Atrial fibrillation in adult congenital heart disease and the general population.

BACKGROUND: Atrial fibrillation (AF) in adult patients with congenital heart disease (ACHD) may appear early, depending on individual characteristics. OBJECTIVES: The goals of this study were to investigate the epidemiological spectrum of AF in the entire cohort of ACHD and compare it with that in the general population. METHODS: A retrospective study was performed in the nationwide cohort 2000-2014 with AF onset during 2003-2014. RESULTS: In the cohort of ACHD, 2350 patients had AF; the incidence increased with age, plateauing around age 70. In patients aged 25-29, 45-49, 65-69, 75-79, and ≥80 years, the annual incidence was 1.3, 7.9, 20.6, 23.7, and 21.4/1000 per year, respectively. In the general population without CHD, 347,979 patients had AF; the annual incidence was <1/1000 per year in those aged <55 years but increased steadily with age (3.6, 8.6, and 14.2/1000 per year in aged 65-69, 75-79, and ≥80 years, respectively). Compared with individuals without ACHD, ACHD patients aged <50 years and those aged both 50-54 and 55-59 years exhibited a 20-fold and 10-fold higher incidence of AF, respectively. Patients with complex congenital heart disease and Ebstein's anomaly had the highest risk of AF (cumulative risk >10% by age 50 and >20% by age 60), followed by those with tetralogy of Fallot, tricuspid atresia, endocardial cushion defect, and secundum atrial septal defect (cumulative risk >5% by age 50 and >10% by age 60). CONCLUSION: Compared with individuals without ACHD, AF in patients with ACHD likely appeared 30 years earlier, with a 10- to 20-fold higher incidence plateauing around age 70. Yet, incidence in individuals without ACHD continued to increase. AF burden in patients with ACHD is not expected to increase in a never-ending way.

Epidemiology of Cardiomyopathy in Taiwanese Population Aged Younger Than 65 Years.

Background: Few studies have investigated the epidemiology of cardiomyopathy (CMP) in the general population in Taiwan. The aim of this study was to investigate this issue. Methods: We identified patients aged < 65 years and diagnosed with CMP between 2001 and 2014 from the National Health Insurance Database of Taiwan 2000-2014. Those with known or presumed causes of CMP were further identified. Results: We identified 38,868 CMP patients (male/female = 2.13). Half had known or presumed causes of CMP, including coronary artery disease (23.6%), congenital heart disease (1.6%), metabolic disease (8.4%), conduction disturbance/dyssynchrony (2.2%), myocarditis (0.5%), muscular dystrophy (1.42%), Kawasaki disease (0.2%), nutrition problems or alcoholism (2.9%), and unspecified causes (12.4%). The incidence rates of CMP without known causes were 1.13 and 8.70 per 100,000 person-years in pediatric (0-19 years) and adult (20-64 years) populations, respectively. After an initial peak during infancy (9.16 per 100,000 person-years), the incidence declined to a nadir in those aged from 5 to 14 years, and then steadily increased during adulthood (26.51 per 100,000 person-years in those aged 60-64 years). Although mortality was higher in the pediatric (11.4%) than in the adult (1.5%) patients, the proportion of sudden death to all deaths was similar in the pediatric (9.9%) and adult (10.5%) patients. Conclusions: This study provides an epidemiological continuum of CMP in a Taiwanese population aged < 65 years. The results revealed an initial peak during infancy, followed by a decline in adolescence and a subsequent steady rise. The prognosis was poorer in the pediatric patients, and poorest in the infants. However, the risk of sudden death was the same in the adult and pediatric patients.

The Postnatal Risk, Resuscitation Success Rate and Outcomes of Pediatric Sudden Death in Taiwan.

