Prognosis of Chronic Kidney Disease and Metabolic Syndrome in Adults With Congenital Heart Disease

Background@#Few studies have examined the incidence of chronic kidney disease (CKD) and metabolic syndrome (MS) and their combined prognostic effects in adult congenital heart disease (ACHD). Our aims were to identify the incidence and prognostic implications of CKD and MS in ACHD. @*Methods@#This is retrospective cohort study. We included 2,462 ACHD ≥ 20 years of age who were treated at a tertiary hospital in Korea from 2006 to 2018. CKD was defined as an estimated glomerular filtration rate < 60 mL/min/1.73m 2 . MS was diagnosed based on the presence of abnormal metabolic parameters: blood sugar level, obesity, dyslipidemia, and hypertension. The primary outcome was all-cause mortality from 2006 through 2019 using data from the Ministry of the Interior and Safety in Korea. @*Results@#The incidence of CKD and MS in ACHD was 7.6% and 35.9%, respectively. The coexistence rate of CKD and MS was 4.6%. Although MS was not independently associated with mortality in the multiple analysis (adjusted hazard ratio [aHR], 1.07; 95% confidence interval [CI], 0.79–1.46), it was closely related to the presence of CKD (adjusted odds ratio, 2.62; 95% CI, 1.89–3.63). ACHD patients with CKD had a significantly increased risk of mortality compared with those without CKD (aHR, 2.84; 95% CI, 2.00–4.04). @*Conclusions@#In patients with ACHD, the distribution of MS is higher, and both MS and its components were associated with CKD. Given the CKD was independently associated with mortality, close monitoring and management of renal dysfunction and metabolic parameters in ACHD patients is needed.

A Retrospective Population-Based Survival Study of Idiopathic Pulmonary Arterial Hypertension in Korea

Background@#Few studies used nationwide data to assess the survival rates (SRs) and death risk for idiopathic pulmonary arterial hypertension (IPAH; ICD-10 I27.0) in Korea. @*Methods@#IPAH data (N = 9,017; female:male = 6:4) were collected from the National Health Insurance Service in Korea, from 2006 through 2017. The data consisted of primary diagnoses related to IPAH. The Kaplan–Meier method and Cox proportional-hazards analyses were carried out. @*Results@#The mean age was 62.3 (± 19.4) years, 64.2 (± 18.9) years in female and 59.4 (± 19.8) years in male (P < 0.001). The one-, three-, five- and 10-year SRs for IPAH were 89.0%, 79.8%, 72.3% and 57.0%, respectively. The adjusted hazard ratio (HR) of IPAH was 1.81 (95% confidence interval [CI], 1.26–2.59) in 60–69 age group, 3.42 (95% CI, 2.40–4.87) in 70–79, and 7.73 (95% CI, 5.43–11.0) in 80s. Other risk factors were male, low-income status, diabetes, myocardial infarction, atrial fibrillation, ischemic stroke, hemorrhagic stroke, and malignant neoplasm. @*Conclusion@#The 10-year SR of IPAH was 57% in Korea. The HR for IPAH was significantly high in patients with older age and other risk factors.

Impact of Atrial Fibrillation on Survival in Adults with Congenital Heart Disease: a Retrospective Population-based Study

Background@#The number of adults with congenital heart disease (ACHD) with atrial fibrillation (AF) is expected to increase. We sought to assess the impact of AF on survival in Korean ACHD. @*Methods@#Records of AF in ACHD were extracted from the records of the Korea National Health Insurance Service from 2006 through 2015. Multiple Cox proportional hazards analyses were carried out after adjustment for age, sex, income level, AF, and comorbidities.Survival rates (SRs) with and without AF were compared. The death records from 2006 through 2016 were included. @*Results@#A total of 3,999 ACHD had AF (51.4% were male) and 62,691 ACHD did not have AF (43.5% were male); the proportion of ACHD who were 60 years and older was 53.0% and 27.0% in those with and without AF, respectively (P < 0.001). The age-standardized incidence rate for AF was 1,842.0 persons per 100,000 people in the Korean general population from 2006 through 2015. For AF in ACHD, it was 5,996.4 persons per 100,000 ACHD during the same period, which was higher than that in the general population (P< 0.001). Significantly higher proportion of death (20.9%) occurred in ACHD with AF than without AF (8.3%) (P< 0.001). The adjusted hazard ratio for AF of death in ACHD was 1.39 (95% confidence interval, 1.29– 1.50). The ten-year SR of ACHD with AF was 69.7% whereas it was 87.5% in non-AF (P < 0.001). @*Conclusion@#In ACHD, AF occurs more frequently and has a worse prognosis than seen in the non-valvular general population in Korea. AF is associated with increased death in ACHD, especially with aging.

