Congenital heart defect (CHD) is common in infants with Down syndrome (DS), which is the principle cause of mortality. However, there is no data available for the frequency and types of CHD in infants with DS in Korea. We investigated the frequency of CHD in infants with DS in Korea. After the survey on birth defects was conducted throughout the country, the prevalence of CHD in DS in 2005-2006 was calculated. This study was conducted based on the medical insurance claims database of the National Health Insurance Corporation. The number of total births in Korea was 888,263 in 2005-2006; of them, 25,975 cases of birth defects were identified. The prevalence of DS was 4.4 per 10,000 total births, accounting for 1.5% of all birth defects. Of the 394 infants with DS, 224 (56.9%) had a CHD. Atrial septal defect was the most common defect accounting for 30.5% of DS followed by ventricular septal defect (19.3%), patent duct arteriosus (17.5%), and atrioventricular septal defect (9.4%). Our study will be helpful to demonstrate the current status of DS and to identify the distribution of CHD in infants with DS in Korea.
Adult ; Asian Continental Ancestry Group ; Birth Weight ; Chromosome Aberrations ; Databases, Factual ; Down Syndrome/*complications ; Ductus Arteriosus, Patent/epidemiology ; Female ; Gestational Age ; Heart Defects, Congenital/*epidemiology/etiology ; Heart Septal Defects/epidemiology ; Heart Septal Defects, Atrial/epidemiology ; Heart Septal Defects, Ventricular/epidemiology ; Humans ; Male ; Prevalence ; Republic of Korea/epidemiology

Laryngeal edema developed in 10.1% of studied patients with congenital heart disease after cardiac surgery. The 181 patients were divided into two groups; those with laryngeal edema (group 1) and those without laryngeal edema (group 2). The mean ages in group 1 and 2 were 10 and 22.9 months. Group 1 patients were younger on average than those of group 2 (p< 0.05). The differences in the cardiopulmonary bypass time and anesthesia time between the two groups were not statistically significant. The duration of intubations and ventilatory support before and after the onset of laryngeal edema and the period of the ICU stay were longer in group 1 than in group 2 (p< 0.05). A predictor of postextubation laryngeal edema was not found in our patients from above mentioned parameters. We conclude that the higher incidence of laryngeal edema may be due to young age (most were under 1 year of age), and duration of intubation and ventilatory support.
Adolescent ; Child ; Child, Preschool ; Heart Defects, Congenital/*surgery ; Human ; Incidence ; Infant ; Infant, Newborn ; Intubation, Intratracheal/*adverse effects ; Laryngeal Edema/epidemiology/*etiology ; Postoperative Care ; Postoperative Complications ; Risk Factors

BACKGROUND: Congenital heart disease (CHD) is one of the most common congenital malformations in humans. Inconsistent results emerged in the existed studies on associations between air pollution and congenital heart disease. The purpose of this study was to evaluate the association of gestational exposure to air pollutants with congenital heart disease, and to explore the critical exposure windows for congenital heart disease. METHODS: The nested case-control study collected birth records and the following health data in Tianjin Women and Children's Health Center, China. All of the cases of congenital heart disease from 2013 to 2015 were selected matching five healthy controls for each case. Inverse distance weighting was used to estimate individual exposure based on daily air pollution data. Furthermore, the conditional logistic regression with distributed lag non-linear model was performed to identify the association between gestational exposure to air pollution and congenital heart disease. RESULTS: A total of 8,748 mother-infant pairs were entered into the analysis, of which 1,458 infants suffered from congenital heart disease. For each 10 µg/m³ increase of gestational exposure to PM_2.5, the ORs (95% confidence interval, 95%CI) ranged from 1.008 (1.001-1.016) to 1.013 (1.001-1.024) during the 1^st-2^nd gestation weeks. Similar weak but increased risks of congenital heart disease were associated with O₃ exposure during the 1^st week and SO₂ exposure during 6^th-7^th weeks in the first trimester, while no significant findings for other air pollutants. CONCLUSIONS This study highlighted that gestational exposure to PM_2.5, O₃, and SO₂ had lag effects on congenital heart disease. Our results support potential benefits for pregnancy women to the mitigation of air pollution exposure in the early stage, especially when a critical exposure time window of air pollutants may precede heart development.
Infant ; Pregnancy ; Child ; Humans ; Female ; Air Pollutants/analysis* ; Case-Control Studies ; Prenatal Exposure Delayed Effects/epidemiology* ; Heart Defects, Congenital/etiology* ; China/epidemiology* ; Particulate Matter/adverse effects* ; Maternal Exposure/adverse effects*

OBJECTIVEBranch pulmonary artery stenosis is one of the common congenital heart disease. Stent implantation to relieve branch pulmonary artery stenosis (BPAS) is an alternative to failed surgical or balloon angioplasty. The aim of this study was to explore the indication, methods and complications of using balloon expandable stent placement to treat branch pulmonary artery stenosis, and evaluate the results of stent implantation in the treatment of branch pulmonary artery stenosis.

METHODFrom August 2005 to December 2012, 19 patients underwent an attempt at stent implantation. The median age of those patients was 9.1 years (range 4.0-15.0 years). The median weight was 31.7 kg (range 17.0-60.5 kg); 14/19 patients underwent post surgical repair of tetralogy of Fallot, one patient received post surgical repair of pulmonary atresia with ventricular septal defect, one patient underwent post surgical repair of pulmonary atresia with intact septum, one with native left BPAS, and one was after surgical repair of aortopulmonary window and the other truncus arteriosus. CP stent and NuMED Balloon-in-Balloon catheter were selected according to digital subtracted angiography measurements. After checking for correct position by angiography, the inner balloon and outer balloon was inflated successively to expand the stent to desired diameter. Statistical analysis was performed with the unpaired Student t test.

RESULTA total of 26 stents were implanted successfully in 19 patients. The systolic gradient across the stenosis fell from a median of (36.0 ± 18.3) to (3.8 ± 3.4) mmHg (P < 0.01, 1 mmHg = 0.133 kPa) and the diameter of the narrowest segment improved from (6.0 ± 1.9) to (11.6 ± 3.1) mm (P < 0.01). The right ventricle to aortic pressure ratio fell from 0.68 to 0.49 (P < 0.01). Complications included the following: two stents were malpositioned in the right ventricular outflow tract and one balloon ruptured when dilated the hole of the stent. No other complications occurred. All patients were followed up for 6 months to 6.0 (2.5 ± 1.8) years. One patient underwent stent re-dilation in order to accommodate somatic growth two years later.

CONCLUSIONBalloon expandable stents are safe and effective in relieving BPAS. Stent implantation should be considered the treatment of choice for most patients with BPAS. Stents placed into growing children will require further dilation to keep up with normal somatic growth. Intermediate and long-term follow up studies have shown excellent results after further dilation over time.

Adolescent ; Angioplasty, Balloon ; methods ; Blood Vessel Prosthesis Implantation ; Child ; Child, Preschool ; Constriction, Pathologic ; surgery ; Female ; Heart Defects, Congenital ; complications ; surgery ; Humans ; Male ; Postoperative Complications ; epidemiology ; surgery ; Pulmonary Artery ; pathology ; surgery ; Pulmonary Valve Stenosis ; etiology ; surgery ; Retrospective Studies ; Stents ; Treatment Outcome ; Vascular Surgical Procedures ; methods