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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. Graphical Abstract

For the anomalous aortic origin of the coronary artery with interarterial course, surgical repair is recommended. We describe a technique of neo-ostium creation at the correct anatomic position for the right coronary artery arising from a single left coronary ostium without an intramural course and coursing between the ascending aorta and the main pulmonary artery.

A 23-year-old female patient with a history of pulmonary valvectomy for pulmonary stenosis at 2 years of age underwent pulmonary valve repair, which consisted of remnant cusp extension using a fresh pericardium and commissural resuspension. An immediate postoperative computed tomographic scan showed full movement of the extended anterior cusp during systole, yet flail motion during diastole. However, follow-up magnetic resonance imaging at 10 months postoperatively revealed a small amount of pulmonary flow regurgitation (2.0%).

Cor triatriatum is a rare congenital anomaly which has wide-ranging severity depending on the structure of the additional intra-atrial septum. We report the case of a boy who was incidentally diagnosed with cor triatriatum by echocardiography during diagnosis and treatment of Kawasaki disease. He has had no definite life-threatening symptoms except recurring respiratory infections. Serial follow-up of such patients is mandatory to check for the potential risks related to the anomalous inter-atrial structure.

Methylmalonic acidemia (MMA) is an autosomal recessive inborn error of metabolism caused by inadequate function of methylmalonyl-CoA mutase. We studied five Korean patients diagnosed with mut MMA, here, we report five new missense mutations (G94E, R369C, S344Y, N189K, and T230I) and a previously reported mutation (R369H) that, this is the first time this mutation has been identified in Korean individuals. Genetic heterogeneity in mut MMA is high. The R369H mutation has been identified in America and Japan. To date, more than 55 different mutations have been identified in mut MMA. A majority of mutations is novel with only three (G717V, E117X, and N219Y) being reported more frequently, the G717V mutation was found in Africa-Americans and Ghanaian. The E117X mutation has been found in Japan. The N219Y mutation has been found in Caucasians and Arab. The R369H mutation is the first mutation identified in three nations (Korea, Japan, and America).

Neuroblastoma is the third most common pediatric cancer and can mimic many other diseases. However, clinicians need to be aware about the possibility of neuroblastoma, when other more obvious clinical conditions such as the Kawasaki disease (KD) are present. KD is an acute febrile vasculitis of childhood with coronary complications. There were a few reports for autopsy of KD patients with neuroblastoma. A 2-y-old girl was admitted for definite KD with five clinical signs. Her fever did not respond to retreatment with intravenous high dose immunoglobulin. Later a chest computerized tomography scan revealed a mass lesion in the right lower lung field, and ultimately diagnosed as neuroblastoma on the paravertebral area of thoracic spine levels T4 to T10. The second patient was a 3-y-old boy who was diagnosed as incomplete KD with left coronary ectasia. His fever also did not respond to retreatment with immunoglobulin, and the abdominal computerized tomography scan with biopsy confirmed neuroblastoma of the right adrenal gland. We report two patients with KD who were additionally diagnosed as having neuroblastoma.

Erythromelalgia is a rare clinical syndrome characterized by erythema, increased temperature, and severe burning pain that can be aggravated by warmth or relieved by cold. Erythromelalgia occurs either as a primary, idiopathic form, or secondary to a number of diseases and conditions. Although fairly well studied in adults, the characteristics, pathogenesis, and natural history are poorly characterized in the pediatric age group. Different therapeutic options have been tried, but no optimal treatment has been suggested for erythromelalgia. We report a rare case of linear erythromelalgia in a 12-year-old girl involving her central body from the peripheral extremities, which seemed to be secondary due to vasculitis. Clinical progress waxed and waned on maintenance aspirin and propranolol.

