OBJECTIVES: To provide reference basis for reducing the mortality for children under 5 years old and promote the healthy development, the mortality for children under 5 years old and the main causes for death in Liuyang City from 2013 to 2020 are analyzed. METHODS: The data of 725 cases of death for children under 5 years old in Liuyang City from 2013 to 2020 were collected.The causes and difference of death among the children were analyzed retrospectively by descriptive statistic methods. RESULTS: There were a total of 144 516 live births in Liuyang City from 2013 to 2020. The mortality for children under 5 years old was 5.01‰, for infants was 3.39‰, and for newborns was 1.63‰. The male child mortality was 5.28‰, and the female child mortality rate was 4.72‰, with significant difference (P>0.05). The mortality for children under 5 years old was seasonal fluctuation, without significant difference among seasons (P>0.05). For the past 5 years, the top 3 causes for death among children under 5 years old were preterm birth and low birth weight, congenital heart disease, and pneumonia. Before death, 341 cases (47.04%) were treated in provincial hospitals, 198 cases (27.31%) in county-level hospitals, 56 cases (7.72%) in village-level hospitals, and 130 cases (17.93%) were not treated. CONCLUSIONS The mortality for children under 5 years old in Liuyang City is gradually reduced in the past 5 years. The main causes for death are premature birth and low birth weight, congenital heart disease and pneumonia. We should develop healthy education, improve the rate of prenatal diagnosis, promote the construction of obstetrics and paediatrics, and fundamentally reduce the mortality for children under 5 years old.
Cause of Death ; Child ; Child Mortality ; Child, Preschool ; Female ; Heart Defects, Congenital ; Humans ; Infant ; Infant Mortality ; Infant, Newborn ; Male ; Pneumonia/epidemiology* ; Pregnancy ; Premature Birth ; Retrospective Studies

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.
Aorta, Thoracic ; Aortic Coarctation ; Arrhythmias, Cardiac ; Arteries ; Birth Rate ; Echocardiography ; Fetus ; Heart Defects, Congenital ; Heart Diseases ; Hypoplastic Left Heart Syndrome ; Parturition ; Prenatal Diagnosis ; Pulmonary Atresia ; Surgeons ; Tetralogy of Fallot

The number of women with congenital heart disease (CHD) at risk of pregnancy is growing because over 90% of them are grown-up into adulthood. The outcome of pregnancy and delivery is favorable in most of them provided that functional class and systemic ventricular function are good. Women with CHD such as pulmonary hypertension (Eisenmenger syndrome), severe left ventricular outflow stenosis, cyanotic CHD, aortopathy, Fontan procedure and systemic right ventricle (complete transposition of the great arteries [TGA] after atrial switch, congenitally corrected TGA) carry a high-risk. Most frequent complications during pregnancy and delivery are heart failure, arrhythmias, bleeding or thrombosis, and rarely maternal death. Complications of fetus are prematurity, low birth weight, abortion, and stillbirth. Risk stratification of pregnancy and delivery relates to functional status of the patient and is lesion specific. Medication during pregnancy and post-delivery (breast feeding) is a big concern. Especially prescribing medication with teratogenicity should be avoidable. Adequate care during pregnancy, delivery, and the postpartum period requires a multidisciplinary team approach with cardiologists, obstetricians, anesthesiologists, neonatologists, nurses and other related disciplines. Caring for a baby is an important issue due to temporarily pregnancy-induced cardiac dysfunction, and therefore familial support is mandatory especially during peripartum and after delivery. Timely pre-pregnancy counseling should be offered to all women with CHD to prevent avoidable pregnancy-related risks. Successful pregnancy is feasible for most women with CHD at relatively low risk when appropriate counseling and optimal care are provided.
Adult ; Arrhythmias, Cardiac ; Arteries ; Constriction, Pathologic ; Counseling ; Female ; Fetus ; Fontan Procedure ; Heart Defects, Congenital ; Heart Failure ; Heart Ventricles ; Hemorrhage ; Humans ; Hypertension, Pulmonary ; Infant, Low Birth Weight ; Infant, Newborn ; Maternal Death ; Peripartum Period ; Postpartum Period ; Pregnancy ; Stillbirth ; Thrombosis ; Ventricular Function