Epidemiology and Clinical Features of Myocarditis/Pericarditis before the Introduction of mRNA COVID-19 Vaccine in Korean Children:a Multicenter Study

Background@#Korean health authority plans to vaccinate adolescents against coronavirus disease 2019 (COVID-19) starting high school seniors during the summer vacation of 2021.However, the myocarditis/pericarditis following COVID-19 vaccine has been reported recently in adolescents and young adults. This study was performed to answer the urgent questions about the basic epidemiology and clinical course of myocarditis/pericarditis in hospitalized patients prior to the introduction of COVID-19 vaccines in pediatric population. @*Methods@#A retrospective medical record analysis including frequency, clinical characteristics, etiology and outcome of myocarditis/pericarditis was conducted in 17 years and younger patients who were hospitalized in two referral hospitals in Korea between 2010 and 2019. @*Results@#Total 142 patients with myocarditis (n = 119) and/or pericarditis (n = 23) were identified. Median age was 5.4 years (interquartile range, 0.6–12.9 years; range, 11 days–17.8 years), and male was 61%. In adolescents aged 12–17 years, the male to female ratio was 3.2. Myocarditis/pericarditis occurred 0.70 per 1,000 in-patients during the study period: 0.96 (< 1 year), 0.50 (1–5 years), 0.67 (6–11 years) and 1.22 (12–17 years) per 1,000 in-patients, respectively. There was an increasing tendency for the annual frequency from 0.34 in 2010 to 1.25 per 1,000 in-patients in 2019 (P = 0.021). Among the 56 (40%) proven pathogens at admission, Mycoplasma pneumoniae (n = 11, 8%) and enterovirus (n = 10, 7%) were most common. Of the 142 patients, 99 (70%) required pediatric intensive care unit care and 10 (7%) received heart transplantation. In addition, 61 patients (61/131, 47%) without heart medication at admission needed heart medication when they were discharged. Eleven (7.7%) patients died, of which five patients were previously healthy. The median age of deceased patients was lower than the survival group (0.8 vs. 6.3 years, P = 0.014). @*Conclusion@#The frequency of myocarditis/pericarditis was highest among male adolescent in-patients; however, the outcome was favorable in this group without any mortality.

The Waiting List Mortality of Pediatric Heart Transplantation Candidates in Korea before the Pediatric Ventricular Assist Device Era

Background@#Despite advancements in heart transplantation for pediatric patients in Korea, the waiting list mortality has not been reported. Therefore, we investigated the waiting list mortality rate and factors associated with patient mortality. @*Methods@#We reviewed the medical records of pediatric patients who were registered for heart transplantation at three major hospitals in Korea from January 2000 to January 2020.All patients who died while waiting for heart transplantation were investigated, and we identified the waiting list mortality rate, causes of mortality and the median survival periods depending on the variable risk factors. @*Results@#A total of 145 patients received heart transplantations at the three institutions we surveyed, and the waiting list mortality rate was 26%. The most common underlying diseases were cardiomyopathy (66.7%) and congenital heart disease (30.3%). The leading causes that contributed to death were heart failure (36.3%), multi-organ failure (27.2%), and complications associated with extracorporeal membrane oxygenation (ECMO) (25.7%). The median survival period was 63 days. ECMO was applied in 30 patients. The different waiting list mortality percentages according to age, cardiac diagnosis, use of ECMO, and initial Korean Network of Organ Sharing (KONOS) level were determined using univariate analysis, but age was the only significant factor associated with waiting list mortality based on a multivariate analysis. @*Conclusion@#The waiting list mortality of pediatric heart transplantation candidates was confirmed to be considerably high, and age, underlying disease, the application of ECMO, and the initial KONOS level were the factors that influenced the survival period.