Survival rates of preterm infants have improved in the past few decades, and central venous catheters play an important role in the intensive medical treatment of these neonates. Unfortunately, these indwelling catheters increase the risk of intracardiac thrombosis, and they provide a nidus for microorganisms during the course of septicemia. Herein, we report a case of persistent bacteremia due to methicillin-resistant Staphylococcus aureus in an extremely low birth weight (ELBW) infant, along with vegetation observed on an echocardiogram, the findings which are compatible with a diagnosis of endocarditis. The endocarditis was successfully treated with antibiotic therapy, and the patient recovered without major complications. We suggest a surveillance echocardiogram for ELBW infants within a few days of birth, with regular follow-up studies when clinical signs of sepsis are observed.

Background and Objectives We conducted a review of current data on respiratory syncytial virus (RSV) prophylaxis with palivizumab, in Korean children with congenital heart diseases (CHD). In 2009, the Korean guideline for RSV prophylaxis had established up to five shots monthly per RSV season, only for children

Purpose As cardiomyopathy is more prevalent and currently the leading cause of death in Duchenne muscular dystrophy (DMD), early detection of myocardial involvement is important. The purpose of this study was to analyze myocardial strain in DMD children, for the possibility of early detection of myocardial dysfunction. Materials and Methods We reviewed medical records of DMD patients who were >10 years of age (15.6±1.6 years, 12.5–18 years), from March 2013 to June 2014. Data of 24 DMD children who underwent echocardiography with three-layer specific myocardial strain were compared with 24 controls (age: 9.3±4.0 years, 5.5–17 years). Results Epicardial longitudinal strain was lower in DMD (DMD: -9.3±3.8%; control: -12.3±4.3%; p=0.012). Radial strain (DMD: 24.1±11.1%; control: 37.3±25.9%; p=0.027) and strain rate (SR) (DMD: 1.68±0.91; control: 2.42±0.84; p=0.006) on parasternal short axis view were lower in DMD. Circumferential strains in the endocardium (DMD: -17.5±4.7%; control: -24.2±5.3%; p

Idiopathic cardiomyopathies (CMPs) are an important heterogeneous group of diseases. With the advance of therapeutic strategies, epidemiologic data on CMP have become very important, but only a few have been reported in Asian children. We conducted a retrospective epidemiologic study of primary CMP in Korean children.Using a multicenter survey, we studied primary CMP among Korean children from January 1998 to December 2006 based on classification (2006) of CMP by the American Heart Association. A total of 277 primary CMP patients were reported from 17 cardiovascular centers. The average annual occurrence of new cases of primary CMP was 0.28 per 100,000 Korean children younger than 15 years of age (95% confidence interval (CI) 0.24-0.31). Dilated CMP (DCMP) was 66.43%, hypertrophic CMP (HCMP) 23.47%, restrictive CMP (RCMP) 6.50% and others 3.61%. The point prevalence of primary CMP at the end of the study was estimated as 2.11/100,000 (95%CI 1.83-2.43), DCMP 1.39/100,000, HCMP 0.51/100,000, RCMP 0.16/100,000 and others 0.04/100,000. Survival rates over 9 years were 69.8% in DCMP, 90.3% in HCMP, and 47.2% in RCMP.Recent point prevalence of childhood primary CMP in Korea was estimated as 2.11/100,000. Further epidemiologic study with a nationwide survey is necessary.

a Division of Cardiovascular Surgery, Congenital Heart Disease Center, Severance Cardiovascular Hospital, Department of Thoracic and Cardiovascular Surgery, Yonsei University College of Medicine, Seoul, Republic of Korea b Division of Pediatric Cardiology, Congenital Heart Disease Center, Severance Cardiovascular Hospital, Department of Pediatrics, Yonsei University College of Medicine, Seoul, Republic of Korea

