Steroid pulse therapy was useful to reduce the fever duration and medical costs for patients with Kawasaki disease. Steroid pulse therapy and additional IVIG treatment were not significantly different in terms of preventing the development of coronary artery aneurysm.

We assessed the usefulness of transthoracic Doppler-derived indexes obtained in the proximal pulmonary artery (PA) branch for estimating pulmonary vascular resistance (PVR) in 45 children with congenital heart disease (CHD) and 23 normal control subjects. The acceleration time, inflection time (InT), deceleration index, and peak velocity, which were measured from the systolic PA flow velocity curve obtained at the sites of the main PA, and right and left PA, were compared with the PVR in patients with CHD. In addition, changes in either Doppler-derived indexes or PVR during 100% oxygen administration were compared in 22 patients showing a baseline PVR >or=4.6 U/m(2) (high PVR). The heart-rate-corrected InT (InTc) values obtained in the left PA in the high PVR group were significantly lower than those in the main PA (4.7 +/- 1.5 vs. 7.5 +/- 3.0; p < 0.001). The InTc obtained from the left PA separated patients with high and low PVR (4.7 +/- 1.4 vs. 9.9 +/- 2.4; p < 0.001) and no significant differences in InTc were found between the low PVR and the control groups. An increase in InTc to >6 during 100% oxygen administration for the high PVR group indicated good PA reactivity with a sensitivity of 93%, specificity of 100%, and agreement of 95% (kappa = 0.83). Moreover, this InTc index correlated inversely with PVR (r = -0.80). In conclusion, our method can noninvasively separate high and low PVR and assess the PA reactivity for high PVR in children with CHD.

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ows compared with findings on coronary angiography (CAG), MRI, and intravascular ultrasound. RESULTS: Linear shadows were detected in 11 CALs on high-resolution 2DE in 9 patients with Kawasaki disease. The outer diameters of CALs on 2DE (7.0 Ϯ 2.1 mm) were larger than those on CAG (4.4 Ϯ 1.6 mm), whereas the inner diameters between linear shadows (3.9 Ϯ 1.6 mm) were almost equal to the diameters of CALs on CAG. There was a statistically significant positive correlation (y ϭ 0.99x Ϫ 0.10; r 2 ϭ 0.77) between the diameters of CALs on CAG and the inner diameters between linear shadows on 2DE. A thickened intima was revealed in the same regions that showed linear shadows on 2DE, in 7 of 11 lesions on MRI, and in all 4 lesions on which intravascular ultrasound was performed. In 3 patients who had been followed up over 3 years, linear shadows inside CALs on 2DE persisted, and the diameter between linear shadows was almost consistent with the diameter of CALs on CAG. CONCLUSIONS: These results suggest that linear shadows inside CALs on 2DE would reflect the existence of a thickened intima. We expected that following up the changes of linear shadows inside CALs was useful for noninvasive evaluation of coronary arterial remodeling such as intimal hypertrophy or stenotic change. LONG-TERM FOLLOW-UP RESULTS OF PERCUTANEOUS CATHETER INTERVENTION FOR CORONARY ARTERY LESIONS AFTER METHODS:Patients who developed coronary stenotic lesions caused by KD and were treated with PCI were investigated. Restenosis or obstruction was diagnosed when the stenosis was Ն75% according to coronary angiography or ischemic change was observed by myocardial perfusion imaging. RESULTS: A total of 55 stenotic lesions were reported in 49 patients in 5 institutions. The types of PCI included percutaneous transluminal coronary angioplasty (n ϭ 22), stent implantation (n ϭ 7), percutaneous transluminal coronary rotational ablation (PTCRA) (n ϭ 22), and combination of PTCRA with stent implantation (n ϭ 4). Median age at PCI was 14.5 years, and the median follow-up period in the PCI group was 6.3 years. of 55 stenotic lesions in the PCI group, 52 (95%) were dilated successfully by PCI. Immediate complications in the PCI group included neoaneurysm in 5 patients, transient bradycardia in 3 patients, and atrial fibrillation in 1 patient. Treatment for restenosis in the PCI group included re-PCI in 3 patients, coronary artery bypass grafting in 6 patients, and heart transplantation in 1 patient. No patient in the PCI group died. There was no difference in effectiveness among the 3 PCI devices (percutaneous transluminal coronary angioplasty versus stent implantation versus PTCRA: log-rank test, P ϭ .3). CONCLUSIONS: PCI for KD can be accomplished and can be effective in the long-term.

We describe the unusual case of a patient with an antenatal aorto-left ventricular tunnel (ALVT) diagnosis and severe left ventricular (LV) dysfunction who underwent successful repair immediately after birth. To the best of our knowledge, no such case has been reported in the English literature. Our case demonstrated that neonates at the worst end of the ALVT spectrum can survive and achieve normalization of LV function through a timely and multidisciplinary approach.