Jae Suk Baek

Diagnostic characteristics of supplemental laboratory criteria for incomplete Kawasaki disease in children with complete Kawasaki disease

Purpose In 2004, the American Heart Association (AHA) had published an algorithm for the diagnosis of incomplete Kawasaki disease (KD). The aim of the present study was to investigate characteristics of supplemental laboratory criteria in this algorithm. Methods We retrospectively examined the medical records of 355 patients with KD who were treated with intravenous immunoglobulin (IVIG) during the acute phase of the disease. Laboratory data were obtained before the initial IVIG administration and up to 10 days after fever onset. In 106 patients, laboratory testing was performed more than twice. Results The AHA supplemental laboratory criteria were fulfilled in 90 patients (25.4%), and the frequency of laboratory examination (odds ratio [OR], 1.981; 95% confidence interval [CI], 1.391-2.821; P<0.001) was a significant predictor of it. The fulfillment of AHA supplemental laboratory criteria was significantly associated with refractoriness to the initial IVIG administration (OR, 2.388; 95% CI, 1.182-4.826; P=0.013) and dilatation of coronary arteries (OR, 2.776; 95% CI, 1.519-5.074; P=0.001). Conclusion Repeated laboratory testing increased the rate of fulfillment of the AHA supplemental laboratory criteria in children with KD.

Segmental Analysis of Right Ventricular Longitudinal Deformation in Children before and after Percutaneous Closure of Atrial Septal Defect

Background The aim of study is to identify the dependence of right ventricular (RV) free wall longitudinal deformation on ventricular loading through segmental approach in relatively large number of patients with atrial septal defect (ASD). Methods Patients with ASD (n = 114) and age matched healthy children (n = 60) were echocardiographically examined the day before percutaneous device closure and within 24 hours afterwards. RV free wall deformation parameters, strain (є) and strain rate (SR), were analyzed in the apical (єA, SRA) and basal (єB, SRB) segments. Measured deformation parameters were adjusted for RV size (єAL, SRAL, єBL, SRBL) by multiplying by body surface area indexed RV longitudinal dimension. Regression analyses determined the relationships of these deformation parameters with RV loading parameters that were measured by catheterization. Results єBL and SRBL were not different between pre-closure patients and controls (p = 0.245, p = 0.866), and were decreased post-closure (p = 0.001, p = 0.018). Post-closure єBL was lower than in controls (p = 0.001). Pre-closure єAL and SRAL were higher than in controls (p = 0.001, p < 0.001), but decreased after closure (all p < 0.001). The pulmonary to systemic flow ratio was related to procedural differences of єBL (p = 0.017) and of SRBL (p = 0.019). RV end diastolic pressure was negatively related to post-closure єBL (p = 0.020) and post-closure SRBL (p = 0.012), and the procedural SRBL difference (p = 0.027). Conclusion The longitudinal deformation of the RV basal segment is dependent and its remodeling is also dependent on volume loading in children with ASD.

Prediction of unresponsiveness to second intravenous immunoglobulin treatment in patients with Kawasaki disease refractory to initial treatment

Purpose This study investigated predictors of unresponsiveness to second-line intravenous immunoglobulin (IVIG) treatment for Kawasaki disease (KD). Methods This was a single-center analysis of the medical records of 588 patients with KD who had been admitted to Asan Medical Center between 2006 and 2014. Related clinical and laboratory data were analyzed by univariate and multivariate logistic regression analyses. Results Eighty (13.6%) of the 588 patients with KD were unresponsive to the initial IVIG treatment and received a second dose. For these 80 patients, univariate analysis of the laboratory results obtained before administering the second-line IVIG treatment showed that white blood cell count, neutrophil percent, hemoglobin level, platelet count, serum protein level, albumin level, potassium level, and C-reactive protein level were significant predictors. The addition of methyl prednisolone to the second-line regimen was not associated with treatment response (odds ratio [OR], 0.871; 95% confidence interval [CI], 0.216–3.512; P=0.846). Multivariate analysis revealed serum protein level to be the only predictor of unresponsiveness to the second-line treatment (OR, 0.160; 95% CI, 0.028–0.911; P=0.039). Receiver operating characteristic curve analysis to determine predictors of unresponsiveness to the second dose of IVIG showed a sensitivity of 100% and specificity of 72% at a serum protein cutoff level of <7.15 g/dL. Conclusion The serum protein level of the patient prior to the second dose of IVIG is a significant predictor of unresponsiveness. The addition of methyl prednisolone to the second-line regimen produces no treatment benefit.

