Several cases of Kawasaki disease (KD) were unresponsive to the initial treatment with intravenous immunoglobulin (IVIG). We retrospectively analyzed all children admitted with KD to determine the occurrence and variables associated with the initial IVIG treatment failure. All patients who fulfilled the criteria for KD and were treated with a single dose (2 g/kg) of IVIG between January 1995 and August 2001 were enrolled. An analysis of the patients who had initially failed to respond to IVIG was performed. A total of 120 patients were enrolled during the study period. There were 68 boys (56.7%). Fourteen patients (11.6%) were found to be unresponsive to initial IVIG treatment. Patients who were anemic (Hb<10 G/DL), had a high neutrophil count (> 75%), a high band count, and low albumin were at risk of failure to respond to a single dose of IVIG. We found no correlation among age, gender, days since starting IVIG treatment, and erythrocyte sedimentation rate (ESR) with failure of the initial IVIG treatment. There were 12 patients (10%) who developed coronary artery aneurysms. The failure of a single dose of IVIG treatment occured in up to 11.6% of our Kawasaki patients. We found that low hemoglobin (<10 G/DL), high neutrophil count (> 75%), high band count, and a low albumin were associated with the requirement for retreatment with a second dose of IVIG.

This study was conducted to assess the use of the serum cardiac troponin T (cTnT) level as a noninvasive indicator to differentiate acute myocarditis and chronic dilated cardiomyopathy in pediatric patients. Myocarditis and dilated cardiomyopathy are clinically difficult to differentiate. Endomyocardial biopsy proved to be quite useful. However, the nature of the procedure--invasiveness, time-consuming, and limited sensitivity--caused some concerns, especially in pediatric patients. Hence, we attempted to find an alternative method that could give a prompt diagnosis of acute myocarditis. Twenty cases with clinically suspected myocarditis or dilated cardiomyopathy and a control group of 21 cases with moderate left-to-right shunt and congestive heart failure were recruited. History, physical examination, electrocardiogram, chest roentgenogram, echocardiogram, cTnT, creatine kinase MB isoenzyme (CK-MB mass), and/or endomyocardial biopsy were compared. The gold standard used to diagnose myocarditis is endomyocardial biopsy (Dallas criteria) and/or recovery from cardiovascular problems within 6 months of follow-up. Ten patients were diagnosed as having myocarditis (group 1) and 10 with chronic dilated cardiomyopathy (group 2). The control group of 21 cases was designated as group 3. The median serum cTnT levels were 0.088 (0.04-3.11), 0.010 (0.010-0.990), and 0.010 (0.010-0.550) ng/ml in groups 1, 2, and 3, respectively. The mean CK-MB mass level for groups, 1, 2, and 3 were 18.35 (7.14-70.00), 4.80 (0.54-108.00), and 2.26 (0.95-7.06) ng/ml. The study showed that both the cTnT and CK-MB mass levels were significantly higher in group 1 than either group 2 or group 3. Histopathology was studied in 9 cases. In 2 of 5 cases and in all 4 cases in group 1 and group 2 histopathology was pathologically proved. Levels of cTnT and CK-MB were significantly higher for myocarditis than for dilated cardiomyopathy and left-to-right shunt with CHF. Further study is needed to assess the optimum cTnT level for differentiating both conditions.

The location, size, and rim of an atrial septal defect (ASD) are major determining factors for transcatheter closure. We compared the measurements of ASD size and the characteristics of atrial septal rim using cardiac magnetic resonance imaging (MRI) with those obtained using transesophageal echocardiography (TEE). Patients with an ASD that met established criteria were selected for evaluation by cardiac MRI and TEE. There were 66 patients who underwent both TEE and cardiac MRI. Bland-Altman comparative analysis was performed to demonstrate agreement between measurement of ASD by MRI and balloon sizing compared to measurement of ASD by TEE and balloon sizing. Twelve patients were excluded from transcatheter closure of ASD. TEE did not demonstrate an adequate measurement of the posterior inferior rim in 10 of 66 patients. Fifty-four patients underwent transcatheter closure using the Amplatzer septal occluder. Of these, 52 patients had successful closure (ASD measurements of 25.9 +/- 5.6 mm using MRI and 21.9 +/- 5.8 mm using TEE). The median device size was 28 mm (range, 11-38). Patients who had successful closure had a significantly smaller major axis of ASD and larger posterior inferior rim compared to those of patients who were excluded from the closure procedure. Cardiac MRI showed a better correlation of ASD diameter measurement to balloon sizing compared to TEE. We believe that the most important predictive factor for successful transcatheter closure of an ASD is an adequate posterior inferior rim, which is best visualized using cardiac MRI.

