Retrospective Multicenter Study of Respiratory Syncytial Virus Prophylaxis in Korean Children with Congenital Heart Diseases

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 <1 year of age with hemodynamic significance CHD (HS-CHD). SUBJECTS AND METHODS: During the RSV seasons in 2009-2015, we performed a retrospective review of data for 466 infants with CHD, examined at six centers in Korea. RESULTS: Infants received an average of 3.7±1.9 (range, 1-10) injections during the RSV season. Fifty-seven HS-CHD patients (12.2%) were hospitalized with breakthrough RSV bronchiolitis, with a recurrence in three patients, one year after the initial check-up. Among patients with simple CHD, only five (1.1%) patients received one additional dose postoperatively, as per the limitations set by the Korean guideline. Among the 30 deaths (6.4%), five (1.1%) were attributed to RSV infection; three to simple CHD, one to Tetralogy of Fallot, and one to hypertrophic cardiomyopathy (HCM). Of the three HCM patients that exceeded guidelines for RSV prophylaxis, two (66.6%) were hospitalized, and one died of RSV infection (33.3%). CONCLUSION: In accordance to the Korean guideline, minimal injections of palivizumab were administered to patients having HS-CHD

An Adolescent Patient with Coarctation of Aorta Treated with Self-Expandable Nitinol Stent

Transcatheter treatment of aortic coarctation, with balloon angioplasty or stent implantation, is now an acceptable alternative to surgical repair. However these procedures may result in complications, such as vascular wall injury and re-stenosis of the lesion. A nitinol self-expandable stent, when deployed at the coarctation site, produces low constant radial force, which may result in a gradual widening of the stenotic lesion leaving less tissue injury ('stretching rather than tearing'). For an adolescent with a native aortic coarctation, a self-expandable stent of 20 mm diameter was inserted at the discrete stenotic lesion of 5 mm diameter without previous balloon dilatation procedure. No further balloon dilatation was done immediately after the stent insertion. With the self-expandable stent only, the stenosis of the lesion was partially relieved immediately after the stent deployment. Over several months after the stent insertion, gradual further widening of the stent waist to an acceptable dimension was observed.

The results of cardiopulmonary exercise test in healthy Korean children and adolescents: single center study / 소아과

PURPOSE: The cardiopulmonary exercise test (CPET) is an important clinical tool for evaluating exercise capacity and is frequently used to evaluate chronic conditions including congenital heart disease. However, data on the normal CPET values for Korean children and adolescents are lacking. The aim of this study was to provide reference data for CPET variables in children and adolescents. METHODS: From August 2006 to April 2009, 76 healthy children and adolescents underwent the CPET performed using the modified Bruce protocol. Here, we performed a medical record review to obtain data regarding patient' demographics, medical history, and clinical status. RESULTS: The peak oxygen uptake (VO2Peak) and metabolic equivalent (METMax) were higher in boys than girls. The respiratory minute volume (VE)/CO2 production (VCO2) slope did not significantly differ between boys and girls. The cardiopulmonary exercise test data did not significantly differ between the boys and girls in younger age group (age, 10 to 14 years). However, in older age group (age, 15 to 19 years), the boys had higher VO2Peak and METMax values and lower VE/VCO2 values than the girls. CONCLUSION: This study provides reference data for CPET variables in case of children and adolescents and will make it easier to use the CPET for clinical decision-making.

