Results and Complications After Single-Stage Repair of Aortopulmonary Window and Interrupted Aortic Arch in a 32-Week Preterm and a Full-Term Neonate.

BACKGROUND An aortopulmonary window (APW) is an uncommon congenital defect of the septation between the ascending aorta and pulmonary trunk. The combination of APW and interrupted aortic arch (IAA) is even rarer, with the hallmark characteristics of high peri-operative mortality and postoperative obstruction of the aortic arch, pulmonary artery, and left main bronchus. These complications often need re-interventions. CASE REPORT We present 2 cases with diagnoses of APW and IAA that were treated with single-stage repair. Case 1: A male 32-week premature newborn (weight 1789 g) had APW type I and IAA type A. He had severe postoperative aortic arch obstruction on postoperative day 1, and we re-intervened promptly. He was still asymptomatic after 6 years. Case 2: A male term neonate had APW type III and IAA type A. He had left vocal cord paralysis and left bronchial compression postoperatively. We applied prolonged noninvasive respiratory supports. The complications resolved without re-intervention on postoperative day 66. Progressive arch stenosis at anastomosis after operation required close follow-up with echocardiography. CONCLUSIONS These 2 reports highlight the feasibility of single-stage surgical repair while addressing 2 challenges: (1) Recurrent arch stenosis: Lower body weight and direct end-to-side anastomosis without patch augmentation could be risk factors for re-intervention. (2) Bronchial compression: Presentation of the second reported case implied that bronchial compression may not warrant immediate re-intervention unless there is complete obstruction, persistent atelectasis, or recurrent infection. Further studies on long-term outcomes of different surgical procedure would help us to clarify the proper way to avoid re-intervention.

Hemodynamic and Echocardiographic Characteristics and the Presence of Pulmonary Hypertension in Patent Ductus Arteriosus Patients who Underwent Transcatheter Closure.

We investigated the hemodynamic parameters of pediatric PDA patients and focused on the influence of PDA size on pulmonary arterial pressure and the prevalence of pulmonary hypertension. A total of 52 patients aged between 2 months and 20 years who received transcatheter closure of a PDA from January 2018 to June 2022 in our institution were retrospectively recruited. Their hemodynamic parameters collected both by echocardiography and by cardiac catheterization were analyzed to delineate the influence of PDA size on the pulmonary vascular system. The echocardiographic-based ductal size and indexed PDA size were 1.93 mm (1.15-6 mm) and 4.05 mm/m2 (2.03-25.47 mm/m2), respectively. The pulmonary artery pressure measured was 20.83 mmHg (8-45 mmHg). We found a positive correlation between indexed PDA size and mean pulmonary arterial pressure (mPAP) (Pearson correlation coefficient = 0.47, p < 0.001). A subgroup analysis showed that 28 patients (53.8%) developed pulmonary hypertension (PH) (defined as mPAP > 20 mmHg). The median age of the PH group was 1.02 years [range: 0.19-8.64], which was significantly younger than the non-PH group's median age of 3.43 years [range: 0.42-19.96] (p = 0.001). The indexed PDA size for the PH group, 4.69 mm/m2, was significantly higher than that of the non-PH group, 3.2 mm/m2 (p = 0.004). The major risk factor for patients with PH was the PDA/BSA index, with an OR of 2.181 (95% CI, 1.224-3.887). Our demographic data showed younger patients with a higher PDA/BSA index are more likely to develop pulmonary hypertension.

Clinical outcomes of handmade polytetrafluoroethylene trileaflet-valved conduit used for pulmonary valve replacement.

