Paediatric heart transplantation recipients ≥7 years of age receiving donors with pre-existing coronary atherosclerosis showed progressive coronary artery disease.

BACKGROUND: This study aimed to determine the effect of donor-transmitted atherosclerosis on the late aggravation of cardiac allograft vasculopathy in paediatric heart recipients aged ≥7 years. METHODS: In total, 48 patients were included and 23 had donor-transmitted atherosclerosis (baseline maximal intimal thickness of >0.5 mm on intravascular ultrasonography). Logistic regression analyses were performed to identify risk factors for donor-transmitted atherosclerosis. Rates of survival free from the late aggravation of cardiac allograft vasculopathy (new or worsening cardiac allograft vasculopathy on following angiograms, starting 1 year after transplantation) in each patient group were estimated using the Kaplan-Meier method and compared using the log-rank test. The effect of the results of intravascular ultrasonography at 1 year after transplantation on the late aggravation of cardiac allograft vasculopathy, correcting for possible covariates including donor-transmitted atherosclerosis, was examined using the Cox proportional hazards model. RESULTS: The mean follow-up duration after transplantation was 5.97 ± 3.58 years. The log-rank test showed that patients with donor-transmitted atherosclerosis had worse survival outcomes than those without (p = 0.008). Per the multivariate model considering the difference of maximal intimal thickness between baseline and 1 year following transplantation (hazard ratio, 22.985; 95% confidence interval, 1.948-271.250; p = 0.013), donor-transmitted atherosclerosis was a significant covariate (hazard ratio, 4.013; 95% confidence interval, 1.047-15.376; p = 0.043). CONCLUSION: Paediatric heart transplantation recipients with donor-transmitted atherosclerosis aged ≥7 years had worse late cardiac allograft vasculopathy aggravation-free survival outcomes.

Mid-term outcomes of the Pulsta transcatheter pulmonary valve for the native right ventricular outflow tract.

OBJECTIVES: The aim of this study is to present the mid-term outcomes of Pulsta valve. BACKGROUND: The Pulsta valve is a Self-expandable knitted nitinol-wire stent mounted with a treated tri-leaflet α-Gal-free porcine pericardial valve for percutaneous pulmonary valve implantation (PPVI) in patients with native right ventricular outflow tract (RVOT) lesions. METHODS: A multi-center clinical trial using Pulsta valve® was designed for patients with severe pulmonary regurgitation (PR) in the native RVOT in multiple centers in South Korea and 25 patients were enrolled. Before PPVI, severe PR (mean PR fraction: 45.5 ± 6.9%) and enlarged RV volume (mean indexed RV end-diastolic volume; 169.7 ± 13.0 ml/m2 ) was present. The mean age was 21.6 ± 6.6 years old. RESULTS: All patients were successfully implanted with 26, 28, or 32 mm diameter of Pulsta valve loaded on the 18 or 20 French delivery catheters. At 6 months follow up, indexed RV end-diastolic volume was decreased to 126.9 ± 16.9 ml/m2 . At mean 33.1 ± 14.3 months follow-up, the mean value of mean pressure gradient in Pulsta valve was 6.5 ± 3.0 mmhg without significant PR. There was no serious device-related adverse event. CONCLUSIONS: A multi-center clinical trial was completed successfully with planned Pulsta valve implantation and demonstrated good mid-term effectiveness without device-related serious adverse events.

Procedural Predictors and Outcomes of Percutaneous Secundum Atrial Septal Defect Closure in Children Aged <6 Years.