Transcatheter Closure of Secundum Atrial Septal Defect in Patients Over 60 Years Old

BACKGROUND AND OBJECTIVES: Atrial septal defect (ASD) is the one of most common congenital heart diseases detected in adults. Along with remarkable development of device technology, the first treatment strategy of secundum ASD has been transcatheter closure in feasible cases. However, there are only a few publications regarding the results of transcatheter closure of ASD in elderly patients, especially those over 60 years of age. We report our results of transcatheter closure of ASD in patients over 60 years old. SUBJECTS AND METHODS: Between May 2006 and December 2011, 31 patients over 60 years old (25 female and 6 male; mean 66.7+/-5.25 years old, range 61-78 years old) were referred to our center. RESULTS: A total of 23 patients underwent therapeutic catheterization to close secundum ASD, and the closure was successful in 22 patients (95.7%). All patients who underwent the procedure survived except for one patient who expired because of left ventricular dysfunction. A small residual shunt was observed in two (9%) of 21 patients before discharge but disappeared at follow-up. All patients eventually had complete closure. There were five patients who had coronary problems. One patient underwent percutaneous coronary intervention using a stent at the same time as transcatheter closure of ASD. Atrial arrhythmias were detected in 6 of 23 patients (26.1%) before the procedure. One patient was successfully treated by radiofrequency ablation before the procedure. No patients displayed new onset arrhythmia during the follow-up period. Follow-up echocardiographic evaluation showed a significantly improved right ventricular geometry. CONCLUSION: We conclude that transcatheter closure of ASD is a safe and an effective treatment method for patients over 60 years old if the procedure is performed under a thorough evaluation of comorbidities and risk factors.

Transcatheter Closure of Secundum Atrial Septal Defect in Patients Over 60 Years Old

BACKGROUND AND OBJECTIVES: Atrial septal defect (ASD) is the one of most common congenital heart diseases detected in adults. Along with remarkable development of device technology, the first treatment strategy of secundum ASD has been transcatheter closure in feasible cases. However, there are only a few publications regarding the results of transcatheter closure of ASD in elderly patients, especially those over 60 years of age. We report our results of transcatheter closure of ASD in patients over 60 years old. SUBJECTS AND METHODS: Between May 2006 and December 2011, 31 patients over 60 years old (25 female and 6 male; mean 66.7+/-5.25 years old, range 61-78 years old) were referred to our center. RESULTS: A total of 23 patients underwent therapeutic catheterization to close secundum ASD, and the closure was successful in 22 patients (95.7%). All patients who underwent the procedure survived except for one patient who expired because of left ventricular dysfunction. A small residual shunt was observed in two (9%) of 21 patients before discharge but disappeared at follow-up. All patients eventually had complete closure. There were five patients who had coronary problems. One patient underwent percutaneous coronary intervention using a stent at the same time as transcatheter closure of ASD. Atrial arrhythmias were detected in 6 of 23 patients (26.1%) before the procedure. One patient was successfully treated by radiofrequency ablation before the procedure. No patients displayed new onset arrhythmia during the follow-up period. Follow-up echocardiographic evaluation showed a significantly improved right ventricular geometry. CONCLUSION: We conclude that transcatheter closure of ASD is a safe and an effective treatment method for patients over 60 years old if the procedure is performed under a thorough evaluation of comorbidities and risk factors.

Respiratory syncytial virus infection cases in congenital heart disease patients / 소아과

PURPOSE: Respiratory syncytial virus (RSV) is one of the main pathogens causing lower respiratory infections (LRI) in young children, usually of limited severity. However, in congenital heart disease (CHD) patients, one of the high-risk groups for RSV infection, RSV can cause serious illnesses and fatal results. To elucidate the effects of RSV infection in CHD patients, we observed RSV infection cases among CHD patients and non-CHD patients. METHODS: On admission of 343 LRI patients over 3 years, 77 cases of RSV infection were detected by the RSV antigen rapid test of nasopharyngeal secretion. We compared RSV infection cases among groups of CHD and non-CHD patients. RESULTS: During the winter season, RSV caused 20-50% of LRI admissions in children. In patients with completely repaired simple left to right (L-R) shunt diseases such as ventricular septal defect, atrial septal defect, and patent ductus arteriosus, RSV infections required short admission days similar to non-CHD patients. In patients with repaired CHD other than simple L-R shunt CHD, for whom some significant hemodynamic problems remained, RSV infection required long admission days with severe clinical course. In children with unrepaired CHD, RSV infection mostly occurred in early infant age, with long admission days. RSV infections within a month after cardiac surgery also required long admission days and severe clinical course. CONCLUSION: To avoid the tragedic outcome of severe RSV infection in the CHD patients, efforts to find the subgroups of CHD patients at high risk to RSV infection are needed, and effective preventive treatment should be applied.