OBJECTIVES: To mitigate the shortage of homograft sources, the use of handmade trileaflet expanded polytetrafluoroethylene valves in pulmonary valve replacement has shown excellent results from multicentre studies conducted in Japan. However, world-wide data outside Japan are relatively insufficient. This study presents the long-term results of a single surgeon's use of flipped-back trileaflet method in a 10-year case series. METHODS: We have developed an efficient way to make a trileaflet-valved conduit utilizing flipped-back method for pulmonary valve replacement and have employed the technique since 2011. Retrospective data were studied between October 2010 and January 2020. Echocardiography, electrocardiogram, Pro-Brain Natriuretic Peptide and Magnetic Resonance Imaging data were analysed. RESULTS: Fifty-five patients were reviewed and median follow-up duration was 2.9 years. The majority of diagnoses was Tetralogy of Fallot (n = 41), and these patients subsequently underwent secondary pulmonary valve replacement at a median age of 15.6 years. Survival was 92.7% with the longest follow-up period being 10 years. There was no need for reoperation, and freedom from reintervention was 98.0% at 10 years. There were 4 deaths (3 in-hospital and 1 outpatient). One patient eventually received transcatheter pulmonary valve implantation. Postoperative echocardiography showed mild or less pulmonary stenosis and pulmonary regurgitation degree in 92.2% and 92.0% of patients, respectively. Comparable magnetic resonance imaging data (n = 25) showed significant reduction in right ventricular volumes but not in ejection fractions. CONCLUSIONS: Our series showed satisfactory long-term function of handmade flipped-back trileaflet-valved conduit used in our patients. The simple design is efficiently reproducible without complex fabrication process.

Resuscitated Sudden Cardiac Arrest of a Neonate with Congenital LQT Syndrome-Associated Torsades de Pointes: A Case Report and Literature Review.

Sudden infant death syndrome (SIDS), the most common cause of infant death in developed countries, is attributed to diverse trigger factors. Malignant cardiac dysrhythmias are potentially treatable etiologies, and congenital long QT syndrome (LQTS) is the most common cardiac ionic channelopathy confronted. ß-Blockers or class Ib agents are the drugs of choice for the control of arrhythmias, and an implantable cardioverter defibrillator (ICD) should be considered for secondary prevention in survivors of lethal cardiac death. We report the case of a 4-day old neonate, later genetically confirmed as LQT type 3 (LQT3), who survived a pulseless torsades de pointes (TdP) attack and was successfully treated with propranolol, mexiletine, and ICD implantation.

Physiological QT Interval Changes in Early Infancy Using Post-Menstrual and Post-Natal Age Calculation for Electrocardiogram Long QT Interval Screening in Taiwan.

BACKGROUND: Prolongation of the QT interval is associated with the risk of sudden infant death syndrome. QT interval differs depending on age at the time of screening. Screening protocols have yet to be established for Taiwanese patients. OBJECTIVES: To construct QT interval reference values during early infancy, to investigate whether QT interval change differs according to age calculation methods, and to identify an optimal QT correction method and associated infant factors. METHODS: Electrocardiographic readings and QT intervals were recorded cross-sectionally in 595 healthy infants and screened prospectively for long QT interval. Corrected QT intervals with Bazett's (QTc-B) and Fridericia's (QTc-F) formulas were compared by post-natal and post-menstrual screening age, sex, body mass index (BMI), heart rate (HR), birth and family history. RESULTS: QTc-B and QTc-F decreased in the second month (31-60 days), and peaked in the third month (61-90 days). QT interval length was similar between post-menstrual and post-natal ages for QTc-B. Simple linear regression showed that post-menstrual age, post-natal age, HR and BMI were associated with QTc-F, while only sex and HR were associated with QTc-B. Although both QTc-B and QTc-F were significantly associated with HR, QTc-B was less affected by HR than QTc-F (ß = -0.1, p < 0.05 for QTc-B vs. ß = -0.3, p < 0.001 for QTc-F). Female infants tended to have slightly longer QTc intervals. CONCLUSIONS: QT interval in early infancy changed physiologically, peaking in the third month. The rate of QT change was not affected by different age correction methods. QTc-B was less affected by age, BMI and HR, although differences in sex should be noted.

Reference ranges and Z-scores for fetal cardiac measurements from two-dimensional echocardiography in Asian population.