Aortic Root Translocation with Arterial Switch for Transposition of the Great Arteries or Double Outlet Right Ventricle with Ventricular Septal Defect and Pulmonary Stenosis

Double outlet right ventricle (DORV) and transposition of the great arteries (TGA) with ventricular septal defect (VSD) and pulmonary stenosis (PS) are complex heart diseases, the treatment of which remains a surgical challenge. The Rastelli procedure is still the most commonly performed treatment. Aortic root translocation including an arterial switch operation is advantageous anatomically since it has a lower possibility of conduit blockage and the left ventricle outflow tract remains straight. This study reports successful aortic root transpositions in two patients, one with DORV with VSD and PS and one with TGA with VSD and PS. Both patients were discharged without postoperative complications.

Comparison of the offset distance of the tricuspid septal leaflet in neonates with Ebstein’s anomaly and neonates with pulmonary atresia with intact ventricular septum

An indexed offset distance of the tricuspid septal leaflet ⩾8 mm/m2 is a quantitative criterion for the diagnosis of Ebsteins anomaly. The purpose of this study was to investigate the validity of this criterion for the discrimination of Ebsteins anomaly from pulmonary atresia with intact ventricular septum in neonatal patients. A total of 122 neonatal patients, 56 with Ebsteins anomaly and 66 with pulmonary atresia with intact ventricular septum, were enrolled. Diagnosis of each anomaly was based on typical morphologic features. Echocardiographic variables, including the offset distance of the tricuspid septal leaflet, were measured via an offline analysis of images recorded before 1 month of age. The offset distance of the tricuspid septal leaflet was indexed by the body surface area, and the indexed offset distances in the Ebsteins anomaly and pulmonary atresia with intact ventricular septum groups were 34.2 mm/m2 (7.1-119.1 mm/m2) and 7.2 mm/m2 (0.0-25.6 mm/m2), respectively. The indexed offset distance was ⩾8 mm/m2 in 29 (43.9%) of the patients with pulmonary atresia with intact ventricular septum; clinical and echocardiographic characteristics were comparable between these 29 patients and the remaining 37 patients with pulmonary atresia with intact ventricular septum. When an indexed offset distance ⩾8 mm/m2 was applied as a cut-off for the diagnosis of Ebsteins anomaly, the sensitivity was 0.963 and the specificity was 0.561. In conclusion, indexed offset distance ⩾8 mm/m2 cannot be used as a cut-off for the diagnosis of complicated Ebsteins anomaly in neonatal patients with pulmonary atresia with intact ventricular septum.

A case demonstrating a percutaneous closure using the amplatzer duct occluder for paravalvular leakage after tricuspid valve replacement.

There has been a dramatic increase in the number and type of procedures performed in the field of cardiac intervention in the past decade. Percutaneous intervention is becoming an increasingly recognized modality for the management of prosthetic paravalvular leakages (PVLs) in severely symptomatic non-surgical candidates. Herein, we report our experience of percutaneous closure using the Amplatzer duct occluder for a PVL in a patient who underwent tricuspid valve replacement.

Cardiac Resynchronization Therapy in Infant with Dilated Cardiomyopathy during Extracorporeal Membrane Oxygenator

Although heart transplantation is a final therapeutic option in pediatric patients with dilated cardiomyopathy (DCMP), the shortage of pediatric heart donors is a major obstacle. In adults with DCMP characterized by cardiac dyssynchrony, cardiac resynchronization therapy (CRT) is known to be an effective treatment option. However, there is a lack of evidence on the effectiveness of CRT in infants with DCMP. Several studies have reported improvement in hemodynamics and cardiac performance following CRT in infants with DCMP. Here, we report CRT in an infant with DCMP during extracorporeal membrane oxygenation with 5 months of follow-up.