Patent ductus arteriosus (PDA) is a congenital heart disease defined as the arterial connection between the pulmonary artery and the aorta. The ductus arteriosus is an essential structure that shunts blood away from the lungs during fetal life, and only becomes abnormal if it remains patent more than three months after birth in term infants. Spontaneous delayed closure of the ductus arteriosus occurred in 79% of infants during the neonatal period.1) The overall incidence in infants born prematurely is about 16 times higher than the incidence in full term infants. The rate of PDA in extremely premature neonates is close to 30%. Preterm infants with symptomatic heart failure secondary to persistent patency of the ductus arteriosus may be treated by surgical ligation or medically with conservative treatment such as indomethacin or ibuprofen. Medical intervention is usually the treatment of choice due to the risks involved with surgical ligation. Early surgical ligation was supported as the optimal therapy for PDA because it ensured definitive ductal closure with minimal morbidity and mortality in theses high risk infants.1),2) Diagnosis of PDA is usually based on clinical examination and transthoracic echocardiography. Now that color Doppler imaging has been introduced, it is possible to accurately assess even a tiny PDA.1),3) PDAs are usually classified as small, moderate or large by size measurement, and as type A (conical ductus), B (window like ductus), C (tubular ductus), D (ductus with multiple narrowing) or E (elongated ductus) by its configuration on an angiogram of the aortic arch.3) The traditional approach of PDA closure, either with surgery or, more recently, with transcatheter techniques, has been the mainstay of treatment. And management of PDA has continued to progress as innovative technologies have become available. Thus, the outcome and goals for PDA closure have changed and depend on the treatment modality. Indeed, strategies for management of PDA continue to evolve.

Dilated cardiomyopathy, which mostly has an idiopathic etiology or is caused by genetic inheritance or infection, can cause irreversible congestive heart failure. Hypocalcemia is a rare etiology of reversible dilated cardiomyopathy. Here we report the case of a two-month-old girl with congestive heart failure who was diagnosed as having dilated cardiomyopathy secondary to hypocalcemia. After calcium and vitamin D replacement therapy, the patient showed a rapid reduction in hypocalcemic tetany and a rapid recovery of left ventricular function. The cause of the hypocalcemia was vitamin D deficient rickets. She was exclusively breast-fed as an infant, and her mother had a vitamin D deficiency and was diagnosed with osteomalacia.

A 29-year-old woman was referred to our institute for symptomatic hypoxemia. Her dyspnea was aggravated while sitting or standing and relieved while in supine position. She did not have any pulmonary disease. Transthoracic echocardiography and heart computed tomography revealed an underestimated small atrial septal defect (ASD) with a left-to-right shunt. A cardiac catheterization was performed to evaluate pulmonary hypertension. It revealed a normal pulmonary artery pressure and a large ASD with bidirectional shunt during Valsalva maneuver by intracardiac echocardiogram. Her arterial oxygen saturation decreased from 93% while supine to 79% while at a 15° sitting position. Thus, the patient was diagnosed with platypnea-orthodeoxia syndrome. The ASD was successfully closed with Amplatzer® (St. Jude Medical) septal occluder and both platypnea and orthodeoxia were resolved immediately after the procedure.