Our study reports the results of a comparison of closure of atrial septal defect (ASD) surgically with transcatheter closure using the Amplatzer septal occluder. Patients having an ASD and a surgical closure or transcatheter closure between January 1999 and July 2000 were selected. There were 103 patients who had ASD. All 64 patients in group 1 (surgery) had a successful operation, with only 2 patients with a mild residual shunt. There were 39 patients enrolled for transcatheter closure of the ASD (group 2). Four patients were excluded initially. The median age for group 1 was 25 years (range 2.3-64 years) compared to 11.7 years (range 2-69 years) in group 2 (p = 0.035). In group 1, the mean ASD diameter measured was 28.4 +/- 10.2 mm compared to 23.4 +/- 5.7 mm in group 2 (p = 0.003). In 29 patients, devices were deployed with sizes from 10 mm to 30 mm (median 24 mm). Three patients were excluded because a larger device (>30 mm) was not available and devices were not successfully deployed in another 3 patients. One patient had a device embolized into the right ventricle (surgical removal and closure of the ASD). Complications occurred in 13 patients in group 1 and 4 patients in group 2. Complete occlusion occurred in 27 of 28 group 2 patients (96.4%) during the follow-up period (10.2 +/- 5.4 months). The Amplatzer septal occluder is a new device for closure of different-sized ASDs. The intermediate-term follow-up demonstrated excellent closure results. The benefit for each patient was demonstrated in less morbidity and less time spent in the hospital.

The objective of this study was to assess the effect of coenzyme Q10 (CoQ10) as supplementation to conventional antifailure drugs on quality of life and cardiac function in children with chronic heart failure due to dilated cardiomyopathy (DCM). The study was an open-label prospective study performed in two of the largest pediatric centers in Thailand from August 2000 to June 2003. A total of 15 patients with idiopathic chronic DCM were included, with the median age of 4.4 years (range, 0.6-16.3). Presenting symptoms were congestive heart failure in 12 cases (80%), cardiogenic shock in 2 cases (13.3%), and cardiac arrhythmia in 1 case (6.7%). Sixty-one percent of patients were in the New York Heart Association functional class 2 (NYHA 2), 31% in NYHA 3, and 8% in NYHA 4. Cardiothoracic ratio from chest x-ray, left ventricular ejection fraction, and left ventricular end diastolic dimension in echocardiogram were 0.62 (range, 0.55-0.78), 30% (range, 20-40), and 5.2 cm (range, 3.8-6.5), respectively. CoQ10 was given at a dosage of 3.1 ? 0.6 mg/kg/day for 9 months as a supplementation to a fixed amount of conventional antifailure drugs throughout the study. At follow-up periods of 1, 3, 6, and 9 months, NYHA functional class was significantly improved, as was CT ratio and QRS duration at 3 and 9 months follow-up with CoQ10 when compared to the baseline and post-discontinuation of CoQ10 at 9 months (range, 4.8-10.8). However, when multiple comparisons were taken into consideration, there was no statistical significant improvement. In addition to the conventional antifailure drugs, CoQ10 may improve NYHA class and CT ratio and shorten ventricular depolarization in children with chronic idiopathic DCM.

Objectives. To determine in-hospital mortality and complications of cardiac surgery in pediatric patients and identify predictors of hospital mortality. Methods. Records of pediatric patients who had undergone cardiac surgery in 2005 were reviewed retrospectively. The risk adjustment for congenital heart surgery (RACHS-1) method, the Aristotle basic complexity score (ABC score), and the Society of Thoracic Surgeons and the European Association for Cardiothoracic Surgery Mortality score (STS-EACTS score) were used as measures. Potential predictors were analyzed by risk analysis. Results. 230 pediatric patients had undergone congenital cardiac surgery. Overall, the mortality discharge was 6.1%. From the ROC curve of the RACHS-1, the ABC level, and the STS-EACTS categories, the validities were determined to be 0.78, 0.74, and 0.67, respectively. Mortality risks were found at the high complexity levels of the three tools, bypass time >85 min, and cross clamp time >60 min. Common morbidities were postoperative pyrexia, bleeding, and pleural effusion. Conclusions. Overall mortality and morbidities were 6.1%. The RACHS-1 method, ABC score, and STS-EACTS score were helpful for risk stratification.