The 16p11.2 microdeletion has been reported in patients with developmental delays and intellectual disability. The distal 220-kb deletion in 16p11.2 is associated with developmental delay, autism spectrum disorder, epilepsy, and obesity at a young age. We have reported a case of distal 16p11.2 deletion syndrome in a preterm infant with unusual facial morphology and congenital heart disease. We suggest using chromosome microarray analysis to detect chromosomal abnormalities in newborns, especially preterm infants with unusual morphologies.
Autism Spectrum Disorder ; Chromosome Aberrations ; Epilepsy ; Gene Deletion ; Heart Defects, Congenital ; Humans ; Infant, Newborn ; Infant, Premature* ; Intellectual Disability ; Microarray Analysis ; Obesity ; Premature Birth

BACKGROUND: Patent ductus arteriosus (PDA) ligation is usually performed by congenital cardiac surgeons. However, due to the uneven distribution of congenital cardiac surgeons in South Korea, many institutions depend solely on adult cardiac surgeons for congenital cardiac diseases. We report the outcomes of PDA ligations performed by adult cardiac surgeons at our institution. METHODS: The electronic medical records of 852 neonates at Chung-Ang University Hospital, Seoul, South Korea from November 2010 to May 2014 were reviewed to identify patients with PDA. RESULTS: Of the 111 neonates with a diagnosis of PDA, 26 (23%) underwent PDA ligation. PDAs were ligated within 28 days of birth (mean, 14.5±7.8 days), and the mean gestational age of these patients was 30.3±4.6 weeks (range, 26 to 40 weeks) with a mean birth weight of 1,292.5±703.5 g (range, 480 to 3,020 g). No residual shunts through the PDA were found on postoperative echocardiography. There was 1 case of 30-day mortality (3.8%) due to pneumonia, and 6 cases of in-hospital mortality (23.1%) after 30 days, which is comparable to results from other centers with congenital cardiac surgery programs. CONCLUSION: Although our outcomes may not be generalizable to all hospital settings without a congenital cardiac surgery program, in select centers, PDA ligations can be performed safely by adult cardiac surgeons if no congenital cardiac surgery program is available.
Adult* ; Birth Weight ; Cardiac Surgical Procedures ; Diagnosis ; Ductus Arteriosus, Patent* ; Echocardiography ; Electronic Health Records ; Gestational Age ; Heart Defects, Congenital ; Heart Diseases ; Hospital Mortality ; Humans ; Infant, Newborn ; Korea ; Ligation* ; Mortality ; Parturition ; Pneumonia ; Seoul ; Surgeons* ; Thoracic Surgery

PURPOSE: We investigated the clinical course of infants with congenital heart disease (CHD) who experienced thyroid dysfunction within 100 days of birth. METHODS: We performed retrospective medical reviews of 54 CHD patients (24 male patients) who underwent a thyroid function test (TFT) between January 2007 and July 2016. Data were collected on birth history, diagnosis of CHD, underlying chromosomal or genetic abnormalities, medication history, surgery, ventilator care, and exposure to iodine contrast media (ICM). Results of neonatal screening tests (NSTs) and TFTs were reviewed. RESULTS: A total of 36 patients (29 transient, 7 permanent) showed thyroid dysfunction. Among the seven patients with permanent hypothyroidism, three had an underlying syndrome, three showed abnormal NST results, and one was admitted to the intensive care unit for macroglossia and feeding cyanosis. We found that infants with transient thyroid dysfunction had a lower birth weight and were more commonly exposed to thyroid disrupting medication and/or ICM. However, these risk factors were not significant. A total of 8 patients with a history of ICM exposure showed thyroid dysfunction. Excluding 3 patients with elevated thyroid stimulating hormone before ICM exposure, 5 patients recovered from transient thyroid dysfunction. CONCLUSIONS: We observed thyroid dysfunction in two-thirds of CHD infants (53.7% transient, 13.0% permanent) who had risk factors and received TFT screening within 100 days, despite normal NSTs. Further studies with larger sample sizes are required to revise the criteria for TFT screening in CHD infants.
Birth Weight ; Contrast Media ; Cyanosis ; Diagnosis ; Heart ; Heart Defects, Congenital* ; Humans ; Hypothyroidism ; Infant* ; Infant, Newborn ; Intensive Care Units ; Iodine ; Macroglossia ; Male ; Mass Screening ; Neonatal Screening ; Parturition ; Reproductive History ; Retrospective Studies ; Risk Factors ; Sample Size ; Thyroid Function Tests ; Thyroid Gland* ; Thyrotropin ; Ventilators, Mechanical