The Recent Surgical Result of Total Anomalous Pulmonary Venous Return

BACKGROUND AND OBJECTIVES: Recent surgical results from total anomalous pulmonary venous return (TAPVR) are reported to have improved; however, mortality and morbidity are still high in the univentricles. This review was performed in order to demonstrate surgical results from TAPVR for recent years in a single institute. SUBJECTS AND METHODS: A total of 53 patients with TAPVR underwent surgery between January 2000 and December 2008. Preoperative anatomy and hemodynamics were evaluated by 2-dimensional echocardiogram. We reviewed medical records on preoperative management, age, and body weight at time of surgery, postoperative mortality, and pulmonary venous obstruction. RESULTS: The study included 36 biventricle patients and 17 univentricle patients. Median age and weight at time of surgery were 26 days and 3.5 kg, respectively. During the mean follow up period of 48.2 months, a total of 11 deaths and 8 early deaths occurred. Postoperative pulmonary venous stenosis occurred in 8 patients. Among 36 biventricle patients, mortality occurred in 1 patient and postoperative pulmonary venous stenosis occurred in 6 patients. No statistical significances were observed between postoperative pulmonary venous stenosis and other factors. Among 17 univentricule patients, mortality was 10, including 7 early deaths, and postoperative pulmonary venous stenosis occurred in 2 patients. Combined palliations were modified Blalock-Taussig (BT) shunts in 7, pulmonary artery banding (PAB) in 3, bidirectional cavopulmonary shunt in 5, and the Fontan procedure in 1. The highest mortality was recorded in patients with a modified BT shunt (85.7%) and next in patients with PAB (66.6%). Mortality was not related to anatomy of TAPVR, nor preoperative obstruction, but with significant age and weight at time of surgery. CONCLUSION: Despite improvement in operative results from TAPVR, surgery in univentricles is highly risky, especially with low weight and early age, with concomitant palliation.

Outcome after Fontan Operation in Korea

PURPOSE: Despite extracardiac conduit Fontan had many advantages, long-term results related to longevity of conduit, and anticoagulation were not proven. This study was to evaluate the long-term outcome of hospital survivors with extracardiac Fontan circulation. METHODS: Between 1996 and 2006, 200 patients underwent extracardiac conduit Fontan operation. Median age at the Fontan operation was 3.4 years (range:16 months-35.7 years). All patients (89.5 %) except 21 patients of one stage Fontan operation underwent bi-directional cavopulmonary shunt. Fenestration was required in 85 patients (42.5%). RESULTS: There has been 6 hospital mortalities (3.0%), and 7 late mortalities (3.6%) at a mean follow-up of 52.4+/-32.2 months (range; 18days-120 months). Overall 10-years survival was 92.4 2.1%. Multivariate analysis identified severe infection at early postoperative periods (hazard ratio =12.439, P=0.001), and high pulmonary arterial pressure at preoperative period (hazard ratio=3.445, P=0.038) as risk factors for mortality. Reoperation was performed in 24 patients (12.0%), and freedom from reoperation was 82.4+/-4.1% at 10 years. Arrhythmia occurred in 32 patients (16.0%) after Fontan operation, and freedom from arrhythmia was 85.14.4% at 10 years. Risk factors for arrhythmia were heterotaxy syndrome (P=0.001), Follow up duration (P=0.027) and the age at Fontan operation (P=0.001). Freedom from thromboembolism was 92.91.9% at 10 years. The conduit cross- sectional area decreased by 14%, and the extent of decrease of the conduit cross-sectional remained stable irrespective of the follow-up duration. 95.2% of patients had New York Heart Association class I. CONCLUSIONS: After 10 years of follow-up, the overall survival, and the functional status of survivors of the extracardiac Fontan procedure are satisfactory. Fenestration has a beneficial effect on the results of high-risk Fontan patients. The incidence of late deaths, reoperations, obstructions of the cavopulmonary pathway, arrhythmias and thromboembolisms is low.

