Background: In spite of improved survival after palliation for single ventricle, interstage mortality for a single ventricle with heterotaxy syndrome is unknown. The purpose of this study was to quantify interstage mortality and influence mortality risk factors. Methods: From November 1994 until February 2012, all patients that had a functional single ventricle and heterotaxy syndrome who underwent palliative operations at our center were included. Patients with hypoplastic left heart syndrome and operative mortality cases were excluded. The factors that influenced interstage mortality were determined by multivariate Cox analysis. Results: There were 16 patients with interstage mortality (41.0%), much higher than the non-heterotaxy group (vs. 11.3%, P = 0.001, OR = 5.478). The major presumptive causes of death were infection or sepsis (37.5%) and unknown sudden death (31.3%). When we compared the survival group and the mortality group with heterotaxy syndrome, Blalock-Taussig shunt as a 1st palliation is most common for both groups but there were more for the mortality group (81.2% vs. 52.2%), and there were more with bidirectional cavo-pulmonary shunt as a 1st palliation in the survival group (10 patients vs. 2 patients). The existence of pulmonary vein stenosis at initial diagnosis was more common for the mortality group. In multivariate Cox analysis, however, the duration of hospitalization at palliation, the duration of intensive care unit stay after palliation and the existence of pulmonary vein stenosis at diagnosis were significant risk factors. Conclusion: Interstage mortality for a functional single ventricle with heterotaxy syndrome is significantly higher than for non-heterotaxy syndrome. Therefore more attention should be given to the prevention of interstage mortality in these patients with risk factors.

Background and Aim to Read We report the results of a bicuspid expanded polytetrafluoroethylene (ePTFE) valved conduit used for right ventricular outflow tract reconstruction (RVOTR). Methods Between November 2005 and February 2009, 12 conduits were used for RVOTR. The mean age and weight of patients were 43.5 ± 46.4 months and 13.4 ± 8.6 kg. The main diagnosis was tetralogy of Fallot with pulmonary atresia in eight patients. The most common conduit size was 18 mm. The mean follow-up was 88.0 ± 35.9 months. Results There were no operative and late mortalities. At discharge, the mean peak systolic pressure gradient across the RVOT was 14.1 ± 11.3 mmHg. There was no conduit valve regurgitation in nine patients. At the latest echocardiography (mean follow-up: 84.3 ± 35.5 months), the mean peak systolic pressure gradient across the RVOT was 59.7 ± 20.2 mmHg, and there was no conduit valve regurgitation in six patients. Freedom from conduit malfunction was 100% and 83.3%, at 1 and 8 years, respectively. Two conduits were explanted due to sternal compression and four from conduit malfunction. Freedom from explantation was 83.3% and 74.2% at 2 and 8 years, respectively. Conclusions ePTFE bicuspid valved conduit has good late function in terms of valve regurgitation, but the pressure gradient across the conduit increases with time, which is the main cause of conduit failure and explantation.

In this study, we sought predictors of mortality in children with acute myocarditis and of incomplete recovery in the survivor group. We classified our patients into three groups according to their outcomes at last follow-up: full recovery was classified as group I, incomplete recovery was classified as group II, and death was classified as group III. In total, 55 patients were enrolled in the study: 33 patients in group I, 11 patients in group II, and 11 patients in group III. The initial left ventricular fractional shortening – left ventricular fractional shortening – was significantly lower in group III (p=0.001), and the left ventricular end-diastolic dimension z score was higher in groups II and III compared with group I (p=0.000). A multivariate analysis showed that the left ventricular end-diastolic dimension z score (odds ratio (OR), 1.251; 95% confidence interval (CI), 1.004–1.559), extracorporeal membrane oxygenation (OR, 9.842; 95% CI, 1.044–92.764), and epinephrine infusion (OR, 18.552; 95% CI, 1.759–195.705) were significant predictors of mortality. The left ventricular end-diastolic dimension z score was the only factor that predicted incomplete recovery in the survivor group (OR, 1.360; 95% CI, 1.066–1.734; p=0.013). The receiver operating characteristic curve of the left ventricular end-diastolic dimension z score at admission showed a cut-off level of 3.01 for predicting mortality (95% CI, 0.714–0.948). In conclusion, a high left ventricular end-diastolic dimension z score on admission was a significant predictor of worse outcomes, both regarding mortality and incomplete recovery.