Refer to the page 59-66 Negishi et al.1) reported unexpected hospitalization (UEH) in adults with congenital heart disease (CHD) in a single center (National Cerebral Cardiovascular Center, Suita, Japan). They included patients ≥18 years with CHD who were hospitalized at this facility from January 2005 to December 2009. The causes of UEH were arrhythmia, heart failure, infectious disease, hemorrhage or thrombosis, neurological abnormality, and other causes. They investigated the duration of hospitalization for patients who survived to discharge from the hospital, the incidence of death, presence/absence of a heart transplant or heart/lung transplant, and the incidence of a second UEH. They also calculated post-discharge survival and post-discharge event-free survival rates. An event was considered either death or a second UEH, regardless of cause. Overall, 886 patients with CHD underwent 1371 hospitalizations; of these, 145 patients were unexpectedly hospitalized 239 times (17%). The median age at UEH was 27 years old and younger people (18-29 years of age) comprised 58% of all subjects. Underlying heart diseases included repaired Tetralogy of Fallot (21%), single ventricular physiology after the Fontan operation (17%), and Eisenmenger syndrome (12%). Arrhythmia (40%) was the most common cause of UEH followed by heart failure (20%), infectious disease (13%), and hemorrhage or thrombosis (13%). There were four hospital deaths, and 48 patients required readmission. In total, 13 patients died. The rate of event avoidance was 77% for 1 year and 58% for 3 years. Administration of an anticoagulant was the only prognostic factor in the univariate analysis. The rate of UEH was low in this Japanese report, compared with that of reports from Europe.2) The relatively low UEH rate may have been a reflection of the many scheduled hospitalizations for regular hemodynamic evaluations by cardiac catheterization at this facility. The most common UEH was related to arrhythmia. This result agrees with a European study.2) The cause of readmission was often arrhythmia relapse. In addition to drug therapy, electrophysiological and catheter ablation studies, as well as interventions for hemodynamic abnormalities should be actively considered to avoid hospital readmission due to arrhythmia. Similar to acquired heart disease, transplantation is a means to treat refractory heart failure in adult patients with CHD. Transplantation is expected to become increasingly more prevalent as a treatment choice for heart failure in adult patients with CHD. Seddio et al.3) reported that heart transplantation for patients with CHD can be performed with acceptable results, with an overall survival rate of 67% at 10 years in 85 patients. Previous procedures, including the Fontan operation, do not reduce survival rate. Mortality is related to preoperative patient condition. No heart transplantation or heart/lung transplantation was performed during the study period in this Japanese study, which may have been due to the donor shortage in Japan. However, as most patients who undergo the Fontan operation are expected to live longer, there will be more patients developing heart failure. Thus, we must be prepared for the increase in the number of heart failure cases in adults with CHD in the near future. It is difficult to perform heart transplantation in an adult with CHD because of the complex CHD anatomy and multiple previous open heart surgeries. This surgery requires many surgeons trained in congenital heart surgery, adult cardiac surgery, and heart transplantation, as well as retrieval teams who are aware of the need to procure extra tissue for reconstructing the great vessels. Each year the population of adults with CHD grows in number and age. Wren and O'Sullivan4) reported 400% growth in the number of clinics for adults with CHD in the past 10 years. With the high and annually increasing hospital admissions rate, the demand for expert cardiologists to treat adult patients with CHD is increasing. Therefore, cooperation of an adult CHD specialist-based medical team, including echocardiologists, interventionists, electrophysiologists, radiologists, cardiac surgeons, and obstetricians is essential to prepare for patients with complex heart disease in the future.

Ahealthy 30-year-old man who had not had any prior medical or surgical history presented to the emergency department with substernal pain for 2 days that was sharp and exacerbated by inspiration and recumbency. Chest radiograph (Figure 1A) showed mild left atrial enlargement. The ECG showed normal findings. The next day, the patient complained of sudden onset of class 3 dyspnea and frothy blood-tinged sputum. Follow-up …

A 16 year-old Korean girl with cardiomegaly was found to have a "right atrial cystic mass" on transthoracic echocardiography. An unusual cystic structure made the diagnosis difficult. However, transesophageal echocardiography and multidetector computed tomography revealed a coronary artery fistula with a distal saccular aneurysm involving the interatrial septum and draining into the right atrium. Multidetector computed tomo-graphy provided clear anatomic visualization that fully delineated the abnormal structures. Multidetector computed tomography may be considered as a good alternative for transesophageal echocardiography in assessing a tortuous coronary artery fistula and aneurysm with complex anatomy.