OBJECTIVE: To analyze the main causes and characteristics for death among children under 5 years old so as to provide the basis for taking strategic measures for child survival, development, protection.
 METHODS: The data of 5 747 dead children, who were under 5 years old in different cities of Hunan Province in 2013, were retrospectively analyzed. Descriptive statistic methods were used to analyze the causes and characteristics of death among the children. Chi-square test was used to analyze the differences of mortality rate.
 RESULTS: In 2013, the under-5 mortality rate (U5MR) was 7.00‰. By linear trend chi-square test, the U5MR from 2002 to 2013 showed a tendency toward decrease (P<0.05). Among the death causes, drowning was the major one and it accounted for 13.35% of death, following by congenital heart disease, premature birth or low birth weight, pneumonia and accidental suffocation, with a ratio at 12.65%, 11.66%, 10.82%, and 8.13%, respectively. After chi-square test, there were differences in U5MR in the cities of Hunan Province in 2013 (χ2=163.185, P<0.05).
 CONCLUSION Compared with previous data, the U5MR in 2013 is decreased significantly. There are differences in U5MR in the cities, and there are also difference in U5MR between city and rural area in Hunan Province in 2013. Among the death causes for children under 5 years old in Hunan Province in 2013, drowning is in the major one, and pneumonia is the fourth one.
Cause of Death ; Child, Preschool ; Female ; Heart Defects, Congenital ; Humans ; Infant ; Infant, Newborn ; Infant, Very Low Birth Weight ; Pneumonia ; Pregnancy ; Premature Birth ; Retrospective Studies

OBJECTIVETo understand the age-specific and cause-specific mortality rate among children under 5 years old in Beijing from 2003 to 2012.

METHODSDeath surveillance data of children under the age of 5 were obtained from Beijing children mortality surveillance network from 2003 to 2012. Neonatal mortality rate (NMR), infant mortality rate (IMR), under 5-year old children mortality rate (U5MR) and the leading cause of death for under 5-year old children in urban, suburbs, and outer suburbs in Beijing were analyzed.

RESULTSThe NMR, IMR and U5MR in Beijing were 2.08 (253/121 747), 3.11 (379/121 747) and 3.57 (435/121 747) per 1000 live births in 2012, respectively, which declined 54.88%, 50.24% and 54.75% compared with the level in 2003 respectively. The children mortality rates showed a decreasing trend in urban, suburb, and outer suburbs during 2003 and 2012 (NMR was decreased from 0.53%, 0.42%, and 0.48% in 2003 to 0.20%, 0.19%, and 0.23% in 2012; IMR was decreased from 0.73%, 0.58%, and 0.63% in 2003 to 0.30%, 0.29%, and 0.35% in 2012; U5MR was decreased from 0.90%, 0.72%, and 0.82% to 0.33%, 0.34%, and 0.39% in 2012, P < 0.01). There was a steady decline in the U5MR due to congenital heart disease, birth asphyxia, premature birth or low birth weight and traffic accident in Beijing from 2003 to 2012. The mortality rate of congenital heart disease declined from 140.63 to 41.89 per 100 000 live births, birth asphyxia declined from 109.38 to 59.96 per 100 000 live births, premature birth or low birth weight declined from 85.94 to 52.57 per 100 000 live births, traffic accident declined from 26.04 to 6.57 per 100 000 live births (P < 0.01). The mortality rate of congenital heart disease declined remarkably from 216.56 to 52.47, from 119.75 to 23.50, and from 134.58 to 63.11 per 100 000 live births in urban, suburb, and outer suburbs(P < 0.01). Six of the top 8 leading causes of death among children under 5 years old declined remarkably in rural areas. They were congenital heart disease, birth asphyxia, premature birth or low birth weight, traffic accident, drowning, and septicemia, and the mortality rate of them declined from 134.58 to 63.11, from 127.85 to 65.54, from 100.94 to 60.69, from 33.65 to 12.14, from 33.65 to 0.00, and from 26.92 to 4.85 per 100 000 live births, respectively (P < 0.05). There was no drowning death case in rural areas in recent 4 years. The top 5 leading causes of death among children under 5 years old in Beijing in 2012 were birth asphyxia, premature birth or low birth weight, congenital heart disease, pneumonia, and accidental suffocation. The mortality rate of these top 5 leading causes were 59.96, 52.57, 41.89, 24.64, and 15.61 per 100 000 live births in 2012.

