Baseline Characteristics of the Korean Registry of Pulmonary Arterial Hypertension

Despite recent advances in understanding of the pathobiology and targeted treatments of pulmonary arterial hypertension (PAH), epidemiologic data from large populations have been limited to western countries. The aim of the Korean Registry of Pulmonary Arterial Hypertension (KORPAH) was to examine the epidemiology and prognosis of Korean patients with PAH. KORPAH was designed as a nationwide, multicenter, prospective data collection using an internet webserver from September 2008 to December 2011. A total of 625 patients were enrolled. The patients' mean age was 47.6 ± 15.7 yr, and 503 (80.5%) were women. The diagnostic methods included right heart catheterization (n = 249, 39.8%) and Doppler echocardiography (n = 376, 60.2%). The etiologies, in order of frequency, were connective tissue disease (CTD), congenital heart disease, and idiopathic PAH (IPAH) (49.8%, 25.4%, and 23.2%, respectively). Patients with WHO functional class III or IV at diagnosis were 43.4%. In total, 380 (60.8%) patients received a single PAH-specific treatment at the time of enrollment, but only 72 (18.9%) patients received combination therapy. Incident cases during the registry represented 297 patients; therefore, the incidence rate of PAH was 1.9 patients/yr/million people. The 1st-, 2nd-, and 3rd-yr estimated survival rates were 90.8%, 87.8%, and 84.4%, respectively. Although Korean PAH patients exhibited similar age, gender, and survival rate compared with western registries, they showed relatively more CTD-PAH in the etiology and also systemic lupus erythematosus among CTD-PAH. The data suggest that earlier diagnosis and more specialized therapies should be needed to improve the survival of PAH patients.ope

Sibling Relationships of Adolescents with Congenital Heart Disease

Adolescents with congenital heart disease (CHD) continuously need family support because of their repeated follow ups, treatments, and complications. However, sibling relationships have not been well studied among adolescents with CHD. The purpose of the present study was to explore the relationships between adolescents with CHD and their siblings, and to examine these relationships according to birth order and age. Adolescents aged from 13 to 21 years who had been diagnosed with CHD and had siblings were included as participants. The Sibling Relationship Questionnaire (SRQ) was used. The SRQ consists of four factors: warmth/closeness, conflict, relative power/status, and rivalry. A univariate general linear model was conducted to identify the sibling relationship factors according to birth order and sibling ages. The score for relative power/status of participants who were the eldest sibling was higher than that of younger siblings. The score for rivalry increased as sibling age increased. Therefore, healthcare providers need to investigate sibling relationships and to explain the importance of self-identity and power balance between adolescents with CHD and their siblings to parents.ope

Myocardial Tissue Doppler Velocity in Child Growth

Background: In adults, tissue Doppler imaging (TDI) is a recommended component of routine echocardiography. However, TDI velocities are less accepted in pediatrics, due to their strong variability and age dependence in children. This study examines the distribution of myocardial tissue Doppler velocities in healthy children to assess the effect of age with cardiac growth on the various echocardiographic measurements. Methods: Total 144 healthy children were enrolled in this study. They were recruited from the pediatric outpatient clinic for routine well-child visits. The statistical relationships between age and TDI values were analyzed. Also, the statistical relationships between body surface area (BSA) and TDI values, left ventricle end-diastolic dimension (LVEDD) and TDI values were analyzed. Also, we conducted multivariate analysis of cardiac growth parameters such as, age, BSA, LVEDD and TDI velocity data. Results: All of the age, BSA, and LVEDD had positive correlations with deceleration time (DT), pressure half-time (PHT), peak early diastolic myocardial velocity, peak systolic myocardial velocity, and had negative correlations with peak late diastolic velocity (A) and the ratio of trans-mitral inflow velocity to early diastolic velocity of mitral annulus (E/E'). In the multivariate analysis, all of the age, BSA, and LVEDD had positive correlations with DT, PHT, and negative correlations with A and E/E'. Conclusion: The cardiac growth parameters related alterations of E/E' may suggest that diastolic myocardial velocities are cardiac growth dependent, and diastolic function has positive correlation with cardiac growth in pediatric group. This cardiac growth related myocardial functional variation would be important for assessment of cardiac involvement either in healthy and sick child.ope

Increasing demand for hospitalization in adults with congenital heart disease

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Clinical outcomes of initial dexamethasone treatment combined with a single high dose of intravenous immunoglobulin for primary treatment of Kawasaki disease.