Background and Objectives The profile of infective endocarditis (IE) has changed and is now showing an increasing prevalence of IE among congenital heart disease (CHD) patients. We studied the change of clinical profiles of IE over the past 25 years in patients with CHD at a single institution. Subjects and Methods We reviewed medical records retrospectively for 325 patients diagnosed with IE between January 1, 1987, and March 31, 2012. We analyzed and compared the differences in patient characteristics and outcomes between 1987-2000 (group A) and 2001-2012 (group B). Results Over the 25-year period, 93 cases of IE in CHD patients were diagnosed (59 cases in group A and 34 cases in group B). Ventricular septal defect was the most common underlying cardiac disease observed during the entire period. The most common causative pathogen was Streptococcus in both groups. Group A contained 16 cases (27.1%) that had undergone cardiac surgery, whereas this number was 19 (55.8%) in group B. The number of patients who had undergone palliative care or surgery using prosthetic materials was higher among group B patients (p

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

A 5-month-old Korean boy who presented with lethargy and cardiomyopathy was diagnosed with very long chain acyl coenzyme A dehydrogenase (VLCAD) deficiency by organic acid, fatty acid, acylcarnitine, and molecular genetic analysis. The patient was a compound heterozygote for mutations in the VLCAD gene. One allele contains a 3-bp deletion in exon 6, deleting glutamic acid in codon 130 (E130del ); this allele is of paternal origin. The patient’s maternally derived allele is a novel mutation, C1843T in exon 20, which creates a premature termination codon (R615stop ). Although molecular genetic characterization of VLCAD deficiency is limited to a few patients, heterogeneity of mutations is already apparent. However, the E130del is a relatively frequent mutant allele, which has been noted in 2 previously identified patients. The 2 mutant alleles in our patient appear to be responsible for his severe and fatal clinical manifestations. (J Pediatr 1999;135:250-3)

Background: The use of ambulatory blood pressure monitoring (ABPM) in childhood hypertension has been in great advance. ABPM has been able to detect significantly high blood pressure (BP) and also helpful for identifying white coat hypertension. Methods: Between March 2007 and December 2009, we evaluated individuals aged from 7 to 19 years who were referred as high BP with ABPM, echocardiogram, and abdominal computerized tomography, calculation of body mass index (BMI) at Ajou University Hospital. ABPM was performed using the Tonoport V monitors and SpaceLab 90217 monitors. Seventy were evaluated with 55 boys and 5 girls. The mean age of them was 13.1 ± 2.8 years (mean ± standard deviation). Results: Forty of seventy (57.1%) were diagnosed as hypertension in ABPM and 8 of 40 were diagnosed as secondary hypertension with underline diseases such as hyperthyroidism, chronic renal disease, Takayasu’s arteritis, coarctation of aorta. The mean BMI in 40 was 24.8 ± 4.72 kg/m. Twenty-three of seventy (32.8%) were obese with higher prevalence than in general population. White coat hypertension was diagnosed in 30 of 70 (42.9%). Conclusions: ABPM could provide more detailed data including mean values of BP, load, and night dip in assessment of children's BP. According to increasing children’s obesity, use of ABPM was thought to be necessary for evaluation of their risk of hypertension and useful for diagnosis of masked hypertension and white coat hypertension. Further more study of ABPM in children and adolescents would be needed for absolute standards of ABPM. (J Korean Soc Hypertens 2012;18(1):46-51)

Patent ductus arteriosus (PDA), defined as an arterial connection between the pulmonary artery and the aorta, is a common congenital cardiac defect. The connection can be closed surgically or by transcatheter methods. The overall incidence of PDA in infants born prematurely is about 16 times higher than that in full term infants. Preterm infants with symptomatic heart failure secondary to persistent PDA can be treated by surgical ligation or by conservative treatment involving indomethacin or ibuprofen. The latter, more conservative approach, is usually the treatment of choice due to the risks involved with surgical ligation in premature babies.1),2) Transcatheter techniques for PDA closure have been evolving for over 40 years, and most PDAs can be safely closed in the laboratory setting using currently available coils and devices. Transcatheter closure of the ductus has been effective, with shorter hospital stay and lower cost compared to surgical closure.2),3)