BACKGROUND: Percutaneous atrial septal defect (ASD) closure is the treatment of choice for patients with a suitable ASD anatomy; however, the procedural characteristics and outcomes in children aged <6 years are unclear. The feasibility and safety of percutaneous ASD closure in children aged <6 years was evaluated and the predictors of procedural failure and challenging cases were identified.Methods and Results:Patients from a single center between 2006 and 2018 (n=407) were retrospectively evaluated. There were 265 (65.1%) female patients. The median age at the time of the procedure and ASD size were 3.4 (0.9-5.9) years and 13.3 (3.8-27.0) mm, respectively. Medical records and echocardiographic images were analyzed. A challenging case was indicated by the use of non-conventional techniques. The procedure was completed in 399 patients (98.0%). Post-procedural acute complications occurred in 5 patients, including 1 with device embolization. Two patients underwent surgical device removal. During the follow up (30.3 [3.6-140.8] months), aggravated mitral regurgitation occurred in 5 patients. A multivariate logistic regression revealed large-sized ASD as a predictor of procedural failure (odds ratio=1.828, 95% confidence interval: 1.139-2.934, P=0.012) and challenging cases (odds ratio=1.371, 95% confidence interval: 1.180-1.593, P<0.001). CONCLUSIONS: Percutaneous ASD closure is feasible and safe in children aged <6 years; however, patients with large-sized ASD are at high risk of procedural failure and becoming a challenging case.

Predictive Parameters of Decreased Left Ventricular Global Longitudinal Strain at 1 Month After Pediatric Heart Transplantation.

Previous reports indicate that the decreased left ventricular global longitudinal strain (LVGLS) seen in the early postoperative period of pediatric heart transplant patients generally recovers over the course of 1-2 years. In this study, we investigate the predictive capacity of preoperative parameters on the LVGLS decline seen at 1 month post transplant. Forty-six transplant subjects with 2D echocardiographic images sufficient for speckle tracking echocardiography were enrolled. We excluded patients diagnosed with cardiac allograft vasculopathy or with an episode of rejection 1 month before or after their echocardiographic examinations. The mean LVGLS was significantly reduced at 1 month when compared to 1 year following transplant (- 15.5% vs. - 19.4%, respectively, p < 0.001). The predictors of LVGLS that decline at 1 month were the LV mass z-score [odds ratio (OR) 1.452; 95% confidence interval (CI) 1.007-2.095, p = 0.046], recipient age (OR 1.124; 95% CI 1.015-1.245, p = 0.025), and donor age (OR 1.081; 95% CI 1.028-1.136, p = 0.002) in the univariate logistic regression analyses. Although multivariate analysis yielded no significant predictors, higher LV mass z-scores showed a trend associated with the decline of LVGLS (p = 0.087). The donor/recipient weight ratio was associated with the LV mass z-score (R2 = 0.412, p < 0.001).

Factors associated with reduction of left ventricular mass in children on peritoneal dialysis.

AIM: This study aimed to investigate sensitive factors involved in left ventricular mass reduction in children with end-stage renal disease (ESRD) undergoing peritoneal dialysis. METHODS: Thirty-five subjects on peritoneal dialysis were enrolled. Two successive echocardiographic and clinical data for each subject were obtained. Blood pressure and left ventricular mass index (LVMI) were indexed through a division with the normal 95th percentile value. Differences in numeric data between two datasets were calculated. RESULTS: The mean age was 12.9 ± 4.6 years. Predictors of left ventricular hypertrophy and its persistence were systolic blood pressure index (P = 0.019 and P = 0.046) and E' velocity (P = 0.035 and P = 0.031) in univariate analysis. However, differences in these predictors between the datasets were not related to the change in indexed LVMI. Reduction in indexed LVMI was correlated to a reduction of indexed left atrial volume (R = 0.638, P = 0.001), trans-mitral A velocity (R = 0.443, P = 0.011), and serum blood urea nitrogen level (R = 0.372, P = 0.028) and an elevation of haemoglobin level (R = -0.374, P = 0.027). CONCLUSION: The extent of circulating volume expansion is potentially the main predictive factor for change of LVMI, because the volume dependent diastolic functional variables correlate to the change of LVMI. Further study with a large number of ESRD children including a group under fluid volume control is needed to investigate the role of volume expansion on the change of LVMI.

Clinical Characteristics of Transplant-associated Encephalopathy in Children.