Currently available fetal echocardiographic reference values are derived mainly from North American and European population studies, and there is a lack of reference z-score for fetal echocardiographic measurement in Asian populations. The aim of this study was to establish normal ranges of echocardiographic measurements and z-scores in healthy Asian fetuses. A total of 575 healthy pregnant Taiwanese with an estimated gestational age from 14 to 38 weeks were enrolled voluntarily for this observational study. Standard two-dimensional echocardiography was performed to obtain measurements of the cardiac chambers and great arteries of the developing fetuses. In contrast to past studies, our sample was more evenly distributed for estimated gestational age (p<0.001). We present percentile graphs for 13 fetal echocardiographic measurements from the knowledge of estimated gestational age, biparietal distance, head circumference, abdominal circumference, and femur length. Most cardiac structures and developmental markers had linear models as the best-fitting, except for transverse aortic isthmus by estimated gestational age and transverse ductus arteriosus by femur length. Our findings indicate that estimated gestational age was generally the best model for fetal heart development, while head circumferences could be used as an optimal developmental marker to predict left atrium, right atrium, right ventricle, pulmonary annulus, and ductus arteriosus. Lastly, we developed nomograms for each of the 13 fetal heart measurements by each developmental markers. This is the first study providing echocardiographic reference ranges and nomograms for Asian fetuses. Computing z-scores from nomograms helps in standardizing comparisons and adds additional prognostic information to the diagnosis of congenital heart disease.

Impact of Persistent Left Superior Vena Cava Detection in a Normal Neonatal Population through Echocardiography.

BACKGROUND: Persistent left superior vena cava (PLSVC) is a vascular anomaly that is usually asymptomatic and detected incidentally. The incidence of PLSVC has seldom been evaluated in normal populations. In this study, we determined the incidence of PLSVC in a normal neonatal population using transthoracic echocardiography. We also evaluated the associations between PLSVC and asymptomatic congenital heart diseases. MATERIALS AND METHODS: In this retrospective study, we identified healthy neonates based on echocardiography results from 2008 to 2017. Based on the echocardiography findings, we categorized the patients into a PLSVC group and a control group (patients without PLSVC). Chi-square and logistic regression tests were used for data analysis. RESULTS: Of the 19,488 neonates assessed in this study, 56 were found to have PLSVC, and the remaining 19,432 neonates comprised the control group. The incidence of PLSVC was 0.29% in our population. In the PLSVC group, 3.6% of the patients exhibited bicuspid aortic valves, and 10.7% of the patients exhibited secundum-type atrial septal defects. Both the incidence and association of these conditions were higher in the PLSVC group than in the control group. CONCLUSIONS: Based on the echocardiography examination results, we discovered that the incidence of PLSVC in Taiwanese neonates was 0.29%. Although the neonates with PLSVC were asymptomatic and exhibited no health concerns, they were associated with higher incidence rates of bicuspid aortic valves and secundum-type atrial defects. Additional follow-up and evaluation regarding these findings may be warranted.

Noncardiac DiGeorge syndrome diagnosed with multiplex ligation-dependent probe amplification: A case report.

DiGeorge syndrome is not really a rare disease. A microdeletion of chromosome 22q11.2 is found in most patients. Sharing the same genetic cause, a wide spectrum of clinical manifestations such as conotruncal anomaly face syndrome, Cayler cardiofacial syndrome, and velocardiofacial syndrome have been reported. Classic characteristics are cardiac defects, abnormal facial features, thymic hypoplasia, cleft palate, and hypocalcemia. We report a 6-year-old female child presenting with generalized seizure resulting from hypocalcemia. She had no cardiac defects and no hypocalcemia episode in neonatal stage, and had been said to be normal before by her parents until the diagnosis was made. This highlights the importance of extracardiac manifestations in the diagnosis of DiGeorge syndrome, and many affected patients may be underestimated with minor facial dysmorphism. As health practitioners, it is our duty to identify the victims undermined in the population, and start thorough investigations and the following rehabilitation as soon as possible. Multiplex ligation-dependent probe amplification is a rapid, reliable, and economical alternative for the diagnosis of 22q11.2 deletion.