A case of constrictive pericarditis presenting with protein-losing enteropathy / 소아과

Constrictive pericarditis represents a rare cause of protein-losing enteropathy in children. Reported is an 11-year-old girl with protein-losing enteropathy (PLE) as the principal manifestations of constrictive pericarditis. After total pericardiectomy, symptoms and signs of PLE disappeared. Doppler echocardiography including tissue Doppler imaging is a useful noninvasive initial diagnostic tool for differential diagnosis of diastolic heart failure.

Transcatheter Closure of a Large Coronary Arteriovenous Fistula Using an Amplatzer Vascular Plug

We report the successful percutaneous closure of a large coronary fistula from the right coronary artery to the right ventricle in a 4-years-old boy using the Amplatzer vascular plug. The device was positioned and deployed via the arterial system.It allows a smaller delivery profile through small sheath or guide catheters and the technique appeared to be easy, safe, and effective.

N-terminal Pro-B-type Natriuretic Peptide as a Predictive Risk Factor in Fontan Operation / 소아과

PURPOSE: This study aimed to investigate the correlation between the plasma level of N-terminal pro-B-type natriuretic peptide (pro-BNP) and several known risk factors influencing outcomes after Fontan operations, and to assess whether pro-BNP levels can be used as predictive risk factors in Fontan operations. METHODS: Plasma pro-BNP concentrations were measured in 35 patients with complex cardiac anomalies before catheterization. Cardiac catheterization was performed in all subjects. Mean right atrium pressure, mean pulmonary artery pressure (PAP), and ventricular end-diastolic pressure (EDP) were obtained. Cardiac output and pulmonary vascular resistance were calculated by Fick method. RESULTS: Plasma pro-BNP levels exhibited statistically significant positive correlations with mean PAP (r=0.70, P< 0.001), pulmonary vascular resistance (r=0.57, P< 0.001), RVEDP (r=0.63, P< 0.001), LVEDP (r=0.74, P< 0.001), and cardiothoracic ratio (r=0.71, P< 0.001). The area under the ROC curve using pro-BNP level to differentiate risk groups in Fontan operations was high: 0.868 (95 percent CI, 0.712-1.023, P< 0.01). The cutoff value of pro-BNP concentrations for the detection of risk groups in Fontan operations was determined to be 332.4 pg/mL (sensitivity 83.3 percent, specificity 82.7 percent). CONCLUSION: These data suggest that plasma pro-BNP levels may be used as a predictive risk factor in Fontan operations, and as a guide to determine the mode of therapy during follow-up after Fontan operations.

A case of localized persistent interstitial pulmonary emphysema

Interstitial pulmonary emphysema is a well-documented complication of assisted mechanical ventilation in premature infants with respiratory distress syndrome. Localized persistent interstitial pulmonary emphysema (LPIPE) confined to a single lobe was incidentally presented in a 4-day-old female infant. This patient was a normal full-term baby with no respiratory distress symptom and no experience of assisted mechanical ventilation. Chest radiograph showed radiolucent area in right lower lobe zone, which needed differential diagnosis from other congenital lesions such as congenital cystic adenomatoid malformation and congenital lobar emphysema. CT scan showed irregular-shaped air cystic spaces and pathologically, cystic walls primarily consisted of compressed lung parenchyma and loose connective tissue intermittently lined by multinucleated foreign body giant cells.

