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.

Background-The objectives of our study were to determine the frequency of supraventricular tachyarrhythmias (SVTAs) among modifications of the Fontan operation and identify risk factors for developing SVTA. Methods and Results-The population consisted of all patients who had any modification of the Fontan operation at the Mayo Clinic between 1985 and 1993. Clinically significant SVTAs were those requiring initiation or change of antiarrhythmic treatment, and they were divided into early SVTAs (Ͻ30 days after the operation) and late SVTAs (Ն30 days after the operation). Clinical histories were reviewed, and health status questionnaires were sent. Four hundred ninety-nine patients had various modifications of the Fontan operation. Frequency of early SVTA was 15%. Risk factors identified by multivariate analysis for early SVTA were AV valve regurgitation, abnormal AV valve, and preoperative SVTA. Frequency of late SVTA was 6% by 1 year, 12% by 3 years, and 17% by 5 years. Risk factors for late SVTA were age at operation (Ͻ3 or Ն10 years) and systemic AV valve replacement. By univariate and multivariate analysis, the type of Fontan operation was not a significant risk factor for late SVTA when all 6 modifications were considered. However, when we analyzed the frequency of late SVTA for the 2 recently used modifications, we found a lower frequency of late SVTA in patients with atriopulmonary connection with lateral tunnel compared with those with total cavopulmonary connection. Conclusions-Postoperative SVTA continues to be a significant problem. Risk factors for SVTA are AV valve regurgitation, abnormal AV valve, preoperative SVTA, and age at operation. Frequency of SVTA does not appear to be related to type of Fontan procedure except for slightly lower frequency in patients with atriopulmonary connection with lateral tunnel compared with those with total cavopulmonary connection. (Circulation. 1998;98:1099-1107.)

Subnormal VO2max and HRmax values were demonstrated at peak exercise. Several perioperative variables were associated with VO2max and O2sat at peak exercise. The presence of a classic Glenn anastomosis was associated with decreased O2sat at peak exercise, suggesting intrapulmonary shunting with the classic Glenn anastomosis.

This study was conducted to assess the use of serum cardiac troponin T (cTnT) level as a noninvasive indicator to diagnose acute myocarditis in children. Noninvasive conventional methods often fail to diagnose myocarditis, A median cTnT level of 0.088 ng/ml (0.04-3.11) was reported in pediatric patients with acute myocarditis in our previous study. Hence, we attempted to determine the cutfoff level of cTnT to diagnose acute myocarditis in children. Pediatric patients with clinically suspected myocarditis or dilated cardiomyopathy (DCM) and a control group were recruited. History, physical examination, elctrocardiogram, chest roentgenogram, echocardiogram, cTnT level, and/or endomyocardial biopsy and clinical course were studied. The gold standard to diagnose acute myocarditis was endomyocardial biopsy proved according to the Dallas criteria and/or recovery from cardiovascular problems within 6 months of follow-up. Forty-three patients were admitted due to cardiovascular problems from primary myocardial dysfunction. Twenty-four patients were diagnosed as acute myocarditis (group 1), 19 were idiopathic chronic DCM (group 2), and 21 patients had moderate to large ventricular septal defect and congestive heart failure (group 3). Median cTnT level was statistically higher in (group 1) compared to groups 2 and 3. Ejection fraction (EF) and left ventricular end diastolic dimension (LVEDd) z score of acute myocarditis were 38.5% (range, 21-67) and 1.3 (range, -0.8-3.0), respectively, which were significantly better than DCM [28.0% (range, 17-45) and 6.0 (range, 2.0-10.0)]. The cutoff point of cTnT level to diagnose acute myocarditis was 0.052 ng/ml (sensitivity, 71%; specificity, 86%). cTnT level, EF, and LVEDd z score did not predict short-term outcomes of patients. In acute myocarditis, cTnT level and EF were significantly higher and LVEDd z score was significantly lower than in DCM. However, the three parameters had no significant effect on outcomes of the patients. Our data show that cardiac a cTnT level of 0.052 ng/ml is an appropriate cutoff point for the diagnosis of acute myocarditis.

Transcatheter closure of VSD in both Pm VSD and DCSA can be achieved by using either of the device. The Amplatzer® VSD device had the advantage of closure of larger defects with immediate less residual shunt but appeared to have a significant number of 3° AVB, which required pacemaker implantation. The Nit Occlud® Lê VSD Coil had the advantage of closure of both types of defects, in particular DCSA VSD with only small residual shunt.

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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.

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.