Impact of Atrial Fibrillation on Survival in Adults with Congenital Heart Disease: a Retrospective Population-based Study

Background@#The number of adults with congenital heart disease (ACHD) with atrial fibrillation (AF) is expected to increase. We sought to assess the impact of AF on survival in Korean ACHD. @*Methods@#Records of AF in ACHD were extracted from the records of the Korea National Health Insurance Service from 2006 through 2015. Multiple Cox proportional hazards analyses were carried out after adjustment for age, sex, income level, AF, and comorbidities.Survival rates (SRs) with and without AF were compared. The death records from 2006 through 2016 were included. @*Results@#A total of 3,999 ACHD had AF (51.4% were male) and 62,691 ACHD did not have AF (43.5% were male); the proportion of ACHD who were 60 years and older was 53.0% and 27.0% in those with and without AF, respectively (P < 0.001). The age-standardized incidence rate for AF was 1,842.0 persons per 100,000 people in the Korean general population from 2006 through 2015. For AF in ACHD, it was 5,996.4 persons per 100,000 ACHD during the same period, which was higher than that in the general population (P< 0.001). Significantly higher proportion of death (20.9%) occurred in ACHD with AF than without AF (8.3%) (P< 0.001). The adjusted hazard ratio for AF of death in ACHD was 1.39 (95% confidence interval, 1.29– 1.50). The ten-year SR of ACHD with AF was 69.7% whereas it was 87.5% in non-AF (P < 0.001). @*Conclusion@#In ACHD, AF occurs more frequently and has a worse prognosis than seen in the non-valvular general population in Korea. AF is associated with increased death in ACHD, especially with aging.

Epidemiology and Clinical Features of Myocarditis/Pericarditis before the Introduction of mRNA COVID-19 Vaccine in Korean Children:a Multicenter Study

Background@#Korean health authority plans to vaccinate adolescents against coronavirus disease 2019 (COVID-19) starting high school seniors during the summer vacation of 2021.However, the myocarditis/pericarditis following COVID-19 vaccine has been reported recently in adolescents and young adults. This study was performed to answer the urgent questions about the basic epidemiology and clinical course of myocarditis/pericarditis in hospitalized patients prior to the introduction of COVID-19 vaccines in pediatric population. @*Methods@#A retrospective medical record analysis including frequency, clinical characteristics, etiology and outcome of myocarditis/pericarditis was conducted in 17 years and younger patients who were hospitalized in two referral hospitals in Korea between 2010 and 2019. @*Results@#Total 142 patients with myocarditis (n = 119) and/or pericarditis (n = 23) were identified. Median age was 5.4 years (interquartile range, 0.6–12.9 years; range, 11 days–17.8 years), and male was 61%. In adolescents aged 12–17 years, the male to female ratio was 3.2. Myocarditis/pericarditis occurred 0.70 per 1,000 in-patients during the study period: 0.96 (< 1 year), 0.50 (1–5 years), 0.67 (6–11 years) and 1.22 (12–17 years) per 1,000 in-patients, respectively. There was an increasing tendency for the annual frequency from 0.34 in 2010 to 1.25 per 1,000 in-patients in 2019 (P = 0.021). Among the 56 (40%) proven pathogens at admission, Mycoplasma pneumoniae (n = 11, 8%) and enterovirus (n = 10, 7%) were most common. Of the 142 patients, 99 (70%) required pediatric intensive care unit care and 10 (7%) received heart transplantation. In addition, 61 patients (61/131, 47%) without heart medication at admission needed heart medication when they were discharged. Eleven (7.7%) patients died, of which five patients were previously healthy. The median age of deceased patients was lower than the survival group (0.8 vs. 6.3 years, P = 0.014). @*Conclusion@#The frequency of myocarditis/pericarditis was highest among male adolescent in-patients; however, the outcome was favorable in this group without any mortality.

Echogenic Mass Lesion within the Main Pulmonary Artery in a Neonate

Here we present a rare case of pulmonary arterial thrombosis associated with a ductus arteriosus aneurysm that caused severe pulmonary stenosis. A 5-day-old newborn was admitted to our hospital for the evaluation of an intracardiac mass-like lesion found after the detection of a cardiac murmur. Echocardiography and heart computed tomography revealed a mass-like lesion measuring 8.1 mm in diameter across the distal main pulmonary artery to the proximal left pulmonary artery resulting in localized severe stenosis of the left pulmonary artery. Left pulmonary artery angioplasty for surgical resection of the thrombus revealed that the mass was adherent to the proximal part of the left pulmonary artery anterior wall and extended to the ductus arteriosus. Histological examination of the mass showed an old thrombus with dystrophic calcification. Five months after surgery, follow-up echocardiography showed that the left pulmonary artery peak pressure gradient had decreased but the proximal left pulmonary artery stenosis remained. Cardiac catheterization and balloon angioplasty suc cessfully relieved the pulmonary stenosis.

