Jarupim Soongswang

Immunoglobulin failure and retreatment in Kawasaki disease

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.

Cardiac Troponin T: A Marker in the Diagnosis of Acute Myocarditis in Children

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.

Predictors of successful transcatheter closure of atrial septal defect by cardiac magnetic resonance imaging

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.

Comparison of Atrial Septal Defect Closure Using Amplatzer Septal Occluder with Surgery

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.

Cardiac troponin T: its role in the diagnosis of clinically suspected acute myocarditis and chronic dilated cardiomyopathy in children.

AbstractThis 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 results of transcatheter closure of VSD using Amplatzer® device and Nit Occlud® Lê coil.

Objective: We retrospectively reviewed the result of patients who underwent transcatheter closure of ventricular septal defect (VSD) using Amplatzer® Perimembranous or Amplatzer® muscular VSD device (the Amplatzer® group) and Nit Occlud® Lê VSD Coil (the Pfm group). Background: Perimembranous type (PmVSD) and doubly committed subarterial (DCSA) VSD were the major isolated congenital heart defects in Thai children. Transcatheter device closure technique for both types of VSD has emerged as an alternative treatment to surgery. Methods: Retrospectively, data was reviewed between 2003 and 2009. Results: 116 patients were enrolled. Device or coil was successfully implanted in 94%. Complete closure at 24 hr is slightly higher in the Amplatzer® group. The average size of VSD in the Amplatzer® group was larger than the Pfm group (P = 0.001). The Pfm coil was primarily deployed in DCSA VSD when compared with the Amplatzer® group (P < 0.01). At 6 months follow‐up the residual shunt was comparable (P = 0.054). There was only one transient AV block (AVB) in the Pfm group and 5 AVB in the Amplatzer® group. Four pacemakers were placed in the Amplatzer® group. Conclusions: 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.

Transcatheter Closure of Atrial Septal Defects in Children, Middle-Aged Adults, and Older Adults: Failure Rates, Early Complications; and Balloon Sizing Effects

Objectives. To compare the failure ratio and inhospital complications across three age groups of patients and to investigate the effects of balloon sizing on the success and the device diameter. Methods. This retrospective review was of 665 patients who had been listed for transcatheter-based closure of ASD between 1999 and 2010. The patients were divided into three age groups: children (<18 years; n = 183), adults (18–50 years; n = 337), and older adults (>50 years; n = 145). Procedural outcomes and early complications were reviewed. Use of balloon sizing was explored for its benefits. Results. Overall, failure of closure was 6.6% (n = 44). Use of balloon sizing tended to lead to a smaller device/defect ratio that was comparable to procedures without balloon sizing, though it did not predict the success rate (OR 1.4, 95% CI 0.7–2.3). Seven patients reported device embolization (1%). No mortalities were noted. In-hospital complications were 3.4%, with common complications, being vascular complications (1.4%) and cardiac arrhythmia (1.1%). No differences in failure rate or events were found among the three groups. Conclusion. Transcather closure of ASD is feasible and safe, regardless of the patients age. A low rate of early complications was noted. Balloon sizing does not aggravate an oversizing of the device, but does not predict success.

Surveillance of Pediatric Cardiac Surgical Outcome Using Risk Stratifications at a Tertiary Care Center in Thailand

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.

The Effect of Coenzyme Q10 on Idiopathic Chronic Dilated Cardiomyopathy in Children

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.

Fetal Long QT Syndrome Manifested as Atrioventricular Block and Ventricular Tachycardia: A Case Report and a Review of the Literature

Fetal onset of congenital long QT syndrome (LQTS) is a rare manifestation, and prenatal diagnosis is difficult. This report describes a boy who presented with both atrioventricular (AV) block and ventricular tachycardia during the antenatal period. The early postnatal electrocardiogram showed prolongation of the QT interval and AV block, subsequently leading to a polymorphic ventricular tachycardia torsade de pointes. This unique feature of congenital LQTS has a poor outcome, but the boy was successfully treated with beta-blockers and implantation of an automated cardioverter-defibrillator. The intrauterine manifestation of fetal AV block and ventricular tachycardia should raise a high suspicion of congenital LQTS, and the strong association with a malignant clinical course should warrant special evaluation. The literature on the prenatal diagnosis, fetal therapy, and neonatal outcome of this condition also are reviewed.