National database study of survival of pediatric congenital heart disease patients in Taiwan.

BACKGROUND/PURPOSE: The incidence of congenital heart disease (CHD) and severe CHD is 13.08 and 1.51/1000 live births, respectively, in Taiwan, which has had national health insurance since 1995 and child health indices similar to those in the US. This study sought to further elucidate the fatality of CHD patients and their survival from a national database. METHODS: From the national health insurance database 2000-2010, we retrieved data from CHD patients who were diagnosed at age <6 years. The survival status at discharge was ascertained for estimation of survival. RESULTS: In total, 18,843 pediatric CHD patients were identified. The overall prevalence of CHD was 1288 per 100 000 live-births. Severe CHD (tetralogy of Fallot (4.4%), transposition of the great arteries (1.6%) and double outlet right ventricle (1.1%)) accounted for 11.5% of all cases. The 1-month/5-year survival in simple and severe CHD was 99.1%/97.5% and 90.2%/76.4%, respectively (p < 0.0001). The Kaplan-Meier survival at 5 years of age was lowest for hypoplastic left heart syndrome (19.7%), followed by transposition of the great arteries (66.7%), double outlet right ventricle (69.0%), and common ventricle (66.0%). The 5-year survival of the birth cohort in the same study period was 99.3%. CONCLUSION: This national database study revealed that the survival of children with simple CHD was still slightly lower than that of the general population and the survival of severe CHD patients, though only accounting for one-tenth of CHD cases, remained unsatisfactory. Such survival profiles are similar to those from Western reports and warrant a refined and dedicated medical care program for children with CHD.

Transmission of acute infectious illness among cases of Kawasaki disease and their household members.

BACKGROUND/PURPOSE: Kawasaki disease (KD) is a disease of unknown cause and the causative agent is most likely to be infectious in nature. To investigate the household transmission pattern of infectious illness and etiology, we thus initiated a prospective case and household study. METHODS: We enrolled KD cases and their household members from February 2004 to September 2008. The KD cases and their household members accepted questionnaire-based interviews of the contact history, signs of infection, and symptoms to check whether clusters of infectious illness occurred. RESULTS: A total of 142 KD cases and 561 household members were enrolled. Among the 142 KD cases, 136 cases (96%) were typical KD, and six (4%) were atypical KD. Of the 561 household members, 17% were siblings, 46% were parents, 18% were grandparents, and the others were cousins or babysitters. Prior to the onset of their KD illness, 66% (94/142) KD cases had contact with ill household members. On the same day of the onset of KD cases' illness, 4% (6/142) KD cases had household members with illness. After KD cases' disease onset, 70% (100/142) KD cases had at least one other family member with illness. Overall, 61% (343/561) of all the household members had acute infectious illness during KD cases' acute stage, and 92% (130/142) of the families had clusters of infectious illness. CONCLUSION: A total of 66% KD cases had positive contact with ill household members prior to their disease onset and 92% of families had clusters of infectious illness, so KD is strongly associated with infections.

Immunogenicity and safety of a single dose of a CRM-conjugated meningococcal ACWY vaccine in children and adolescents aged 2-18 years in Taiwan: results of an open label study.