BACKGROUND: The epidemiology of pediatric potentially sudden death (SD) events and the rescue rate remain unclear. METHODS: We established a birth cohort (2000-2014) from a national database 2000-2015. RESULTS: Of 3,097,277 live births, we identified 3126 children (56.1% male) with potentially SD events, including 887 who were rescued. The cumulative risk of potentially SD events for each neonate was 0.30, 0.62, 0.91, 1.05, and 1.13 per 1000 by 2 months, 0, 5, 11 and 14 years of age, respectively. Overall, 28.3% of the children were rescued from SD events, with a higher rate in neonates (69.6%) but lower rate in postneonatal infants. A cardiac diagnosis was noted in 596 (19.1%) patients, including congenital heart disease (CHD) (388), cardiac arrest (151), cardiomyopathy (23), myocarditis (12), Kawasaki disease (7) and arrhythmia (36). Coexisting severe CHD and events in postneonatal infancy were associated with a lower chance of resuscitation, whereas events within 1 week of age had a higher chance of resuscitation. Anoxic brain damage was noted in 174 (19.7%) patients and late death occurred in 348 (39.3%) patients after being rescued from SD. Late death was more common in males, those with anoxic brain damage, those with coexisting severe CHD, and postneonatal infants. CONCLUSIONS: In this birth cohort study, the postnatal cumulative risk of potentially SD events was 1 in 885 newborns by 14 years of age. Postneonatal infants and those with coexisting severe CHD had the highest risk and worst outcomes.

Incidence and Postnatal Profile of Fontan Patients by Adolescence from a Nationwide Birth Cohort.

BACKGROUND: Although the Fontan procedure is associated with a variety of long-term complications, it is the mainstay treatment for congenital heart disease with a functioning single ventricle. Data concerning the epidemiological profile are scarce. METHODS: We investigated the current epidemiological profile using a 2000-2008 nationwide birth cohort from a 2000-2014 database (1,967,991 live births), with complete postnatal data for at least 6 years. We identified 363 patients (2792 patient-years of follow-up) who had received the Fontan procedure, giving an incidence of 0.184/1000 live births. RESULTS: The overall Fontan surgical survival rate was 81.8%. In post-Fontan patients, the 10-year survival was 0.822 (±0.026). Causes of death included cardiac (43.8%), infection (20.8%), out-of-hospital death (16.7%), sudden death (8.3%), cerebral vascular accident (8.3%) and malignancy (2.1%). The risk of unexpected death (sudden death and out-of-hospital death) was 4.0%, or 0.55% per post-Fontan patient-year. Arrhythmias were common (12.1%). Supraventricular tachycardia was the most common type of arrhythmia, and occurred prior to the Fontan procedure in 22 patients, with a cumulative risk of 2.2%, 6.3%, and 11.6% by the age of 1, 5 and 10 years, respectively. Arrhythmia intervention was performed in 40.9% of those with arrhythmia, including electrophysiological studies/ablation in 12 and device therapy in 6 patients. CONCLUSIONS: In conclusion, the incidence of Fontan patients was 0.184/1000 live births. Their medical complexity included a high risk of supraventricular tachycardia and unexpected death by adolescence.

Cumulative postnatal risk of pediatric sudden death and all-cause mortality in nationwide birth cohorts of Taiwan and the United States.

BACKGROUND: To investigate the cumulative postnatal risk of pediatric sudden death (SD) for each neonate and the temporal/regional differences which are still unclear. METHODS: We established a birth cohort (2000-2014) from our national database and obtained reference data about the United States (US) from the national website. RESULTS: Among 3,097,277 live births, we identified 1661 children with SD (56.8% male). The postnatal cumulative risk of SD was 0.35, 0.49, 0.56 and 0.59/1000 by age 0, 5, 11 and 14 years. Coexisting cardiac diagnosis was noted in 347 (20.9%) and non-cardiac diagnosis in 300 (18.1%) patients. Cumulative all-cause mortality was 5.3, 6.78, 7.63 and 8.06/1000 by age 0, 5, 11 and 14 years. Risks of SD and all-cause death decreased over birthyear. SD risk decreased particularly after the 2008 birthyear. Risks of SD and all-cause death were the highest in Eastern Taiwan, but SD/all-cause death ratio was high in Taipei metropolitan and Northern Taiwan. Cumulative risk of SD (0.54/1000 by age 14) and all-cause mortality (9.06/1000 by age 14) in the US decreased over time. African-Americans were at the highest risks of SD and all-cause mortality. However, American-Indians/Alaska-natives exhibited the highest SD/all-cause death ratio. CONCLUSION: This study provides the cumulative risk of SD (1 in 1694 newborns) by age 14 years. The time trend underlines the effect of medical advance and education on SD prevention. Distinct regional/ethnic differences in risks of SD and all-cause death in Taiwan and the US indicate the necessity of different strategies to diminish the disparity.