Prognostic factors of coronary aneurysms in Kawasaki disease have been investigated in many studies. The aim of this study was to identify risk factors associated with early and late coronary artery outcomes in treated patients with Kawasaki disease. A total of 392 patients diagnosed with Kawasaki disease from January 2012 to December 2015 in Pusan National University Children's Hospital were retrospectively selected as subjects of the present study to determine risk factors for coronary aneurysms and persistence of coronary aneurysms after a 1-year follow-up. Coronary aneurysms were detected in 30 of 392 patients within 1 month after the occurrence of Kawasaki disease. Coronary aneurysms persisted in 5 of 30 patients after a 1-year follow-up. A long duration of fever (adjusted odds ratio [OR], 1.47; 95% confidence interval [CI], 1.06-2.02; P=0.018) and high platelet count (adjusted OR, 1.00; 95% CI, 1.00-1.01; P=0.009) were found to be independent factors to predict the development of coronary aneurysms in the early phase. Initial coronary severity (adjusted OR, 46.0; 95% CI, 2.01-1047.80; P=0.016) and a high white blood cell count (adjusted OR, 1.17; 95% CI, 1.01-1.36; P=0.028) were found to be significant factors for the persistence of late coronary aneurysms in univariate analysis. However, no significant factors were found in multivariate analysis. These data showed early and late follow-up of coronary aneurysms in our unit. Further studies are needed to determine the mechanisms involved in the disappearance of coronary aneurysms and related factors.

Background and objectives: Left ventricular assist devices enable recovery from severe heart failure and serve as a bridge to heart transplantation. However, chronic mechanical unloading can impair myocardial recovery. We aimed to assess myocyte size, fibrosis, apoptosis, and β-adrenoreceptor levels after rats with left ventricle unloading induced by heterotopic heart transplantation were administered carvedilol and metoprolol. Methods: Thirty rats with heart transplants were divided randomly into control, carvedilol treatment, and metoprolol treatment groups. Follow-up was conducted after 2 and 4 weeks of unloading. Results: Carvedilol and metoprolol treatments did not prevent the decrease in myocyte diameter in unloaded left ventricles. Metoprolol significantly decreased the ratio of the fibrotic area in the unloaded heart, measured using Masson’s trichrome staining after 2 weeks. However, carvedilol and metoprolol did not reduce apoptosis, based on measurements of terminal deoxynucleotidyl-transferase-mediated dUTP nick end-labelling positive cells and the expression of caspase-3 in unloaded hearts after 2 and 4 weeks. Metoprolol treatment did not significantly decrease the mRNA expression of myocardial SERCA2a in the unloaded heart after 2 weeks. Conclusions: Compared to carvedilol treatment, metoprolol treatment improved myocardial fibrosis and SERCA2a expression to a greater extent; however, neither drug prevented myocardial apoptosis.

The most common causes of short stature (SS) in children are familial short stature (FSS) and idiopathic short stature (ISS). Recently, growth plate dysfunction has been recognized as the genetic cause of FSS or ISS. The aim of this study was to investigate monogenic growth failure in patients with ISS and FSS. Targeted exome sequencing was performed in patients categorized as ISS or FSS and the subsequent response to growth hormone (GH) therapy was analyzed. We found 17 genetic causes involving 12 genes (NPR2, IHH, BBS1, COL1A1, COL2A1, TRPS1, MASP1, SPRED1, PTPTN11, ADNP, NADSYN1, and CERT1) and 2 copy number variants. A genetic cause was found in 45.5% and 35.7% of patients with FSS and ISS, respectively. The genetic yield in patients with syndromic and non-syndromic SS was 90% and 23.1%, respectively. In the 11 genetically confirmed patients, a gain in height from −2.6 to −1.3 standard deviations after 2 years of GH treatment was found. The overall diagnostic yield in this study was 41.7%. We identified several genetic causes involving paracrine signaling, the extracellular matrix, and basic intracellular processes. Identification of the causative gene may provide prognostic evidence for the use of GH therapy in non-SGA children.