We aimed to analyze characteristics of encephalopathy after both hematopoietic stem cell and solid organ pediatric transplantation. We retrospectively reviewed medical records of 662 pediatric transplant recipients (201 with liver transplantation [LT], 55 with heart transplantation [HT], and 67 with kidney transplantation [KT], 339 with allogeneic hematopoietic stem cell transplantation [HSCT]) who received their graft organs at Asan Medical Center between January 2000 and July 2014. Of the 662 patients, 50 (7.6%) experienced encephalopathy after transplantation. The incidence of encephalopathy was significantly different according to the type of organ transplant: LT, 16/201 (8.0%), HT, 13/55 (23.6%), KT, 5/67 (7.5%), and HSCT, 16/339 (4.7%) (P < 0.001). Drug-induced encephalopathy (n = 14) was the most common encephalopathy for all transplant types, but particularly after HSCT. Hypertensive encephalopathy was the most common after KT and HT, whereas metabolic encephalopathy was the most common after LT. The median time to encephalopathy onset also differed according to the transplant type: 5 days after KT (range 0-491 days), 10 days after HT (1-296 days), 49.5 days after HSCT (9-1,405 days), and 39 days after LT (1-1,092 days) (P = 0.018). The mortality rate among patients with encephalopathy was 42.0% (n = 21/50). Only 5 patients died of neurologic complications. Transplant-associated encephalopathy presented different characteristics according to the type of transplant. Specialized diagnostic approach for neurologic complications specific to the type of transplant may improve survival and quality of life in children after transplantation.

Nationwide survey of coronary aneurysms with diameter >6 mm in Kawasaki disease in Korea.

BACKGROUND: We investigated the course of coronary aneurysm with diameter > 6 mm in Kawasaki disease (KD), as well as related therapeutic trends and prognosis in Korea. METHODS: A nationwide questionnaire survey was carried out in 77 hospitals, to investigate, retrospectively, patients with KD who had coronary aneurysms with a diameter > 6 mm between 1990 and 2011. RESULTS: The median age of onset was 3 years (range, 2 months-16 years) in a total of 239 patients. During the acute stage of KD, most patients received i.v. immunoglobulins and aspirin. In addition, 27 patients received steroid therapy. In the current study, the mean coronary aneurysm size was 8.7 ± 3.2 mm (range, 6-21 mm). Twenty-two patients underwent interventional catheterization. Procedures included percutaneous transluminal coronary balloon angioplasty (n = 10), stent placement (n = 9), and percutaneous transluminal coronary rotational ablation (n = 3). Fourteen patients underwent coronary artery bypass graft surgery. Of the 239 patients who had coronary aneurysms with diameter > 6 mm, 13 (5.4%) presented with findings suggestive of myocardial infarction. Five patients died during the follow-up period. CONCLUSIONS: Severe stenosis or occlusion of the coronary artery may occur in some patients who develop coronary aneurysms with diameter > 6 mm; early management such as coronary interventions or surgery should be considered in such cases.

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 Ebstein's anomaly. The purpose of this study was to investigate the validity of this criterion for the discrimination of Ebstein's anomaly from pulmonary atresia with intact ventricular septum in neonatal patients. A total of 122 neonatal patients, 56 with Ebstein's 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 Ebstein's 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 Ebstein's 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 Ebstein's anomaly in neonatal patients with pulmonary atresia with intact ventricular septum.

Outcome of neonates with Ebstein's anomaly in the current era.