BACKGROUND: MenACWY-CRM (Menveo®, Novartis Vaccines, Siena, Italy) is a quadrivalent meningococcal conjugate vaccine developed to help prevent invasive meningococcal disease caused by Neisseria meningitidis serogroups A, C, W, and Y. It is approved within the European Union in persons >2 years of age and in persons from 2 months to 55 years of age in the United States, among other countries. Little is known about the immunogenicity and safety of this vaccine in Taiwanese children >2 years and adolescents. This study assessed the immunogenicity and safety of a single injection of MenACWY-CRM vaccine in Taiwanese subjects aged 2-18 years old. METHODS: In this phase III, multicentre, open-label study 341 subjects received one dose of MenACWY-CRM. Immunogenicity measures were rates of seroresponse (defined as the proportion of subjects with a postvaccination hSBA ≥1:8 if the prevaccination (baseline) titre was <1:4, or at least a fourfold higher hSBA titre than baseline if the prevaccination titre was ≥1:4), percentages of subjects with serum bactericidal activity (hSBA) ≥1:8 for serogroups A, C, W and Y and hSBA geometric mean titres (GMTs). Local and systemic reactions and all adverse events (AEs) were recorded for 7 days, and medically attended AEs for 1 month post-vaccination. RESULTS: Seroresponse rates after MenACWY-CRM vaccination at Day 29 for the serogroups A, C, W, and Y were 83%, 93%, 50%, and 65%, respectively. At Day 29 the percentages of subjects with hSBA ≥1:8 against all four serogroups A, C, W and Y were: 83%, 96%, 96% and 82%, respectively. GMTs against all serogroups rose by ≥7-fold from baseline to Day 29. The vaccine was well tolerated. CONCLUSIONS: A single dose of MenACWY-CRM demonstrated a robust immune response, and an acceptable safety profile in Taiwanese children and adolescents.

Concomitant administration of live attenuated Japanese encephalitis chimeric virus vaccine (JE-CV) and measles, mumps, rubella (MMR) vaccine: randomized study in toddlers in Taiwan.

BACKGROUND: Japanese encephalitis (JE) is the most important cause of viral encephalitis in Asia. METHODS: In this randomized, open-label, multicenter trial in 550 children aged 12 to 18 months in Taiwan, children received one dose of JE-CV and one dose of MMR vaccine. Vaccines were either administered separately 6 weeks apart (Groups 'JE-CV' and 'MMR', named after which vaccine was given first), or concomitantly (Group 'Co-Ad'). JE neutralizing antibody titers were assessed using PRNT50. MMR antibody levels were determined by ELISA. RESULTS: All groups had low seroprotection/seropositivity rates (<10%) before vaccination for all antigens. Forty two days after vaccination, on either Study Day 42 or 84, seroconversion rates for all antigens were high in all groups, irrespective of the order of vaccinations. Seroconversion for JE ranged from 96.9% in Group Co-Ad on D42 to 100% in Group MMR. Non-inferiority was demonstrated for all analyses as the lower bound of the 95% CI of the difference in seroconversion rates between groups was above the pre-defined limit of -10.0%. The immune responses remained high for all antigens and well above the level of protection 12 months after vaccination in all groups. There were no safety concerns. CONCLUSIONS: JE-CV is safe and induces a strong protective immune response which persists over 1 year when co-administered with MMR vaccine.

Viral infections associated with Kawasaki disease.

BACKGROUND/PURPOSE: Kawasaki disease (KD) is a disease of unknown cause. To investigate the infectious etiology of Kawasaki disease, we initiated a prospective case-control study to investigate possible links between common viral infections and Kawasaki disease. METHODS: We enrolled 226 children with KD and 226 age- and sex-matched healthy children from February 2004 to March 2010. Throat and nasopharyngeal swabs were taken for both viral isolation and polymerase chain reaction (PCR) for various viruses. RESULTS: The mean age of the 226 KD cases was 2.07 years, and the male to female ratio was 1.43 (133 boys to 93 girls). Their mean fever duration was 7.5 days with a mean peak temperature of 39.7°C. In addition to the typical symptoms of fever, neck lymphadenopathy, lip fissure and/or strawberry tongue, skin rash, nonpurulent bulbar conjunctivitis, palm/sole erythema, and induration followed by periungual desquamation, these KD cases also exhibited cough (69%), rhinorrhea (58%), and diarrhea (45%). Cases of KD had a significantly higher positive rate of viral isolation in comparison with the control group (7.5% vs. 2.2%, p = 0.02). Compared with the control group, cases of KD were more likely to have overall positive rates of viral PCR (50.4% vs. 16.4%, p < 0.001) and for various viruses including enterovirus (16.8% vs. 4.4%, p < 0.001), adenovirus (8.0% vs. 1.8%, p = 0.007), human rhinovirus (26.5% vs. 9.7%, p < 0.001), and coronavirus (7.1% vs. 0.9%, p = 0.003). CONCLUSION: We found that some common respiratory viruses, such as adenoviruses, enteroviruses, rhinoviruses, and coronaviruses, were associated with KD cases.