CONCLUSIONFrom 2003 to 2012, the NMR, IMR, U5MR and mortality rate of congenital heart disease declined remarkably in urban, suburb, and outer suburb areas in Beijing. There was a decrease trend for the six of the top 8 leading causes of death among children under 5 years old. The mortality rate of drowning dropped markedly in outer suburbs.

Accidents, Traffic ; Asphyxia ; Asphyxia Neonatorum ; Cause of Death ; Child Mortality ; Child, Preschool ; China ; Drowning ; Female ; Heart Defects, Congenital ; Humans ; Infant ; Infant Death ; Infant Mortality ; Pneumonia ; Pregnancy ; Premature Birth ; Rural Population ; Sepsis ; Suburban Population ; Urban Population

PURPOSE: Although discussion about active treatment of trisomy 18 is increasing, there are no previous articles regarding this subject in Korea. In order to provide objective data about the clinical characteristics and survival of patients with trisomy 18, based on the treatment policy, to medical teams and parents with trisomy 18, we reviewed the medical records of such patients at a single center in Korea. METHODS: This is a retrospective study of 22 patients diagnosed with trisomy 18 at the Samsung Medical Center between 1995 and 2013. We collected data about the clinical characteristics, including demographics, birth history, diagnosis method, and associated anomalies. We analyzed the survival in days, according to three broad categories of treatment policy: give-up, conservative management and active treatment. RESULTS: Of the 22 patients with confirmed trisomy 18, the majority were female (19, 86%). The median gestational age was 39 weeks (range, 31-41 weeks) and the median birth weight is 2,029 g (range, 1,130-2,990 g). Among the anomalies associated with trisomy 18, ventricular septal defect (86%) and patent ductus arteriosus (81%) were the most common cardiac anomalies; giant cisterna magna (59%) was the most common central nervous system anomaly; and clenched hands (73%) and low set ears (59%) were the most common structural anomalies. The survival based on the treatment policy was the highest in the active treatment group, followed by the conservative management group. The give-up group had the lowest survival. CONCLUSION: It is possible to achieve an improvement in both survival and symptom relief for patients with trisomy 18, despite poor neurological outcome and high mortality. Medical personnel need to provide objective data on trisomy 18 to the parents, and determine the treatment policy through careful discussion.
Birth Weight ; Central Nervous System ; Cisterna Magna ; Demography ; Diagnosis ; Ductus Arteriosus, Patent ; Ear ; Female ; Gestational Age ; Hand ; Heart Septal Defects, Ventricular ; Humans ; Korea ; Medical Records ; Mortality ; Parents ; Reproductive History ; Retrospective Studies ; Trisomy*

Vertebral, anal, cardiac, tracheo-esophageal, renal, and limb(VACTERL) association is defined as the presence of at least three of the above-mentioned six manifestations. An estimated incidence of the VACTERL association is 1 in 20,000 to 35,000 live births although the diagnostic criteria vary. The VACTERL association is highly heterogeneous in clinical presentation. It may represent a spectrum from the less severely affected to the more severely affected. Diagnosis is difficult because of the number of disorders that have overlapping features with trisomy 13 syndrome, trisomy 18 syndrome, trisomy 21 syndrome, Feingold syndrome, and so on. The incidence of trisomy 18 syndrome, a type of a chromosomal disorder, is estimated to be 1 in 6,000-8,000 live births. It includes characteristic craniofacial anomalies, clenched hand with overlapping of index finger over third, fifth finger over fourth, underdeveloped thumbs, short sternum, cardiac anomalies such as ventricular septal defect, and renal anomalies such as horseshoe kidney. Approximately over 50% of infants with trisomy 18 syndrome live less than one week. In 1983, Khoury et al. reported VACTERL association combined with trisomy 18 syndrome. Here, we report a case of a low birth weight female infant with VACTERL association, whose second diagnosis is Edward syndrome, and that she also has another combined anomaly, meningomyelocele. To the best of our knowledge, this is the first reported case of VACTERL association with meningomyelocele combined with trisomy 18 syndrome in Korea.
Chromosome Disorders ; Diagnosis ; Down Syndrome ; Female ; Fingers ; Hand ; Heart Septal Defects, Ventricular ; Humans ; Incidence ; Infant ; Infant, Low Birth Weight ; Infant, Newborn ; Kidney ; Korea ; Live Birth ; Meningomyelocele* ; Sternum ; Thumb ; Trisomy*