Ebstein's anomaly is frequently detected before birth, with prenatal detection accounting for the majority of cases in the current population. This study aimed to identify the outcome variables among these infants. The medical records of 59 patients with neonatal Ebstein's anomaly managed at the Asan Medical Center between January, 2001 and June, 2012 were investigated retrospectively. In 46 cases, the diagnosis was made prenatally. Surgical/interventional procedures were performed for 27 of the analyzed patients. Biventricular repair was successful for 12 patients but not for 9 patients with pulmonary atresia. The median follow-up period was 1.96 years (range 0.0-10.4 years). The overall mortality rate was 23.7 % (14/59). Of the 14 deaths, 5 occurred within several hours after birth. The 1- and 5-year survival rates were 78.6 and 76.3 %, respectively. Univariate analysis identified several variables related to the time to death: fetal distress (p = 0.002), prematurity (p = 0.036), low birth weight (p = 0.003), diameter of the atrial septal defect (p = 0.022), and pulmonary stenosis/atresia (p = 0.001). Neither the Carpentier classification (p = 0.175) nor the Celermajer index (p = 0.958) was a significant variable. According to the multivariate analysis, fetal distress (p = 0.004) and pulmonary atresia/stenosis (p < 0.001) were significant determinants of outcome. In conclusion, fetal distress and pulmonary atresia/stenosis are significant predictors of mortality in the current population of patients with neonatal Ebstein's anomaly. A close cooperation of associated clinicians is required for an improvement in outcome. To establish a better surgical strategy for patients with Ebstein's anomaly and pulmonary atresia, studies of larger populations are required.

Prenatal diagnosis of common arterial trunk: a single-center's experience.

INTRODUCTION: To report our experience in fetuses prenatally diagnosed with common arterial trunk (CAT) and to evaluate the postnatal outcomes. MATERIAL AND METHODS: This was a retrospective study conducted at Asan Medical Center, Seoul, Korea, between 2003 and 2012. Maternal medical and fetal echocardiographic records regarding fetuses were prenatally diagnosed with CAT were reviewed. Postnatal outcomes of survivors were also assessed during the mean follow-up of 36 months. RESULTS: Of the 17 fetuses that were prenatally diagnosed with CAT and had a final diagnosis by postnatal echocardiography or autopsy, 12 were confirmed to have CAT and 5 were incorrectly diagnosed with CAT. Of the latter, 3 had pulmonary atresia and 2 had aortic atresia. The diagnostic accuracy was 71%. The median gestational age at prenatal diagnosis of confirmed CAT was 24.4 weeks (range 21.1-34.3). The truncal valve was prenatally thickened in 8 fetuses. All but 1 of the 12 confirmed CAT cases had associated heart and/or extracardiac anomalies. Of the 8 liveborn cases of postnatally confirmed CAT, 2 died before or after surgery respectively. The remaining 6 remained alive after successful corrective surgery. CONCLUSION: Prenatal diagnosis of CAT can be difficult in some cases and other diseases should be excluded before diagnosing CAT. Isolated CAT can be repaired by postnatal corrective surgery with a good outcome.

Mitral regurgitation due to chordae tendineae rupture in an infant with aortic coarctation.

A 2-month-old male suddenly developed mitral regurgitation aggravation while waiting for surgical repair of aortic coarctation. There were no signs of infection or history of trauma. Emergency surgery was performed, during which rupture of chordae tendineae of the A2 portion of the anterior mitral leaflet was confirmed. At last follow-up, 11 months postsurgery, the patient was in an acceptable state, with continuing moderate mitral valve regurgitation. This case demonstrates an unusual but dangerous natural course of aortic coarctation and suggests an additional basis of urgent repair of aortic coarctation.

Perivascular brightness of coronary arteries in Kawasaki disease.

OBJECTIVE: Because perivascular echo brightness (PEB) of coronary arteries has been proposed as a criterion for diagnosis of incomplete Kawasaki disease, we assessed the clinical importance of PEB during the acute phase of disease. STUDY DESIGN: We enrolled 58 patients with Kawasaki disease who underwent two-dimensional strain analysis of images of pericoronary tissue taken during the acute and the convalescent phases. Echogenicity of pericoronary tissue and of the blood pool was determined by speckle tracking in the respective areas of imaging as the averages of integrated backscatter over a single cardiac cycle. PEB was defined as echogenicity of pericoronary tissue minus blood pool. RESULTS: PEB did not differ in the acute phase in patients and control subjects (P = .10) and between phases of disease (P = .25). In comparison between patient groups, the presence of pericardial effusion was higher in patients with higher PEB during the acute phase (n = 30) than in the remaining patients (33% versus 4%, P < .01). CONCLUSIONS: PEB did not differ between patients and control subjects and is only associated with the presence of pericardial effusion during the acute phase of Kawasaki disease. Our data do not confirm the reliability of PEB as a useful diagnostic sign of incomplete Kawasaki disease.