PURPOSE: To investigate the clinical effects of a single high dose intravenous immunoglobulin (IVIG) combined with initial dexamethasone as a primary treatment on Kawasaki disease (KD). MATERIALS AND METHODS: Between January 2008 and December 2010, we reviewed the medical records of 216 patients with complete KD patients that were admitted to a single medical center. 106 patients were treated with a single high dose of IVIG (2 g/kg) alone and 110 patients received IVIG and dexamethasone (0.3 mg/kg per day for three days). RESULTS: The combined IVIG plus dexamethasone patient group had a significantly shorter febrile period and duration of hospital stay (1.4±0.7 days vs. 2.0±1.2 days, p<0.001; 5.8±1.7 days vs. 6.9±2.5 days, p<0.001, respectively) than the IVIG alone group. The combined IVIG plus dexamethasone group required IVIG retreatment significantly less than the IVIG only group (12.7% vs. 32%, p=0.003). After completion of the initial IVIG, C-reactive protein levels in the combined IVIG plus dexamethasone group were significantly lower than those in the IVIG only group (2.7±4.0 mg/dL vs. 4.6±8.7 mg/dL, p=0.03). In the combined IVIG plus dexamethasone group, the incidence of coronary artery lesions tended to be lower without worse outcomes at admission after initial infusion of IVIG and in follow-up at two months; however, the differences were not significant (8.2% vs. 11.3%, p=0.22; 0.9% vs. 2.8%, p=0.29). CONCLUSION: Initial combined therapy with dexamethasone and a single high-dose of IVIG resulted in an improved clinical course, in particular a shorter febrile period, less IVIG retreatment, and shorter hospital stay without worse coronary outcomes.ope

The First Pediatric Heart Transplantation Bridged by a Durable Left Ventricular Assist Device in Korea

Treatment options for children with end-stage heart failure are limited. We report the first case of a successful pediatric heart transplantation bridged with a durable left ventricular assist device in Korea. A 10-month-old female infant with dilated cardiomyopathy and left ventricular non-compaction was listed for heart transplantation. During the waiting period, the patient's status deteriorated. Therefore, we decided to provide support with a durable left ventricular assist device as a bridge to transplantation. The patient was successfully bridged to heart transplantation with effective support and without any major adverse events.ope

Respiratory syncytial virus infection in children with congenital heart disease: global data and interim results of Korean RSV-CHD survey

Respiratory syncytial virus (RSV) is a main cause of hospitalization for bronchiolitis and pneumonia in infants worldwide. Children with hemodynamically significant congenital heart disease (HS-CHD), as well as premature infants are at high risk for severe RSV diseases. Mortality rates for CHD patients hospitalized with RSV have been reported as about 24 times higher compared with those without RSV infection. Recently with advances in intensive care, mortality rates in CHD patients combined with RSV have decreased below 2%. The requirements of intensive care and mechanical ventilation for CHD patients with RSV infection were still higher than those without RSV infection or with non-CHD children. RSV infection has frequently threatened CHD infants with congestive heart failure, cyanosis, or with pulmonary hypertension. As a progressive RSV pneumonitis in those infants develops, the impairment of oxygen uptake, the breathing workload gradually increases and eventually causes to significant pulmonary hypertension, even after the operation. Preventing RSV infection as much as possible is very important, especially in infants with HS-CHD. A humanized monoclonal antibody, palivizumab, has effective in preventing severe RSV disease in high-risk infants, and progressive advances in supportive care including pulmonary vasodilator have dramatically decreased the mortality (<1%). Depending on the global trend, Korean Health Insurance guidelines have approved the use of palivizumab in children <1 year of age with HS-CHD since 2009. Korean data are collected for RSV prophylaxis in infants with CHD.ope