Shock and unresponsiveness to repeated courses of intravenous immunoglobulin in Kawasaki disease: a nationwide database study.

BACKGROUND: We aimed to investigate the clinical implications of unresponsiveness to single or repeated courses intravenous immunoglobulin (IVIG) and Kawasaki disease (KD) shock syndrome in patients with KD in an era of a single brand of IVIG. METHODS: Data were collected from National Health Insurance database 2010-2013. Characteristics of the KD patients were analyzed, including age, gender, shock, and associated coronary aneurysms. RESULTS: There were 3043 KD patients (male: 1872) identified. Among them, 46 (1.51%) had KDSS, 261 patients (8.5%) had IVIG unresponsiveness, and 225 patients (7.4%) developed coronary aneurysms. Moreover, 51 patients did not respond to the second course IVIG therapy, i.e., re-IVIG unresponsiveness. KDSS was associated with the occurrence of IVIG unresponsiveness (P < 10-4) and re-IVIG unresponsiveness (P = 0.02). In addition to male gender and KD shock syndrome, IVIG unresponsiveness (OR: 2.18, 95% CI: 1.48-3.22, P = 0.001) and re-IVIG unresponsiveness (OR: 2.87, 95% CI: 1.40-5.89, P = 0.004) were both independent risk factors for coronary aneurysms. CONCLUSIONS: In a nationwide KD cohort, both IVIG unresponsiveness and re-IVIG unresponsiveness increase the risk of coronary aneurysms. Such observation addresses the importance of refining the treatment for IVIG unresponsiveness, at least in those with KD shock syndrome.

Adult Congenital Heart Disease in a Nationwide Population 2000-2014: Epidemiological Trends, Arrhythmia, and Standardized Mortality Ratio.

BACKGROUND: The adult congenital heart disease (ACHD) population will grow with medical advances, but data are limited. We investigated the epidemiological profile of ACHD in Taiwan, a country with a congenital heart disease program since 1955, population of 23 million, and easily accessible high-standard medical care. METHODS AND RESULTS: ACHD patients, born after 1954, were identified from the nationwide database 2000-2014. The ACHD prevalence in the population aged 18 to 59 was 140.53, 157.08, 182.45, and 217.00 per 100 000 in 2000, 2005, 2010, and 2014, respectively (increasing time trend, P<0.0001). Percentage of severe ACHD also increased over time (P<0.0001) and was 11.70% in 2014. The 5 leading ACHD diagnoses were ventricular septal defect, secundum atrial septal defect, patent ductus arteriosus, pulmonary stenosis, and tetralogy of Fallot. Freedom from tachyarrhythmia at age 50 years was 0.574 and 0.710 for severe and simple ACHD, respectively. Cardiac causes accounted for the majority of deaths, followed by malignancy in simple ACHD and external causes/sudden death/out-of-hospital death in severe ACHD patients. The proportion of unexpected death was 10%. Compared with the general population, the standardized mortality ratio was higher not only in severe ACHD (3.164; 95% confidence interval, 2.664-3.664), but also in women with simple ACHD (1.704; 95% confidence interval, 1.499-1.909), with a higher proportion of cardiac, labor, and sudden death as causes of death. CONCLUSIONS: We demonstrated an increasing trend in ACHD prevalence and medical complexity. They are at risk of tachyarrhythmia, higher mortality, and unexpected deaths, suggesting a gap in their medical care.

