· As the number of patients with congenital heart disease increases, the medical burden increases. · Various fusion imaging techniques using percutaneous procedures have been introduced. · With advances in technology, convenient ambulatory devices have been introduced. · A well-organized team approach is required to resolve advanced heart failure in patients with congenital heart disease. |
Clustered regularly interspaced short palindromic repeats and CRISPR-associated protein 9 (CRISPR-Cas9) system has made a big step in the genome editing which still requires technical developments for the efficient applications in the many fields including congenital heart diseases (CHDs), closely related to the genomic abnormality. In this review, we tried to cover the most updated researches of CRISPR-Cas9 in the CHDs to understand the current technologies, eventually becoming therapeutic bases for the CHD patients. |
Questions: This study aimed to describe the survival of premature infants with critical congenital heart disease (CHD) and to identify the risk factors including the new modified version of the Risk Adjustment for Congenital Heart Surgery (M-RACHS) associated with mortality. Finding: For premature infants with critical CHD, survival rate was 76.9% and very low birth weight (VLBW), persistent pulmonary hypertension of the newborn (PPHN), bronchopulmonary dysplasia (BPD), and M-RACHS 5 or more were associated with in-hospital mortality. Meaning: VLBW, PPHN and BPD, as well as M-RACHS≥5, were risk factors for mortality among premature infants with critical CHD. |
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... |
The efficacy of percutaneous stent implantation for congenital heart disease (CHD) in Korea, where stent availability is limited, has not been determined. This study evaluated the acute and midterm results of stent implantation in different CHD subgroups. Stents were implanted in 75 patients with 81 lesions: (1) pulmonary artery stenosis (PAS) group, 56 lesions in 51 patients; (2) coarctation of the... |
During the last 10 years, there have been major technological achievements in pediatric interventional cardiology. In addition, there have been several advances in cardiac imaging, especially in 3-dimensional imaging of echocardiography, computed tomography, magnetic resonance imaging, and cineangiography. Therefore, more types of congenital heart diseases can be treated in the cardiac catheter laboratory today than ever before. Furthermore, lesions previously... |
Generally, aspirin is used as a protective agent against thrombogenic phenomenon after pulmonary valve replacement (PVR) using a bioprosthetic valve. However, the appropriate duration of aspirin use is unclear. We analyzed the impact of postoperative duration of aspirin use on the longevity of bioprosthetic pulmonary valves in patients who underwent repair for congenital heart diseases. We retrospectively reviewed the clinical data... |
Despite developments in surgical techniques and other interventions, right ventricular (RV) failure remains an important clinical problem in several congenital heart diseases (CHD). RV function is one of the most important predictors of mortality and morbidity in patients with CHD. RV failure is a progressive disorder that begins with myocardial injury or stress, neurohormonal activation, cytokine activation, altered gene expression,... |
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.... |
Although antenatal diagnostic technique has considerably improved, precise detection and proper management of the neonate with congenital heart disease (CHD) is always a great concern to pediatricians. Congenital cardiac malformations vary from benign to serious conditions such as complete transposition of the great arteries (TGA), critical pulmonary and aortic valvular stenosis/atresia, hypoplastic left heart syndrome (HLHS), obstructed total anomalous pulmonary... |
Purpose : Early postoperative arrhythmias are a major cause of mortality and morbidity after open heart surgery in the pediatric population. We evaluated the incidence and risk factors of early postoperative arrhythmias after surgery of congenital heart disease. Methods : From January 2002 to December 2008, we retrospectively reviewed the medical records of the 561 patients who underwent cardiac surgery in Kyungpook National University... |
Purpose : Growth impairment is usually observed in children with congenital heart disease (CHD). Studies on the final height and weight of this population are insufficient. Our aim was to evaluate the final height of children with CHD and to identify the relationship between CHD and growth. Methods : We recorded the values of final height and weights of 105 CHD... |
Purpose : Despite recent advances in pulmonary hypertension management and surgery, appropriate guidelines remain to be developed for operability in congenital heart disease with pulmonary artery hypertension (PAH). Our aim was to evaluate clinical outcomes of patients with severe PAH who underwent surgical closure of left-to-right shunt lesions (LRSL) on the basis of pulmonary reactivity. Methods : We retrospectively reviewed 21... |
Purpose : To investigate the nature of deviant voice physiology in preoperative children with congenital heart disease. Methods : Ninety-four children with congenital heart disease were enrolled. Their cries and related acoustic variables (fundamental frequency, duration, noise to harmonic ratio, jitter¢¬ and shimmer) were analyzed using a multi-dimensional voice program. Results : The average fundamental frequency showed a significant decrease in patent... |
Purpose : To analyze abnormal ventricular activation in childhood congenital heart disease induced by postoperative changes in ventricular volume and pressure and ventricular scar formation using signal-averaged electrocardiography (SAECG). Methods : Fifty-two patients who had undergone open heart surgery (OHS) were enrolled. Patients were divided into the following 3 groups: right ventricular volume overload (atrial septal defect, group1), left ventricular... |