Heart Transplantation in a Patient with Complex Congenital Heart Disease, Physiologic Single Lung, and Severe Pulmonary Hypertension

Here we report a case of successful heart transplantation (HT) in a patient with high risk on HT due to her complex congenital heart disease and its consequent complications; physiologic single lung and severe pulmonary arterial hypertension. HT was successfully performed in our patient using meticulous perioperative management techniques, such as control of collateral vessels, appropriate donor selection, pulmonary vasodilator therapy, post-transplant extracorporeal membranous oxygenation support, and atrial septostomy for right ventricular unloading.ope

Echocardiographic Assessment of Patients with Glycogen Storage Disease in a Single Center

Glycogen storage disease (GSD) is a hereditary metabolic disorder caused by enzyme deficiency resulting in glycogen accumulation in the liver, muscle, heart, or kidney. GSD types II, III, IV, and IX are associated with cardiac involvement. However, cardiac manifestation in other GSD types is unclear. This study aimed to describe whether energy deprivation and the toxic effects of accumulated glycogen affect the heart of patients with GSD. We evaluated the left ventricle (LV) wall mass, LV systolic and diastolic function and myocardial strain with conventional echocardiography and two-dimensional speckle-tracking echocardiography (2D STE) in 62 patients with GSD type I, III, VI and IX who visited the Wonju Severance Hospital in 2021. Among the GSD patients, the echocardiographic parameters of 55 pediatrics were converted into z-scores and analyzed. Of the patients, 43 (62.3%), 7 (11.3%) and 12 (19.4%) patients were diagnosed with GSD type I, type III, and type IX, respectively. The median age was 9 years (range, 1-36 years), with 55 children under 18 years old and seven adults over 18 years. For the 55 pediatric patients, the echocardiographic parameters were converted into a z-score and analyzed. Multiple linear regression analysis showed that the BMI z-score (p = 0.022) and CK (p = 0.020) predicted increased LV mass z-score, regardless of GSD type. There was no difference in the diastolic and systolic functions according to myocardial thickness; however, 2D STE showed a negative correlation with the LV mass (r = -0.28, p = 0.041). Given that patients with GSD tend to be overweight, serial evaluation with echocardiography might be required for all types of GSD.ope

Early to Mid-Term Follow-Up Outcomes of Percutaneous Closure of Atrial Septal Defects Using Recent Generation Devices: a Single-Center Experience

BACKGROUND AND OBJECTIVES: This study aimed to describe our early to mid-term experience with transcatheter atrial septal defect (ASD) closure using the Occlutech Figulla® Flex II device (FSO), Gore® Cardioform septal occluder (GSO), and Amplatzer® septal occluder (ASO) after they were first approved in Korea in 2014, and to compare the three aforementioned kinds of ASD closure devices. METHODS: Between September 2014 and August 2016, 267 patients underwent transcatheter ASD closure in our institution. Baseline characteristics, hemodynamic features, comorbidities, and procedural success and complication rates were analyzed retrospectively. The unpaired Student t-test or variance analysis was used in the statistical analysis. RESULTS: The FSO was most commonly used (n=152, 56.9%), followed by the ASO (n=98, 36.7%) and GSO (n=17, 6.4%). Baseline characteristics and hemodynamic features were similar between the devices, except that the defect size and pulmonary flow-to-systemic flow ratio were lower in the GSO group than in the other groups. Overall, the procedural success rate remained at 100%, and major complication rate was <1%. No late complication occurred during the follow-up. CONCLUSIONS: The FSO and GSO are feasible, safe options for use in transcatheter ASD closure, and they are comparable to the ASO.ope