The outcome of open heart surgery for congenital heart disease in infants with low body weight less than 2500 g.

Although the outcome of neonatal cardiac surgery has dramatically improved, low body weight (LBW) is still considered an important risk for open heart surgery. The factors contributing to poor outcomes in LBW infants, however, are still unclear. We investigated risk factors for poor outcomes in infants weighing <2500 g who underwent surgical correction with cardiopulmonary bypass (CPB). From January 1995 to December 2009, 102 consecutive patients were included in this study. Median age and body weight at the time of surgery was 19 (range 1 to 365) days and 2.23 kg (range 1.3 to 2.5), respectively. Corrective surgery was performed on 75 infants. The median follow-up duration was 45.03 months (range 0.33 to 155.23). There were 23 (22.5%) hospital mortalities. Emergency surgery and low cardiac output (LCO) were associated with early mortality; however, body weight, Aristotle basic complex score, and type of surgery was not. Early morbidities, including delayed sterna closure, arrhythmia, and chylothorax, occurred in 39 (38.2%) infants. The overall actuarial survival rate at 10 years was 74.95% ± 4.37%. In conclusion, among infants weighing <2500 g who underwent open heart surgery with CPB, perioperative hemodynamic status, such as emergency surgery and LCO, strongly influenced early mortality. In contrast, LBW itself was not associated with patient morbidity or mortality.

Pediatric heart transplantation: how to manage problems affecting long-term outcomes?

Since the initial International Society of Heart Lung Transplantation registry was published in 1982, the number of pediatric heart transplantations has increased markedly, reaching a steady state of 500-550 transplantation annually and occupying up to 10% of total heart transplantations. Heart transplantation is considered an established therapeutic option for patients with end-stage heart disease. The long-term outcomes of pediatric heart transplantations were comparable to those of adults. Issues affecting long-term outcomes include acute cellular rejection, antibody-mediated rejection, cardiac allograft vasculopathy, infection, prolonged renal dysfunction, and malignancies such as posttransplant lymphoproliferative disorder. This article focuses on medical issues before pediatric heart transplantation, according to the Korean Network of Organ Sharing registry and as well as major problems such as graft rejection and cardiac allograft vasculopathy. To reduce graft failure rate and improve long-term outcomes, meticulous monitoring for rejection and medication compliance are also important, especially in adolescents.

The impact of additional antegrade pulmonary blood flow at bidirectional Glenn shunt on long-term outcomes.

OBJECTIVES: We investigated the impact of additional antegrade pulmonary blood flow on the long-term outcomes after bidirectional Glenn shunt. METHODS: From 2001 to 2015, 279 patients underwent bidirectional Glenn shunt as an interim palliation for a functionally single ventricle. After excluding patients with a previous Kawashima or Norwood operation, 202 patients with preexisting antegrade pulmonary blood flow before bidirectional Glenn shunt were included in this study. Antegrade pulmonary blood flow was eliminated in 110 patients (no antegrade pulmonary blood flow group) and maintained in 92 patients (antegrade pulmonary blood flow group). The impact of antegrade pulmonary blood flow at bidirectional Glenn shunt on long-term outcome was analyzed using inverse probability of treatment weighting. RESULTS: Median age and body weight at bidirectional Glenn shunt were 8 months and 7.8 kg, respectively. Prolonged chest tube drainage or readmission for effusion after bidirectional Glenn shunt was more frequent in the antegrade pulmonary blood flow group (odds ratio, 3.067; 95% confidence interval, 1.036-9.073; P = .043). In the no antegrade pulmonary blood flow group, B-type natriuretic peptide level was decreased further until the Fontan operation (P = .012). In the no antegrade pulmonary blood flow group, oxygen saturation was lower just after bidirectional Glenn shunt, although it was increased further until Fontan operation (P < .001), despite still lower oxygen saturation before Fontan operation compared with antegrade pulmonary blood flow group (P < .001). The McGoon ratio was decreased in both groups without intergroup difference, although the McGoon ratio before Fontan operation was higher in the antegrade pulmonary blood flow group (2.3 ± 0.4 vs 2.1 ± 0.4, P = .008). Overall transplant-free survival was worse in the antegrade pulmonary blood flow group (hazard ratio, 2.37; confidence interval, 1.089-5.152; P = .030). CONCLUSIONS: Maintaining antegrade pulmonary blood flow at bidirectional Glenn shunt was beneficial for higher oxygen saturation and larger pulmonary artery size before Fontan operation. However, it was unfavorable for overall transplant-free survival with a sustained higher risk of death or transplant until the elimination of antegrade pulmonary blood flow.