Plastic bronchitis is a rare disorder characterized by the formation of extensive, obstructing endobronchial casts. It is associated with asthma and complex cardiac defects such as those requiring the Fontan procedure. The treatment of plastic bronchitis comprises conventional therapy involving spontaneous expectoration and bronchoscopic removal and specific therapy with several new drugs. Herein, we describe the cases of 2 patients... |
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... |
Purpose : The objective of this study was to assess ventricular function by tissue Doppler imaging (TDI) in children with congenital heart disease (CHD) who have been undergoing open heart surgery (OHS) using cardiopulmonary bypass. We tried to compare the parameters of tissue Doppler imaging before and after OHS in patients with congenital heart disease. Methods : This study was conducted... |
Purpose : This study was performed to assess how a fetal diagnosis of congenital heart disease affects parents, as regards pregnancy management and care of infants after birth. Methods : Database search to find out abnormal fetal echocardiography performed at Seoul National University Children's Hospital from July 1988 to June 2003 revealed 370 examinations. After excluding both arrhythmias without structural cardiac... |
Purpose : The purpose of this study was to investigate the relationship between depression and resilience in adolescents with congenital heart disease(CHD) and to identify the variables associated with depression. Methods : The Resilience Scale(cronbach’s α=0.92), Children's Depression Inventory(cronbach’s α= 0.72) and Maternal Behavior Research Instrument(cronbach’s α=0.88) were applied and analyzed to assess depression and resilience among 231 adolescents after... |
Purpose : Aneurysms of sinus valsalva are rare anormalies thought to be primarily congenital in origin, progressing into death by acute heart failure in cases of rupture. Surgical correction is the only method of treatment. With these clinical implications, we reviewed the clinical characteristics and surgical results of patients with ruptured sinus of valsalva aneurysm. Methods : Between January 1991... |
Purpose : This study was undertaken to learn the usefulness, cost-effectiveness and safety of intraoperative transesophageal echocardiography(ITEE) during open heart surgery for congenital heart diseases. Methods : The usefulness and safety of ITEE were assessed in 254 patients who underwent open heart surgery for congenital heart diseases from January 1, 2001 to June 30, 2003, with retrospective chart reviews and a... |
Purpose : This is a retrospective descriptive study to determine the incidence of congenital heart disease(CHD) in Jeju and to estimate the suitability of the Jeju population as the subject for a regional birth cohort study. Methods : All patients with CHD diagnosed by two dimensional echocardiography in Cheju National University Hospital and Halla General Hospital from January 1999 to... |
Isolated right ventricular hypoplasia, unassociated with severe pulmonary or tricuspid valvar malformations, is a rare primary congenital cardiac anomaly in which of the trabecular portion of right ventricle fails to develop. An atrial septal defect or a patent foramen ovale serves as an escape valve. We observed a 2-day-old neonate with this disorder who suffered from cyanosis. The diagnosis was... |
Purpose : To assess wall motion of the left ventricle in the various forms of congenital heart disease and to select appropriate methods by which to gauge left ventricular wall motion. Methods : We evaluated left ventricular function, including volume, global ejection fraction, and regional wall motion(Centerline method, Local Ejection Fraction, Curved Perimeter method and Radial method). The evaluation was done... |
Purpose : In acyanotic congenital heart disease of children with left to right shunt, 99mTc- Macroaggregate albumin(MAA) was employed to determine the scintigraphic severity of pulmonary parenchymal damage. Methods : These data of lung perfusion scan using 99mTc-MAA were compared with hemodynamic values obtained from cardiac catheterization. Results : The mean Pulmonary arterial pressure(≥30mmHg), total pulmonary resistance(≥2 Wood unit)... |
Purpose : Many reports exist about the frequency of congenital heart disease(CHD) in the world, but according to the definition of CHD and subjects of study group, many differences existed between the reports. We conducted this study to evaluate the incidence and frequency of CHD by color Doppler echocardiography in neonate, and to suggest a preliminary model of incidence of... |
Purpose : In order to find out how frequently and what kind of extra-cardiac anomalies (CAs) is associated with congenital heart disease(CHD) in general and in each CAs, autopsies with congenital cardiovascular malformations were studied. Methods : Autopsy files of Seoul National University Children' s Hospital from 1991 to 1995 were reviewed to find cases of CHDs. Other extra-CAs were tabulated from the records... |
Purpose : The proportion of interventional catheterization in congenital heart disease is being increased in Korea, especially in Sejong Heart Institute, so we performed statistical analysis on the cardiac catheterization cases. Methods : Total 233 cases of congenital heart disease confirmed by cardiac catheterization at the Sejong Heart Institute between Jaunary 1995 and June 1995 were analyzed retrospectively. Results : 1) The... |
Purpose : Fetal echocardiography is used for the prenatal diagnosis of congenital heart disease. Fetal echocardiography allows decisions to be made in advance where the fetal abnormality is not compatible with life after delivery or where early correction is required. Our fetal echocardiographic experience was analysed retrospectively to guide future clinical application. Methods : 138 mothers had fetal echocardiography from April 1992 to December... |