Background and Objectives Obesity is a chronic disease that requires good eating habits and an active life style. Obesity may start in childhood and continue until adulthood. Severely obese children have complications such as diabetes, hypercholesterolemia, hypertension and atherosclerosis. The goal of this study was to determine the effects of exercise programs on anthropometric, metabolic, and cardiovascular parameters in obese children. Subjects and Methods Fifty four obese children were included. Anthropometric data such as blood pressures, body mass index (BMI) and obesity index (OI) were measured. Blood glucose, total cholesterol, triglycerides, low density lipoprotein-cholesterol (LDL-C), high density lipoprotein-cholesterol (HDL-C), aspartate aminotransferase (AST), alanine aminotransferase (ALT), high sensitive-CRP (hs-CRP), brachial-ankle pulse wave velocity (BaPWV) and ankle brachial index (ABI) were measured. Physical fitness measurements were done. Obese children were divided into three groups: an aerobic exercise group (n=16), a combined exercise group (n=20), and a control group (n=18). Obese children exercised in each program for 10 weeks while those in the control group maintained their former lifestyle. After 10 weeks, anthropometric data and cardiovascular parameters were compared with the data obtained before the exercise program. Results LDL-C, waist circumference, and systolic blood pressure decreased significantly in the aerobic exercise group compared to the control group (p

Hypertension is a major risk factor for myocardial infarction, stroke, and cardiovascular mortality in adults; its treatment reduces the risk of cardiovascular events. In recent times, attention is being paid to monitoring of blood pressure from childhood to adulthood. Childhood hypertension is associated with hypertension in later life, and early intervention is important. In the Korean socioeconomic background, a rapid increase is observed in the number of obesity cases and the rate of increase in the incidence of obesity is more in childhood. The strong association of high blood pressure with obesity and the marked increase in the incidence of childhood obesity indicate that both hypertension and prehypertension are becoming significant health issues. In this comprehensive review, we acquaint the clinician with the available literature on childhood hypertension to provide recommendations for the diagnosis, evaluation, and treatment of hypertension based on the available evidence and consensus in Korean clinical conditions.

Department of Pediatrics, Ewha Womans University, Department of Pediatrics*, College of Medicine, Inje University, Department of Preventive Medicine, Ewha Womans University, Department of Pediatric, Hong Ik Hospital, Seoul Department of Pediatrics, College of Medicine, Ajou University, Suwon, Department of Pediatrics, College of Medicine, Han Yang University, Seoul Department of Pediatrics, College of Medicine, Seoul National University, Seoul, Korea

for gender, age, and height on ≥three occasions. Knowing that blood pressure values increase in children as they grow older, the purposes of this study were to measure blood pressure by an oscillometric device and to determine normal values and percentile curves for children. Methods :Systolic and diastolic blood pressures were measured twice with an oscillometric device in 3,545 boys and 3,145 girls under six years of age, in Seoul. Using this data, we determined average blood pressure values and percentile curves based on gender and age; we subdivided these values into blood pressures of 50 th , 90 th , 95 th , and 99 th percentiles, by percentile of height. The regression coefficients and standard deviations of the systolic and diastolic blood pressure values were obtained from linear regression models. Results :Older boys and girls had higher systolic and diastolic blood pressure values. Older boys and girls in the same percentile of height for age had higher systolic and diastolic blood pressure values. Taller boys and girls within the same age group had higher systolic and diastolic blood pressure values. Conclusion :Blood pressure standards based on gender, age, and height were obtained via an oscillometric method. Llimitation of this study is that the study population was not from the whole country, but exclusively from Seoul. Nonetheless, the data from this study will be helpful in diagnosing and managing hypertension in Korean children. (Korean J Pediatr 2008;51:998-1006)