Development of Cardiac Events and Functional Recovery Prediction Models for Pediatric Dilated Cardiomyopathy.

Background: Since both the risk of death and the probability of spontaneous functional recovery (FR) coexist in association with pediatric dilated cardiomyopathy (DCMP), management should be based on individualized outcome predictions. Methods: A single-center retrospective review of 105 pediatric patients (age at presentation ≤ 18 years) with DCMP, managed between 1994 and 2017, was performed. Logistic regression was conducted to identify variables associated with FR and cardiac events (CEs), i.e., death or heart transplantation (HTPL), within 2 years after initial presentation. Two outcome prediction models were formulated using these variables. Results: Twenty-six (24.8%) and 51 patients (48.6%) experienced FR and CE, respectively, within 2 years after initial presentation. Predictors of mortality without HTPL were earlier era at presentation (HR: 4.13; 95% CI: 1.88-9.06; p < 0.001) and significant TR (≥moderate; HR: 4.31; 95% CI: 1.26-14.77; p = 0.020) in multivariable Cox regression model. Predictors of FR were recent era (HR: 4.49; 95% CI: 1.40-14.44; p = 0.0012), younger age at initial presentation (HR: 0.98 per 1 month increase; 95% CI: 0.97-0.99, p < 0.001), post-myocarditis DCMP (HR: 4.29; 95% CI: 1.32-13.93; p = 0.015), and arrhythmia-mediated DCMP (HR: 26.88; 95% CI: 2.61-276.70; p = 0.006). Risk factors for CEs was idiopathic DCMP (HR: 2.95; 95% CI: 1.32-6.56, p = 0.008). The low-risk group who had higher probability of FR than CE in prediction model had a slightly higher overall survival rate (71.4 vs. 52.2% at 10 years after presentation; log-rank p = 0.09) and a significantly higher HTPL-free survival rate (67.5 vs. 24.9% at 10 years after presentation; log-rank p < 0.001) than the high-risk group. Conclusions: Prognostication and management strategies for pediatric DCMP may be enhanced by risk stratification using outcome prediction modeling.

Pulmonary valve replacement following repair of tetralogy of Fallot: comparison of outcomes between bio- and mechanical prostheses.

OBJECTIVES: The aims of this study were to evaluate and compare the outcomes after pulmonary valve replacement (PVR) with a mechanical prosthesis (MP) and a bioprosthesis (BP). METHODS: From 2004 through 2017, a total of 131 patients, who had already been repaired for tetralogy or Fallot or its variants, underwent their first PVR with an MP or a BP. Outcomes of interests were prosthesis failure (stenosis >3.5 m/s, regurgitation >mild or infective endocarditis) and reintervention. RESULTS: The median age at PVR was 19 years. BP and MP were used in 88 (67.2%) and 43 (32.8%) patients, respectively. The median follow-up duration was 7.4 years, and the 10-year survival rate was 96.4%. Risk factors for prosthesis failure were smaller body surface area [hazard ratio (HR) 0.23 per 1 m2, P = 0.047] and smaller prosthesis size (HR 0.73 per 1 mm, P = 0.039). Risk factors for prosthesis reintervention were smaller body surface area (HR 0.11 per 1 m2, P = 0.011) and prosthesis size (HR 0.67 per 1 mm, P = 0.044). Probability of prosthesis failure and reintervention at 10 years were 24.6% (19.5% in BP vs 34.8% in MP, P = 0.34) and 7.8% (5.6% in BP vs 11.9% in MP, P = 0.079), respectively. Anticoagulation-related major thromboembolic events were observed in 4 patients receiving an MP. CONCLUSIONS: MP might not be superior to BP in terms of prosthesis failure or reintervention. MP should be carefully considered for highly selected patients in the era of transcatheter PVR.