Clinical and molecular characterization of Korean children with infantile and late-onset Pompe disease: 10 years of experience with enzyme replacement therapy at a single center / 소아과

PURPOSE: Pompe disease (PD) is an autosomal recessive disorder caused by a deficiency of acid alpha-glucosidase resulting from pathogenic GAA variants. This study describes the clinical features, genotypes, changes before and after enzyme replacement therapy (ERT), and long-term outcomes in patients with infantile-onset PD (IOPD) and late-onset PD (LOPD) at a tertiary medical center. METHODS: The medical records of 5 Korean patients (2 male, 3 female patients) diagnosed with PD between 2002 and 2013 at Samsung Medical Center in Seoul, Republic of Korea were retrospectively reviewed for data, including clinical and genetic characteristics at diagnosis and clinical course after ERT. RESULTS: Common initial symptoms included hypotonia, cyanosis, and tachycardia in patients with IOPD and limb girdle weakness in patients with LOPD. Electrocardiography at diagnosis revealed hypertrophic cardiomyopathy in all patients with IOPD who showed a stable disease course during a median follow-up period of 10 years. Patients with LOPD showed improved hepatomegaly and liver transaminase level after ERT. CONCLUSION: As ERT is effective for treatment of PD, early identification of this disease is very important. Thus, patients with IOPD should be considered candidates for clinical trials of new drugs in the future.

Pulmonary Arterial Hypertension and Pregnancy: Single Center Experience in Current Era of Targeted Therapy

OBJECTIVES@#To report our experiences in pregnant patients with pulmonary arterial hypertension (PAH) who were treated with targeted therapy.@*METHODS@#From 2011 to 2017, women who decided to maintain pregnancies in our PAH clinic were included. Clinical data, management, and outcomes of the mothers and fetuses were reviewed.@*RESULTS@#Nine women with PAH and 10 deliveries were reviewed. The median maternal age was 28 (26–32) years old. The functional status of each patient was New York Heart Association functional class II or III at first visit. Sildenafil was prescribed in advance in 9 cases of delivery. Multidiscipline team approach management and intensive care were performed during the peripartum period. There was no maternal or fetal mortality. Severe cardiac events occurred in 2 patients with Eisenmenger syndrome: cardiac arrest and uncontrolled arrhythmia. Non-cardiac events occurred in 3 cases: postpartum bleeding, urinary tract infection, and pneumonia. The median gestational period at delivery was about 34 (32–38) weeks. Three cases were emergent delivery because of unexpected preterm labor. Intrauterine growth restriction developed in 4 fetuses.@*CONCLUSIONS@#Pregnancy could be maintained by the introduction of targeted therapy rather more safely than the previous era in the case of maintenance of pregnancy. Intensive care and a multidisciplinary team approach can possibly improve the outcomes of the pregnant women with PAH and their babies. However, pregnancy in patients with PAH is still strongly prohibited and it can be tried in expert center where there has sufficient multidisciplinary team approach in case of inevitability.

The Effect of Multidisciplinary Approach on the Birth Rate of Fetuses with Prenatally Diagnosed Congenital Heart Disease

BACKGROUND: This study aimed to determine the effect of a multidisciplinary approach on the birth rate of fetuses with prenatally diagnosed congenital heart diseases (CHDs). METHODS: Among the fetuses of 724 gravidas who underwent fetal echocardiography in Samsung Medical Center from January 2013 to June 2017, 463 fetuses with normal cardiac structure, arrhythmia or simple left-to-right shunt were excluded, and the remaining 261 were included in the study. The subjects were subdivided into groups based on whether they were consulted multidisciplinarily, that is, consulted simultaneously by pediatric cardiologists, obstetricians and pediatric cardiac surgeons or not. They were also categorized based on the initial fetal echocardiogram results. RESULTS: Among the fetuses in the multidisciplinary group, 64.5% of the fetuses were given birth to, and the proportion was not different from that in the non-multidisciplinary group (68.6%, P = 0.48). The delivery rate in the multidisciplinary consultation group were 69.2% in the transposition of the great arteries group, 63.6% in the tetralogy of Fallot group, 68.8% in the pulmonary atresia or interrupted aortic arch group, 62.5% in the coarctation of aorta group, 60.0% in the atrioventricular septal defect group, 70.0% in the functional single ventricle group, and 55.6% in the hypoplastic left heart syndrome group; there were no significant differences between the 10 echocardiogram groups. However, when the subjects were categorized into Fontan repair group and biventricular repair group, the Fontan repair group showed a significant increase in the likelihood of delivery when a multidisciplinary approach was taken (P = 0.035). CONCLUSION: When a fetus was diagnosed with a CHD where Fontan repair should be considered, a multidisciplinary approach resulted in increased possibility of delivery.

