitation increased and aortic valve replacement was scheduled in both patient at the age of 13. On admission, two-dimensional echocardiography showed thickening of the aortic cusps, and severe aortic regurgitation was detected by color flow Doppler studies. Successful aortic valve replacement was performed, and histological studies of the cusps showed sequelae of valvulitis. In conclusion, aortic regurgitation is a rare complication of Kawasaki disease, and the aortic valve function, especially occurrence of aortic regurgitation, should be carefully observed in patients with a past history of Kawasaki disease.

Fetal arrhythmias: intrauterine diagnosis, treatment and prognosis.

Fetal echocardiography can provide useful information for the evaluation of fetal arrhythmias. Between 1980 and 1993, 44 fetuses with arrhythmias were diagnosed in utero at 12 and 40 weeks of gestation in Kurume University Hospital. Fetal bradycardia, tachycardia and ectopic beats were revealed in 17, seven and 20 fetuses, respectively, and their clinical features and prognosis were evaluated. In the 17 fetuses with bradycardia, eight were associated with congenital heart defect, and six of these developed to fetal hydrops. Of the 17 fetuses, four died in utero, one was terminated, and six died after birth. The other six cases survived. Three of these had a pacemaker implanted after birth. In the seven fetuses with tachycardia, transplacental anti-arrhythmic drugs were administered in five cases and conversion of the arrhythmia was achieved in four. None of the cases was associated with any congenital heart defect, and none died. Three infants had paroxysmal tachycardia postnatally. In the 20 fetuses with ectopic beats, arrhythmia was observed postnatally in 10, but all of these were resolved within 3 months after birth. Fetal bradycardias carried a poor prognosis in most cases and further studies are required to establish effective treatment. Some cases of fetal tachycardia developed recurrent tachycardia postnatally. Close follow-up of the newborn is therefore necessary.

Effect of low dosage inhaled nitric oxide on pulmonary hypertension in congenital heart disease.

An evaluation of the pulmonary vascular resistance and the reversibility of pulmonary vascular reaction in children with congenital heart disease is essential for determining the surgical indication and for assessing the long-term prognosis. We report the clinical efficacy of low dosage inhaled nitric oxide and investigate the relationship between its effect and hemodynamic parameters in 18 patients with congenital heart disease. The patients were divided into 3 Groups; Group 1 consisting of 3 patients with Eisenmenger syndrome, Group 2 of 10 patients whose mean pulmonary artery pressure was more than 30 mmHg, and Group 3 consisting of 5 patients whose mean pulmonary artery pressure was less than 30 mmHg. High concentration (90%) oxygen, and also normal oxygen (21%) containing 10 parts per million nitric oxide were administered by cardiac catheterization. In Group 1, both the 90% oxygen and the normal oxygen with nitric oxide showed no affect on the hemodynamical variables. In Group 2, the pulmonary artery pressure and the pulmonary vascular resistance both significantly decreased with the 90% oxygen, and with the nitric oxide inhalation, but these decreases were not found in Group 3. In the 15 patients (of Groups 2 and 3 combined) who were considered to have reversible pulmonary vascular changes, significant correlations were found between the baseline pulmonary artery pressure and the magnitude of pulmonary vasodilation. No clinical evidence of toxicity was seen with the administration of the inhaled nitric oxide. These data suggested that inhaled nitric oxide, even in a low dosage, was a potent and selective pulmonary vasodilator in patients with congenital heart disease complicated with pulmonary hypertension. Since a positive correlation was found between the baseline pulmonary artery pressure and the magnitude of pulmonary vasodilation, this examination demonstrated potential efficacy for objective analysis in patients with pulmonary hypertension.