Growth Pattern of the Neo-Aorta after Arterial Switch Operation during Childhood.

BACKGROUND AND OBJECTIVES: Neo-aortic root dilatation (ARD) and annular dilatation (AAD) tend to develop after arterial switch operation (ASO). However, the trend of neo-aortic growth has not been well established. This paper aims to identify this trend, its associated factors, and predictors of neo-aortic dilatation after ASO. METHODS: We analyzed the growth trend of the neo-aortic root, annulus, and sinotubular junction (STJ) z-scores using random coefficients model and the risk factors affecting neo-aortic dilatation in 163 patients who underwent ASO from 2006 to 2015. RESULTS: Among 163 patients, 41 had a ventricular septal defect, and 11 had Taussig-Bing (TB) anomaly. The median follow-up duration was 6.61 years. The increased in the neo-aortic root z-score was different between the trapdoor and non-trapdoor coronary artery transfer techniques (0.149/year, p<0.001 vs. 0.311/year, p<0.001). Moreover, the neo-aortic annulus and STJ z-score significantly increased over time after ASO (0.067/year, p<0.001; 0.309/year, p<0.001). Pulmonary artery banding (PAB) was rather a negative affecting factor. The probabilities of freedom from ARD, AAD, and neo-aortic STJ dilatation at 10 years after ASO were 33.4%, 53.9%, and 65.4%. Neo- aortic regurgitation within 1 year was the predictor of ARD, AAD, and neo-aortic STJ dilatation. TB anomaly, PAB, and native pulmonary sinus z-score were other predictors for ARD. CONCLUSION: The growth of neo-aortic root, annulus, and STJ after ASO was greater than somatic growth during childhood. The coronary artery transfer technique affected the growth pattern of the neo-aortic root.

Analyses of left ventricular myocardial deformation by speckle-tracking imaging during the acute phase of Kawasaki disease.

This study aimed to investigate left ventricular myocardial deformation in children with Kawasaki disease during the acute phase of their illness. A total of 50 patients and 35 normal control subjects were assessed. Data were obtained from the patients during the acute and convalescent phases of Kawasaki disease. Analyses of myocardial deformation [strain (epsilon), strain rate (SR)] was performed using two-dimensional speckle-tracking imaging in three directions (longitudinal, circumferential, and radial) at the basal and mid levels of the left ventricular myocardium. Basal longitudinal epsilon (P < 0.001) and midlongitudinal epsilon (P < 0.0001) were lower during the acute phase of the disease than in the control subjects and associated with serum albumin level and left ventricular mass index (LVMI). Midlongitudinal SR (P < 0.0001) was lower during the acute phase of Kawasaki disease than in the control subjects and associated with LVMI. Decreased systolic SR was not detected in any direction. In conclusion, left ventricular longitudinal systolic epsilon was significantly decreased during the acute phase of Kawasaki disease. This may be a result of myocardial swelling from myocarditis during the acute phase of the disease.

Tetralogy of Fallot with aortic stenosis: repair without aortic valve intervention.

A 14-day-old boy with tetralogy of Fallot (ToF), ductus-dependent pulmonary circulation, and significant aortic valve stenosis (AVS) underwent primary repair of ToF without aortic valve intervention. The pressure gradient through the aortic valve decreased spontaneously after the operation. This may have been due to the reduction of blood flow through the aortic valve postoperatively. For patients with ToF, AVS, and ductus-dependent circulation, ToF repair alone may alleviate the severity of the AVS.