Pulmonary Arterial Hypertension and Pregnancy: Single Center Experience in Current Era of Targeted Therapy

OBJECTIVES: To report our experiences in pregnant patients with pulmonary arterial hypertension (PAH) who were treated with targeted therapy. METHODS: From 2011 to 2017, women who decided to maintain pregnancies in our PAH clinic were included. Clinical data, management, and outcomes of the mothers and fetuses were reviewed. RESULTS: Nine women with PAH and 10 deliveries were reviewed. The median maternal age was 28 (26–32) years old. The functional status of each patient was New York Heart Association functional class II or III at first visit. Sildenafil was prescribed in advance in 9 cases of delivery. Multidiscipline team approach management and intensive care were performed during the peripartum period. There was no maternal or fetal mortality. Severe cardiac events occurred in 2 patients with Eisenmenger syndrome: cardiac arrest and uncontrolled arrhythmia. Non-cardiac events occurred in 3 cases: postpartum bleeding, urinary tract infection, and pneumonia. The median gestational period at delivery was about 34 (32–38) weeks. Three cases were emergent delivery because of unexpected preterm labor. Intrauterine growth restriction developed in 4 fetuses. CONCLUSIONS: Pregnancy could be maintained by the introduction of targeted therapy rather more safely than the previous era in the case of maintenance of pregnancy. Intensive care and a multidisciplinary team approach can possibly improve the outcomes of the pregnant women with PAH and their babies. However, pregnancy in patients with PAH is still strongly prohibited and it can be tried in expert center where there has sufficient multidisciplinary team approach in case of inevitability.

Clinical Implication of Multifocal Atrial Tachycardia in Children for Pediatric Cardiologist

No abstract available.

Current Status of Pediatric Critical Care in Korea: Results of 2015 National Survey

BACKGROUND: The aim of this study was to describe the structure, organization, management, and staffing of pediatric critical care (PCC) in Korea. METHODS: We directed a questionnaire survey for all Upper Grade General Hospitals (n = 43) in Korea in 2015. The first questionnaire was mainly about structure, organization, and staffing and responses were obtained from 32 hospitals. The second questionnaire was mainly about patients and management. Responses to second questionnaire were obtained from 18 hospitals. RESULTS: Twelve from 32 Upper Grade General Hospitals had pediatric intensive care units (PICUs) and 11 of them had the PICU which was exclusive for children. Total number of PICU beds in Korea was 113. The ratio of the number of PICU beds to the number of children was 1:77,460 in Korea and this ratio is lower than that of other developed countries. The mean number of beds in the PICUs was 9.4 ± 9.3 (range, 2–30). There were 16 medical doctors who were assigned for PCC and only 5 of them were full time pediatric intensivists. In the 18 Upper Grade General Hospitals that responded to the second questionnaire survey, there were 97 patients in the PICUs with an average number of 5.7 ± 7.2 (range, 0–22) on the survey day. The mean age of the patients was 3.4 ± 5.6 years. The mean length of hospital stay was 82 ± 271 days. The mean Pediatric Risk of Mortality score III was 9.4 ± 7.8 at the time of admission to the PICUs. CONCLUSION: There is a considerable shortage of PICU beds compared to those in developed countries. In addition, the proportion of PICUs with PCC specialists is much lower than those in the US and European countries.