Contemporary Postnatal Incidence of Acquiring Acute Myocarditis by Age 15 Years and the Outcomes From a Nationwide Birth Cohort.

OBJECTIVES: Acute myocarditis can be lethal, but the incidence remains unclear because of its wide manifestation spectrum. We investigated the postnatal incidence of acute myocarditis and risk factors for morbidity and mortality. DESIGN: Retrospective derived birth cohort study. SETTING: Taiwan National Health Insurance Database for the period 2000-2014. PATIENTS: Children born between 2000 and 2009 with complete postnatal medical care data for at least 5 years. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: From among 2,150,590 live births, we identified 965 patients (54.8% male) admitted with the diagnosis of acute myocarditis, accounting for an overall incidence of 0.45/1,000. The cumulative incidence rates were 0.19/1,000, 0.38/1,000, 0.42/1,000, and 0.48/1,000 by ages 1, 5, 10, and 15 years, respectively. Male predominance was noted in infants and school age children (age group 6-14 yr). Arrhythmias, including tachyarrhythmia (4.8%) and bradyarrhythmia (1.1%), occurred in 56 patients. Extracorporeal membrane oxygenation support was provided to 62 patients (6.4%) and additional left ventricular assist devices in six of them. The mortality at discharge was 6.3%. The presence of ventricular tachyarrhythmia, bradyarrhythmia, and an onset at school age (6-14 yr) were associated with increased odds for the need for extracorporeal membrane oxygenation, which was the only predictor for mortality at discharge (hazard ratio, 7.85; 95% CI, 3.74-9.29). In patients who survived the acute myocarditis, late mortality was relatively low (36/904 = 4.0%). The overall survival of children with acute myocarditis were 90.9%, 90.3%, and 89.8% by the intervals of 1, 5, and 10 years after the myocarditis, respectively. CONCLUSIONS: This birth cohort study determined the cumulative incidence of acute myocarditis for neonates by 15 years old to be one in 2,105. In an era of extracorporeal membrane oxygenation, the need of extracorporeal membrane oxygenation may reflect the severity of acute myocarditis and predict its outcome.

Postnatal Risk of Acquiring Kawasaki Disease: A Nationwide Birth Cohort Database Study.

OBJECTIVE: To investigate the postnatal risk of Kawasaki disease and coronary complications from a nationwide birth cohort in Taiwan, a country with the third-highest incidence of Kawasaki disease worldwide. STUDY DESIGN: We enrolled children born between 2000 and 2009 with complete postnatal medical care records for 2000-2014 in the Taiwan national database. RESULTS: Out of a total of 2 150 590 live births, we identified 6690 (62.6% boys) patients with Kawasaki disease. The onset was mostly (93.9%) within the first 5 years of life (median, 16 months; 38% during infancy), but was rare within the first 3 months of life. The overall cumulative incidence of Kawasaki disease by age 5 years was 2.78‰ (3.33‰ for boys and 2.17‰ for girls; P < .001) and exhibited an increasing trend with birth year (from 2.28‰ for 2000 to 3.67‰ for 2009). The incidence ratio was 1.535 in boys and 1.055 in each increasing year. Kawasaki disease recurred more often in younger patients (cumulative incidence, 2.3% in infants vs 1.7% in children aged 1-4 years). Coronary complications occurred in 16.2% of the patients, including 4 cases of acute myocardial infarction (3 occuring during the acute stage and 1 occurring 5 years later). The probability of a major cardiac event (infarction, undergoing percutaneous coronary intervention or coronary artery bypass grafting, or death) by adolescence was 1.9%. CONCLUSIONS: The postnatal risk of Kawasaki disease was 3‰-4‰ and increased with every birth year. Patients with Kawasaki disease are at substantial risk for a major cardiac events during childhood.

Ventricular Fibrillation in a General Population　- A National Database Study.