Factors associated with reduction of left ventricular mass in children on peritoneal dialysis: LVMI reduction in children on PD

This study aimed to investigate sensitive factors involved in left ventricular mass reduction in children with end‐stage renal disease (ESRD) undergoing peritoneal dialysis.

A comparative study of established z score models for coronary artery diameters in 181 healthy Korean children

Purpose The aim of this study was to investigate the statistical properties of four previously developed pediatric coronary artery z score models in healthy Korean children. Methods The study subjects were 181 healthy Korean children, whose age ranged from 1 month to 15 years. The diameter of each coronary artery was measured using 2-dimensional echocardiography and converted to the z score in the four models (McCrindle, Olivieri, Dallaire, and Japanese model). Descriptive statistical analyses and 1-sample t tests were performed. Results All calculated z scores had P values of ≥0.050 using the Kolmogorov-Smirnov test. The one sample t test showed that the mean z scores did not converge to zero except in 1 model, and the mean right coronary artery (RCA) z score was less than zero in all 4 models. The smaller RCA diameter in this study could be associated with the more distal measuring point used to avoid the conal branch. The percentage of subjects with extreme z score values (≥2.0 and ≥2.5) for the left main coronary artery (LMCA) seems to be higher in the Dallaire (4.9% and 3.3%) and Japanese models (7.1% and 3.8%). Conclusion All 4 models showed statistical feasibility of normal distribution. More precise instructions would be needed for the measurement of the RCA. The higher percentage of extreme z scores for the LMCA is compatible with the basic understanding of anatomic variation in the LMCA.

PS 14-25 B-TYPE NATRIURETIC PEPTIDE IS A GOOD BIOMARKER FOR THE TREATMENT OF HYPERTENSION IN CHILDREN WITH END-STAGE RENAL DISEASE ON PERITONEAL DIALYSIS

Objective: To evaluate the usefulness of serum B-type natriuretic peptide (BNP) level as a clinical biomarker of fluid retention and a target to control body weight for the treatment of hypertension in children on peritoneal dialysis. Design and Method: We included 59 hypertensive patients with end-stage renal disease on peritoneal dialysis who were below 19 years old and have visited Asan Medical Center between February 2005 and February 2016 in this study. We reviewed the changes of body weight (BWt), blood pressure (BP) and serum BNP level between before and after the treatment of hypertension. Data were presented as mean ± standard deviation (median, minimum ∼ maximum). Paired t-test was used to evaluate the changes in BWt, BP and BNP. Linear regression analysis was applied for the correlation between the changes of BNP and BP. Results: We evaluated 56 hypertensive events in 30 patients. Initial findings were as follows: BWt 30.5 ± 22.4 (26.5, 3.0 ∼ 93.5) kg, systolic BP (SBP) 153.3 ± 21.5 (150, 110∼241) mmHg, diastolic BP (DBP) 100.1 ± 22.3, (99.5, 49 ∼ 181) mmHg, BNP 3579.3 ± 6328.9 (1198.5, 305.0 ∼ 22028.0) pg/mL. Follow-up results were as follows: BWt 29.1 ± 21.3 (25.0, 3.12 ∼ 86) kg, SBP 116.4 ± 17.8 (117.5, 82 ∼ 150) mmHg, DBP 73.3 ± 14.2 (75.0, 42.0 ∼ 101.0) mmHg, BNP 63.5 ± 49.2 (60.5, 2.0 ∼ 261.)) pg/mL. SBP (−23.1 ± 13.8, −22.8, −46.9 ∼ 22.5%, P < 0.001), DBP (−24.1 ± 19.2, −24.7, −55.6 ∼ 23.2%, P < 0.001) and BNP (−93.5 ± 8.1, −96.7, −99.9 ∼ −61.0%, P < 0.001) dropped significantly after reduction of BWt (−4.8 ± 4.7, −4.8, −18.7 ∼ 5.6%, P < 0.001). The amount of changes in BNP were significantly correlated with changes in SBP (adjusted R square = 0.221, P < 0.001) and DBP (adjusted R square = 0.203, P < 0.001). Conclusions: We should measure serum BNP in hypertensive patients on peritoneal dialysis and reduce body weight until serum BNP level becomes less than 200 pg/mL if possible.