Potential drug-drug interactions in pediatric outpatient prescriptions for newborns and infants.

OBJECTIVES: To surveyed the quantities, types, and related information of potential drug-drug interactions (DDIs) and estimate the off-label use percentage of pediatric outpatient prescriptions for newborns and infants from the National Health Insurance Research Database (NHIRD) of Taiwan. BACKGROUND: Adverse drug reactions (ADR) may cause morbidity and mortality, potential drug-drug interactions (DDI) increase the probability of ADR. Research on ADR and DDI in infants is of particular urgency and importance but the related profiles in these individuals are not well known. METHODS: All prescriptions written by physicians in 2000 were analyzed to identify potential DDIs among drugs appearing on the same prescription sheet. RESULTS: Of a total of 150.6 million prescription sheets, with 669.5 million prescriptions registered in the NHIRD of Taiwan, six million (3.99%) prescription sheets were for 2.1 million infants with 19.4 million (2.85%) prescriptions. There were 672,020 potential DDIs in this category, accounting for 3.53% per prescription; an estimated one DDI in every three patients. The interactions between aspirin and aluminum/magnesium hydroxide were most common (4.42%). Of the most significant drug-drug interactions, the interaction of digoxin with furosemide ranked first (20.14%), followed by the interactions of cisapride with furosemide and erythromycin (6.02% and 4.85%, respectively). The interactions of acetaminophen and anti-cholinergic agents comprised most types of drug-drug interactions (6.62%). CONCLUSION: Although the prevalence rates of DDIs are low, life-threatening interactions may develop. Physicians must be reminded of the potential DDIs when prescribing medications for newborns and infants.

Parental smoking during pregnancy and its association with low birth weight, small for gestational age, and preterm birth offspring: a birth cohort study.

BACKGROUND: Intrauterine exposure to tobacco smoke has been discerned as an important risk factor for low birth weight (LBW), small for gestational age (SGA), and preterm birth infants. In this cohort study, we investigated the association of the amount of parental smoking during the different pregnancy stages with birth weight and the incidence of preterm delivery. METHODS: Our study population was acquired from the Taiwan Birth Cohort Study. Between June 2005 and July 2006, 21,248 postpartum women were interviewed 6 months after their deliveries by a structured questionnaire. The parents were divided into four groups according to the amount of smoking during preconception, the first trimester, and the second and third trimesters. The relationships of parental smoking with gestational age and birth weight during the different pregnancy stages were assessed using multivariate linear regression. Multiple logistic regression analyses were performed to estimate the odds ratios and 95% confidence intervals of preterm delivery, LBW, and SGA infants during the different parental smoking status and the different pregnancy stages. RESULTS: After adjusting for the physical and socioeconomic status of the parents and for paternal smoking during the same period, we found that maternal smoking decreased birth weight. Compared with the nonsmoking groups, all the maternal smoking groups had higher incidences of LBW, SGA, and preterm birth infants, especially when the mothers smoked >20 cigarettes/day. The association of paternal smoking with LBW, SGA, and preterm birth infants was insignificant. CONCLUSION: Maternal smoking is responsible for increased incidences of LBW and preterm delivery of babies, and therefore, smoking cessation/reduction should be advised to pregnant women to reduce morbidities in their neonates. Further studies are needed to clarify the correlation of fetal health with passive smoking, including exposure to environmental tobacco smoke and to other smokers in the family.

Identifying term breast-fed infants at risk of significant hyperbilirubinemia.