Outcomes of transcatheter closure of ductus arteriosus in infants less than 6 months of age: a single-center experience / 소아과

PURPOSE: Transcatheter device closure of patent ductus arteriosus (PDA) is challenging in early infancy. We evaluated PDA closure in infants less than 6 months old. METHODS: We performed a retrospective review of infants less than 6 months of age who underwent attempted transcatheter device closure in our institution since 2004. To compare clinical outcomes between age groups, infants aged 6–12 months in the same study period were reviewed. RESULTS: A total of 22 patients underwent transcatheter PDA closure during the study period. Patient mean age was 3.3±1.5 months, and weight was 5.7±1.3 kg. The duct diameter at the narrowest point was 3.0±0.8 mm as measured by angiography. The most common duct type was C in the Krichenko classification. Procedural success was achieved in 19 patients (86.3%). Major complications occurred in 5 patients (22.7%), including device embolization (n=1), acquired aortic coarctation (n=2), access-related vascular injury requiring surgery (n=1), and acute deterioration requiring intubation during the procedure (n=1). Two patients had minor complications (9.1%). Twenty-four infants aged 6–12 months received transcatheter device closure. The procedural success rate was 100%, and there were no major complications. The major complication rate was significantly higher in the group less than 6 months of age (P=0.045). There was a trend toward increased major complication and procedural failure rates in the younger age group (P < 0.01). CONCLUSION: A relatively higher incidence of major complications was observed in infants less than 6 months of age. The decision regarding treatment modality should be individualized.

Anomalous Origin of the Right Subclavian Artery in a Patient with D-transposition of the Great Arteries

This case report concerns a young patient with an extremely rare combination of d-transposition of the great arteries (d-TGA) and anomalous origin of the right subclavian artery. In our patient, the right subclavian artery originated from the pulmonary artery, which is why he did not show reversed differential cyanosis. We conclude that the presence of an aortic arch anomaly should be considered in patients with d-TGA who do not present with reversed differential cyanosis. A further imaging work-up, including computed tomography or magnetic resonance imaging, might be helpful.

The Mid-term Results of Thoracoscopic Closure of Atrial Septal Defects

BACKGROUND AND OBJECTIVES: Recently, minimally invasive surgical (MIS) techniques including robot-assisted operations have been widely applied in cardiac surgery. The thoracoscopic technique is a favorable MIS option for patients with atrial septal defects (ASDs). Accordingly, we report the mid-term results of thoracoscopic ASD closure without robotic assistance. SUBJECTS AND METHODS: We included 66 patients who underwent thoracoscopic ASD closure between June 2006 and July 2014. Mean age was 27±9 years. The mean size of the ASD was 25.9±6.3 mm. Eleven patients (16.7%) had greater than mild tricuspid regurgitation (TR). The TR pressure gradient was 32.4±8.6 mmHg. RESULTS: Fifty-two (78.8%) patients underwent closure with a pericardial patch and 14 (21.2%) underwent direct suture closure. Concomitant procedures included tricuspid valve repair in 8 patients (12.1%), mitral valve repair in 4 patients (6.1%), and right isthmus block in 1 patient (1.5%). The mean length of the right thoracotomy incision was 4.5±0.9 cm. The mean cardiopulmonary bypass time was 159±43 minutes, and the mean aortic cross clamp time was 79±29 minutes. The mean hospital stay lasted 6.1±2.6 days. There were no early deaths. There were 2 reoperations. One was due to ASD patch detachment and the other was due to residual mitral regurgitation after concomitant mitral valve repair. However, there have been no reoperations since July 2010. There were 2 pneumothoraxes requiring chest tube re-insertion. There was one wound dehiscence in an endoscopic port. The mean follow-up duration was 33±31 months. There were no deaths, residual shunts, or reoperations during follow-up. CONCLUSION: Thoracoscopic ASD closure without robotic assistance is feasible, suggesting that this method is a reliable MIS option for patients with ASDs.

Clinical Outcomes after Anatomic Repair Including Hemi-Mustard Operation in Patients with Congenitally Corrected Transposition of the Great Arteries

BACKGROUND AND OBJECTIVES: The aims of this study were to determine the early and late outcomes of anatomic repair of congenitally corrected transposition of the great arteries (ccTGA) and to evaluate effectiveness of the hemi-Mustard procedure. SUBJECTS AND METHODS: We conducted a retrospective, single-center study of patients who underwent anatomic repair for ccTGA between July 1996 and December 2013. Sixteen patients were included in the study. The median age at the time of the operation was 3.5 years (range: 0.5-29.7), and the median body weight was 13.3 kg (range: 5.8-54). The median follow-up duration was 7.7 years (range: 0.2-17.4). RESULTS: Atrial switch was achieved using the Mustard procedure in 12 patients (hemi-Mustard procedure in 11) or the Senning procedure in four patients. The ventriculoarterial procedure was performed using the Rastelli procedure in 11 patients and arterial switch in five patients. Six patients underwent tricuspid valvuloplasty. The survival rate was 93.8±6.1%. The rate of freedom from reoperation at 5 years was 92.3±7.4% in the Rastelli group. All patients except one were New York Heart Association class I. All patients except one had mild tricuspid regurgitation. CONCLUSION: Anatomic repair can be performed with a low risk of in-hospital mortality. The hemi-Mustard strategy for selected patients is one solution for reducing early mortality and morbidity, and long-term complications such as venous pathway stenosis or sinus node dysfunction.