Right-sided heart failure is a major problem among patients with congenital heart diseases, due to the prevalence of congenital heart defects and the association of pulmonary hypertension. More attention is focused on the structure of the right heart particularly in association with congenital heart defects and chronic lung disease. The right ventricle (RV) may support the pulmonary circulation, and sometimes the systemic circulation (systemic RV) in congenital heart defects. Despite major progress being made, assessing the RV remains challenging, often requiring a multi-imaging approach and expertise (echocardiography, magnetic resonance imaging, nuclear and cineangiography). Evidence is accumulating that RV dysfunction develops in many of these patients and leads to considerable morbidity and mortality. While there is extensive literature on the pathophysiology and treatment of left heart failure, the data for right-sided heart failure is scarce. Therefore RV function in certain groups of congenital heart disease patients needs close surveillance and timely and appropriate intervention to optimise outcomes. An understanding of RV physiology and hemodynamics will lead to a better understanding of current and future treatment strategies for right heart failure. This will review right-sided heart failure with the implications of volume and pressure loading of the RV in congenital heart diseases.

Congenital heart disease represents a condition commonly associated with pulmonary arterial hypertension (PAH). Eisenmenger syndrome is on the extreme end of the spectrum of PAH in the setting of congenital heart disease. This status implied the irreversibility and inoperability of a subgroup of patients with congenital heart defects. Untreated or even treated, Eisenmenger syndrome is characterized by a progressive increase in pulmonary vascular resistance that leads to right ventricular failure and death. Today, although there have been many advances in understanding of the pathophysiology of Eisenmenger syndrome and also advances in treating this disease, there is still no cure for this progressive condition, but the new vasodilators may be well tolerated and they improve the exercise capacity and hemodynamics without compromising the peripheral oxygen saturation. Eisenmenger syndrome can be prevented in most pediatric patients. With the advances in imaging, surgery and intensive care facilities and also in the early definitive therapy for the majority of lesions associated with a risk of developing Eisenmenger syndrome, a progressive decrease in the prevalence of this disease may be expected. Identifying the risk factors is problematic as the annual mortality rates are relatively low for Eisenmenger patients. We review here the available data on the pathophysiology, clinical presentation, prognosis and management of pulmonary arterial hypertension in patients with congenital heart disease. (Korean Circulation J 2007;37:287-297)

Background and Objectives:The prevalence of pediatric hypertension appears to be increasing, and secondary hypertension is more common in children than in adults. Essential hypertension is increasing in obese adolescents. An elevated blood pressure (BP) during childhood is associated with premature cardiovascular morbidity and mortality in adulthood. Oscillometric devices are convenient and minimize observer error. The purposes of this study were to measure the BP, using an oscillometric device, in relation to age, sex, weight, height and body mass index (BMI), in normal school aged children, and to identify the 95 and 99 percentile values for the diagnosis of hypertension by age group. Subjects and Methods:Between May and June 2005, the oscillometric blood pressure was measured, using a Dinamap monitor (Procare-200, GE, USA), in 9,499 Seoul school children (male 4,993, female 4,506), aged 7-18 years. The BP was measured on the right arm, in the sitting position, using a BP cuff having a width of 40-50% of the arm circumference. The mean and 5, 10, 25, 50, 75, 90, 95 and 99 percentile values and percentile curves of the systolic and diastolic BP were obtained in relation to age, sex, height, weight and BMI. Results:The systolic and diastolic BP levels were significantly increased in relation to age, height, weight and BMI. A correlation analysis showed that the BP levels were significantly correlated with weight (r=0.553), followed by height (r=0.491), BMI (r=0.470) and age (r=0.445) in males, and significantly correlated with weight (r=0.332), followed by height (r=0.317), BMI (r=0.286) and age (r=0.213) in females. Conclusion:Percentile values and percentile curves for the systolic and diastolic BP were obtained in normal children. These data will be useful in the diagnosis and treatment of pediatric hypertension. (Korean Circulation J 2006;36:744-752)