The Use of Quadruple Blood Bags Transfusion of Low Birth Weight Neonates

PURPOSE: Low-birth-weight(LBW) neonates often need multiple transfusions during their neonatal period. The routine use of the 500ml mono-bag system in Korea, even in neonatal transfusions, results in a great waste of blood and exposes the neonate to multiple blood donors. To reduce the waste of blood and the number of exposures, we used quadruple blood bags in the transfusion of LBW neonates. METHODS: From November 1995 to April 1997, 86 neonates weighing less than 2,000gm were enrolled in this study. Forty-four of those neonates needed blood transfusions. Using quadruple blood bags, we were able to safely make 3 units of packed red blood cell(RBC) from each directed donors. RESULTS: Seventy-seven units of packed RBC could be made from 29 directed donors and 58 of those units were actually transfused to the 25 neonates. Blood age of the directed donor blood prepared in the quadruple blood bags were relatively older than those of bank bloods at the time of transfusion, thus, K+ concentration was higher but the total K+ amounts infused through transfusions were within tolerable ranges in both groups. CONCLUSION: Using quadruple blood bag in the transfusion of LBW neonates, we could reduce the number of donor exposures and also the waste of blood. K+ loads to the neonates through the transfusion of directed donor blood were within tolerable range in the neonatal physiology.

Recent Trend of Increasing Proportion of Interventional Catheterization in Congenital Heart Disease

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 distribution of age has become more younger than that of previous reports that is, the proportion of the infant less than 1 year old was 34.8%. 2) The proportion of male sex was equal to that of female. TOF and TGA were observed predominantly in male patients, and PDA and UVH revealed female preponderance. 3) The relative frequencies of each individual congenital heart disease in order of frequency were as follows; TOF 37.3%, PDA 13.3%, VSD 8.2% and UVH 7.7%. 4) Among the 145 (62%) cases of hemodynamic study pre-operation and post-operation cases were 85 and 60 cases, respectively. The cases of interventional catheterization were 84 (36%), and those of endomyocardial biopsy were 4 (2%). 5) The frequencies of each interventional catheterization of 84 cases in order of frequency were as follows: defect closure 28 cases, balloon valvuloplasty 20 cases, balloon angioplasty 17 cases, coil embolization 15 cases, and balloon atrial septostomy 4 cases. 6) There were 2 cases of mortality associated with cardiac catheterization. CONCLUSIONS: The proportion of interventional catheterization in congenital heart disease is being increased in the Sejong Heart Institute, and this trend will spread to the other cardiac center in Korea.

A Family with -D- Phenotype Associated with Fatal Hemolytic Disease of the Newborn / 대한수혈학회지

-D- is a very rare haplotype which determines D without C, c, E or e and exalted D activity. The extremely rare homozygote propositi(-D-/-D-) are usually ascertained through their immune antibodies which react with red cells of all common Rh phenotypes. Authors experienced a woman with -D- phenotype for the first time in Korea. She had a history of abortion and intrauterine fetal death. She delivered a baby with severe hemolytic disease of the newborn at the third pregnancy. In spite of intensive medical interventions, the baby died of hydrops fetalis. An immune antibody to high incidence Rh antigen, namely anti-Hro, was demonstrated in the woman's serum. Family study revealed that all of the family had -D- gene complex and one of her sisters also was -D-homozygote. The sister also had anti-Hro in the serum.

A Case of Hernolytio Disease of the Newborn due to Anti-Dib Antibody / 대한수혈학회지

The Di(a+b-) phenotype is extremely rare among Caucaclans and mostly confined to mongoloids. The incidences of Di antigen among Koreans were reported to be 6.1 to 14.5%, with an average of 9.6%; it can be calculated that the frequency of Di(a+b-) is 0.25% on the average. Authors report the first case of hemolytic disease of the newborn due to anti-Dib in Korea. The Diego phenotypes of the mother, baby and father were Di(a+b-), Di(a+b+) and Di (a-b+), respectively. The mother's serum and eluate from infant's erythrocytes contained anti-DP antibody active in the antiglobulin phase. After transfusions of Di-negative red cells from the mother and mother's relative, the patient was recovered from anemia and jaundice.

Early neonatal Mortality Rate in Newborn Nursery

No abstract available.