BACKGROUND: The aim of this study was to establish a model to identify term breast-fed infants who are at risk of developing significant neonatal hyperbilirubinemia. METHODS: A prospective study was designed to investigate the effects of birth weight, mode of delivery, cephalohematoma, glucose-6-phosphate dehydrogenase (G6PD) deficiency, predischarge total serum bilirubin, variant uridine 5'diphospho-glucuronosyltransferase 1A1 (UGT1A1) gene, and hepatic solute carrier organic anion transporter 1B1 (SLCO1B1) gene on significant hyperbilirubinemia in term breast-fed neonates. Significant hyperbilirubinemia was defined as a bilirubin level exceeding the hour-specific phototherapy treatment threshold recommended by the American Academy of Pediatrics in 2004. RESULTS: Of 240 exclusively breast-fed term neonates, 26 (10.8%) had significant hyperbilirubinemia. The predischarge total serum bilirubin on the third day (odds ratio (OR) = 2.63; 95% confidence interval (CI): 1.87-3.70; P < 0.001) and the variant UGT1A1 gene at nucleotide 211 (OR = 5.00; 95% CI: 1.08-23.03; P < 0.05) were significant risk factors. The area under the receiver operating characteristic (ROC) curve of the predictive probability was 0.964 (95% CI: 0.932-0.984; P < 0.0001). CONCLUSION: Combining the total serum bilirubin on the third day and the variant UGT1A1 gene at nucleotide 211 can predict hyperbilirubinemia well in term breast-fed infants.

Prevalence, mortality, and the disease burden of pediatric congenital heart disease in Taiwan.

BACKGROUND: In Taiwan, the incidence of congenital heart diseases (CHDs) and severe CHDs was 13.08 and 1.51 per 1000 live births, respectively. This study further elucidates the prevalence and mortality of pediatric CHD patients in Taiwan. METHODS: From the National Health Insurance database 2000-2010, we retrieved the data of CHD patients (aged 0-18 years). Mortality data were obtained from the national death statistics. RESULTS: In total, 45,119 pediatric CHD patients were identified, given the prevalence at 918.0 per 100,000 (107.1 for severe CHD and 853.8 for simple CHD). Ventricular septal defect, ostium secundum-type atrial septal defect, patent ductus arteriosus, pulmonary stenosis, and tetralogy of Fallot were the five most frequently diagnosed CHDs. In those aged 0-6 years, the prevalence was 1233.7 per 100,000 (123.5 for severe CHD and 1149.6 for simple CHD). The age-specific prevalence of both simple and severe CHDs declined rapidly after the age of 10 years. From the death registry, we noted that more than 90% of CHD-related deaths occurred before the age of 5 years. The probability of cardiac death in CHD patients during infancy was 4.5%, with the cumulative probability reaching 5.44%, 5.68%, and 6.04% by the ages of 5, 10, and 20 years, respectively. CONCLUSION: Because most CHD deaths occurred within the first 5 years of life (mainly during infancy), the relatively low prevalence of CHDs in the population aged 0-18 years (918/100,000; 74% for those between 0 years and 6 years of age) and the rapid decline in the age-specific prevalence of CHD after the age of 10 years was attributed to noncompliance of the children to medical follow-up after they began schooling.

Prevalence and the long-term coronary risks of patients with Kawasaki disease in a general population <40 years: a national database study.