Multicenter, Single-Arm, Phase IV Study of Combined Aspirin and High-Dose “IVIG-SN” Therapy for Pediatric Patients with Kawasaki Disease

BACKGROUND AND OBJECTIVES: Intravenous immunoglobulin-SN (IVIG-SN) is a new human immunoglobulin product. Its safety is ensured by pathogen-elimination steps comprising solvent/detergent treatment and a nanofiltration process. This multicenter clinical study was designed to evaluate the efficacy and safety of combined aspirin and high-dose IVIG-SN therapy in pediatric patients with Kawasaki disease (KD). SUBJECTS AND METHODS: We evaluated coronary artery lesions (CALs) at 2 and 7 weeks after administering IVIG-SN; total fever duration; and variations in erythrocyte sedimentation rate, N-terminal pro B-type natriuretic peptide or B-type natriuretic peptide, and creatine kinase-myocardial band level before and after treatment with IVIG-SN (2 g/kg). Adverse events were monitored. RESULTS: Forty-five patients were enrolled, three of whom were excluded according to the exclusion criteria; the other 42 completed the study. The male:female ratio was 0.91:1, and the mean age was 29.11±17.23 months. The mean fever duration before IVIG-SN treatment was 6.45±1.30 days. Although most patients had complete KD (40 patients, 90.91%), four had atypical KD (9.09%). After IVIG-SN treatment, one patient (2.38%) had CALs, which was significantly lower than the incidence reported previously (15%) (p=0.022), but not significantly different from recent data (5%). There were no serious adverse events, though 28 patients (63.64%) had mild adverse events. Three adverse drug reactions occurred in 2 patients (eczema, anemia, and increased eosinophil count), all of which were transient. CONCLUSION: IVIG-SN treatment in patients with KD was safe and effective.

Clinical Characteristics of Marfan Syndrome in Korea

BACKGROUND AND OBJECTIVES: Marfan syndrome (MFS) is a connective tissue disorder with autosomal dominant inheritance and a highly variable clinical spectrum. However, there are limited data available on the clinical features of Korean patients with MFS. The aim of the present study was to describe the clinical characteristics and outcomes of Korean patients with MFS. SUBJECTS AND METHODS: We included all patients who were diagnosed with MFS between January 1995 and May 2015 at a single tertiary medical center. Patients with an MFS-related disorder including MASS phenotype (myopia, mitral valve prolapse, borderline and non-progressive aortic root dilatation, skeletal findings, and striae), mitral valve prolapse syndrome, and ectopia lentis syndrome were excluded. A total of 343 Korean patients aged ≥15 years who satisfied the revised Ghent nosology were included. RESULTS: The mean patient age at diagnosis was 35.9±12.6 years and 172 (50.1%) patients were male. Median follow-up duration was 52.8 months. A total of 303 patients (88.6%) had aortic root dilatation with Z score ≥2 or aortic root dissection. Ectopia lentis was relatively less common (163 patients, 55.1%) and systemic score ≥7 was found in 217 patients (73.8%). Among 219 probands, a family history of MFS was present in 97 patients (44.5%) and sporadic cases in 121 patients (55.5%). Among the 157 probands who underwent genetic analysis, 141 (89.8%) had an FBN1 mutation associated with aortic root aneurysm/dissection. Aortic dissection (AD) or intramural hematoma (IMH) was identified in 110 patients (32.1%). Among the 221 patients without AD or IMH, descending aortic aneurysms were identified in 19 patients (8.6%). Two hundred thirteen patients (62%) underwent cardiovascular surgery of any type. Eight patients died during follow-up. CONCLUSION: We described the clinical characteristics and outcomes of Korean MFS patients. Cardiovascular manifestations were commonly detected and FBN1 mutation was present in approximately 90% of patients. In contrast, ectopia lentis was identified in approximately half of patients. Our findings will be informative for the evaluation of patients with MFS.