Transient interruption of accessory pathway (AP) conduction is often encountered during creation of RF lesions, with return of conduction after seconds to weeks. Maximum catheter tip temperature (Tmax) has not been shown to be a good predictor of successful RF ablation. However, other indices related to catheter tip temperature (T) may predict permanent AP interruption. Ninety-one successful RF applications in 58 patients (mean age 11.9 +/- 5.5 years, 38 WPW syndrome, 18 concealed AP, 2 both) were reviewed retrospectively. Forty-two RF applications were transiently successful, with a median time of AP conduction recurrence of 120 seconds (sec; range, 1 sec to > 1 day). This group was compared with 49 permanently successful RF applications. T was measured and controlled using the Medtronic Atakr system (San Jose, CA, USA). RF lesion duration, power output, Tmax and time to Tmax (tmax) were not significantly different between the two groups. By univariate analysis, each of the following indices was able to discriminate between the transient and permanent lesions, and highly correlated with one another, T at the moment of AP interruption (Tsucc; transient 55.0 +/- 7.9 degrees C vs permanent 49.8 +/- 7.7 degrees C, P = 0.0025), time to success (tsucc; transient 4.0 +/- 3.0 sec vs permanent 1.8 +/- 1.3 sec, P = 0.0001), ratio of Tsucc/Tmax (transient 0.76 +/- 0.23 vs permanent 0.57 +/- 0.27, P = 0.0007) and ratio of tsucc/tmax (transient 0.91 +/- 0.69 vs permanent 0.41 +/- 0.41, P = 0.0001). By logistic regression analysis, no single variable or combination of variables was superior to tsucc for prediction of outcome, with a breakpoint of 2.3 seconds having a sensitivity of 74% and a specificity of 65%. During temperature controlled RF application, indices of time and temperature were well-correlated with permanent elimination of AP conduction. Time to interruption of AP conduction< 2.3 seconds after the onset of RF application was predictive of the permanence of successful RF applications. Known relations between RF lesion volume and catheter tip temperature suggest that early conduction block may be an indicator of anatomical proximity of the catheter tip and the AP. These data suggest that, in conjunction with electrogram criteria, selection criteria for optimal sites for RF, application may continue to be refined after the onset of RF application, and support the practice of terminating RF application if AP conduction is not rapidly interrupted.

The objectives of this study were to evaluate the therapy protocol of gamma globulin (GG) re-treatment without steroids. Patients. From Jun. 1994 to Jun. 2001, 220 typical patients were enrolled within 8 days of illness. They had no coronary artery dilatation at admission and before GG treatment. Their risks were evaluated by the Iwasa's risk score system. Of 220 patients, 66% were selected as high-risk patients and 80% were treated with GG. Initial dose of GG was single 2g/kg for the high-risk patients and 1g/kg for the low-risk patients with prolonged fever, 49 patients had GG re-treatment. Maximum dose of GG was 8g/kg and mean dose of GG was 1.8g/kg per patient. Dose of re-treatment after Sep. 1996 was decided by WBC and CRP before and after GG treatment. Results. Of 220 patients, 13 patients had coronary artery lesions (CAL, over 3 mm in diameter) in acute stage and 3 patients had CAL at 30th days of illness, which were all transient dilatation. In high-risk female patients, 51 patients treated with GG had no CAL. In high-risk male patients from Jun. 1994 to Jun. 1999, 60 patients were treated with sulfonated GG (Sulfo-GG) or GG prepared by propylene glycol (PEG-GG). And CAL (8/31) in Sulfo-GG group was significantly higher than CAL (1/29) in PEG-GG group (pϭ0.017). In high-risk male patients after Jul. 1999, when we did not use Sulfo-GG, 1/22 had CAL. The other 3 CAL patients, whose CAL were under 3.1 mm in diameter, were high-risk patient without GG, low-risk patient without GG and high-risk patient with GG.