BACKGROUND: Patients with Kawasaki disease (kDa) may develop coronary arterial lesions and subsequent coronary events. The first reported case in Taiwan was in 1976, and the annual incidence from 2003 to 2006 was 69/100 000 children < 5 years. A population study from Taiwan, a country with a high incidence of kDa, national health insurance, and easily accessible medical care, would adequately reflect the long-term risk. METHODS AND RESULTS: We retrieved the data of kDa patients from a national health insurance 2000 to 2010 database of Taiwan, a country with a child health index similar to those in the United States. The occurrence of coronary complications and interventions was identified by the respective International Classification of Diseases, Ninth Revision, codes. The prevalence of kDa in the population < 40 years was 34.9/100 000 (male/female ratio, 1.47). Coronary complications occurred in 1254 patients (5.37%; male/female ratio, 2.19), with an average annual risk of 2.4% (2.7% for males and 2.0% for females). An acute myocardial infarction occurred in 19 patients (0.08%; 18 males and 1 female), of whom one third were aged between 10 and 15 years (median, 15.7 years; range, 0.7-36.7 years). A coronary intervention was performed by catheterization in 18 patients (all males) at a median age of 24.5 years and by surgery in 10 patients (male/female ratio, 4.0) at a median age of 21.7 years, with mortality at discharge being 0% and 25%, respectively. CONCLUSIONS: This study estimated the overall prevalence of kDa (≈1/2940) in a population < 40 years. They, particularly the males, carry long-term coronary risks from a young age. Risk stratification for a timely coronary intervention and risk modification are mandatory.

Risk of hyperbilirubinemia in breast-fed infants.

OBJECTIVE: To investigate the risk factors for hyperbilirubinemia in infants who are exclusively breast-fed. STUDY DESIGN: A prospective study was conducted to investigate the effects of birth body weight, sex, mode of delivery, glucose-6-phosphate dehydrogenase (G6PD) deficiency, variant UDP-glucuronosyltransferase 1A1 (UGT1A1) gene, and hepatic solute carrier organic anion transporter 1B1 (SLCO1B1) gene on hyperbilirubinemia in neonates who were breast-fed. Hyperbilirubinemia was diagnosed when a full term neonate had a bilirubin level ≧15.0 mg/dL (256.5 µM) in serum at 3 days old. The polymerase chain reaction-restriction fragment length polymorphism method was used as a means of detecting the known variant sites in the UGT1A1 and SLCO1B1 gene. RESULTS: Of 252 infants born at term who were exclusively breast-fed, 59 (23.4%) had hyperbilirubinemia. The significant risk factors were a variant nucleotide 211 in UGT1A1 (2.48; 95% CI, 1.29 to 4.76; P = .006), G6PD deficiency (12.24; 95% CI, 1.08 to 138.62; P < .05), and vaginal delivery (3.55; 95% CI, 1.64 to 7.66; P < .001). CONCLUSION: Breast-fed neonates who are 211 variants in the UGT1A1, G6PD deficiency, and vaginal delivery are at high-risk for hyperbilirubinemia.

Risk factors for liver steatosis in obese children and adolescents.

BACKGROUND: Concurrent with the recent rise of the incidence in obesity, nonalcoholic fatty liver disease is increasingly prevalent in childhood. The aim of this study was to identify non-invasive biomarkers for liver steatosis in obese children and adolescents. METHODS: We used a cross-sectional study to examine risk factors for liver steatosis in obese children and adolescents. Sixty-nine obese subjects aged 6-17 years were recruited. The diagnosis of liver steatosis was made by liver ultrasonography. Anthropometric, serum biochemical variables, and oral glucose tolerance tests were measured. RESULTS: Thirty-eight (55.1%) subjects had liver steatosis. Elevated alanine aminotransferase levels (> 30 IU/L in boys and >19 IU/L in girls) were found in 27 (71.1%) of the 38 subjects with liver steatosis. In multivariate logistic regression analysis, liver steatosis was associated with waist circumference and the change of plasma glucose level before and after oral glucose tolerance testing (C-OGTT). For every 5 cm increase in waist circumference, there was an odds ratio of 1.391 for predicting liver steatosis (95% confidence interval: 1.009-1.916, p = 0.044). C-OGTT was the only laboratory variable that independently predicted liver steatosis, with an odds ratio of 1.198 (95% confidence interval: 1.022-1.404, p = 0.026) for each 5 mg/dL of increase. CONCLUSION: In this hospital-based sample of obese children and adolescents, liver steatosis was common. Liver steatosis was positively associated waist circumference and C-OGTT. These findings have implications for screening liver steatosis in obese children and adolescents.