BACKGROUND: Ventricular fibrillation (VF) is a life-threatening disease that can be remedied by prompt defibrillation. However, data regarding such risk in a general population remain limited. This general population study was to explore the epidemiological profile of VF.Methods and Results:We investigated patients with VF younger than 60 years (average population, 19,725,031) using a national database spanning the period 2000-2010. We identified 3,971 (68.4% male) patients with VF (crude incidence rate: 1.83/100,000). Incidence rates were low in patients younger than 10 years and increased steadily after adolescence. Comorbidities were noted in 2,766 (69.7%) patients, with 2,431 (61%) having cardiac diseases. Over half of the adolescent and young adult patients did not have comorbidities. Among the 838 deaths (mortality rate 21.1%), approximately half (381/838, 45.5%) occurred after arrival at emergency services (ES). The proportion of deaths after arrival at ES relative to total deaths increased sharply to a peak in the 15-19-years age group and thereafter remained stationary. CONCLUSIONS: VF patients, with a male dominance, increased after adolescence and were likely to die at presentation to ES. Approximately half of young adults, with high mortality, did not have comorbidities, suggesting underdiagnosis of underlying primary electrical diseases and the need for implementing automated external defibrillator programs. (Circ J 2016; 80: 2310-2316).

Postnatal cumulative incidence of supraventricular tachycardia in a general pediatric population: A national birth cohort database study.

BACKGROUND: Supraventricular tachycardia (SVT) is a common pediatric tachycardia, but the true incidence is unknown. OBJECTIVE: We sought to investigate the true postnatal incidence and its medical needs. METHODS: We derived a birth cohort comprised children born between 2000 and 2008 who had complete postnatal medical data in the Taiwan National Health Insurance Database for the period from 2000 to 2014. RESULTS: From 1,967,911 live births, we identified 2021 patients with SVT (51.6% men), accounting for an overall incidence of 1.03 per 1000 patient-years (Wolff-Parkinson-White syndrome accounted for 16.2%). The cumulative incidence was 0.06, 0.25, 0.45, 0.88, and 1.39 per 1000 patient-years by the age of 1 month, 1 year, 5 years, 10 years, and 15 years, respectively. Major congenital heart disease (5.3%; hazard ratio 6.66; 95% confidence interval 2.98-14.87) and cardiomyopathy (0.9%; hazard ratio 8.78; 95% confidence interval 3.39-22.78) were associated with mortality. In patients without major congenital heart disease, the cumulative incidence of SVT was 0.05, 0.22, 0.41, 0.84, and 1.33 per 1000 patient-years by the age of 1 month, 1 year, 5 years, 10 years, and 15 years, respectively. By the age of 15 years, the annual risk of death and sudden death was 0.13% and 0.01% per patient-year, respectively. Radiofrequency catheter ablation was performed in 173 patients at the median age of 11 years: 1.7% during infancy, 5.8% by the age of 5 years, and 31.8% by the age of 10 years. The probability of being free from receiving ablation by the age of 15 years was 83.4%. CONCLUSION: This birth cohort study provides the true incidence of pediatric SVT and indicates that almost one-fifth of the patients with SVT have already received ablation in the pediatric ages.

Risk of Respiratory Syncytial Virus Infection in Cyanotic Congenital Heart Disease in a Subtropical Area.

OBJECTIVE: To compare the risk of respiratory syncytial virus (RSV)-associated hospitalization and analyze the epidemiology of RSV infection in patients with cyanotic and acyanotic congenital heart disease (CHD), we analyzed the nationwide health insurance database from 2005-2010. STUDY DESIGN: This study included 1050 patients with cyanotic CHD and 7077 patients with acyanotic CHD. Patients with acyanotic CHD were further classified into hemodynamically significant (hs)-acyanotic and non-hs-acyanotic groups according to whether they underwent surgery or took at least 2 anticongestive medications. RESULTS: RSV-associated hospitalization was higher in the cyanotic group than in hs-acyanotic and non-hs-acyanotic groups both before 1 year of age (4.8% vs 2.1% vs 1.5%, P < .001) and between 1 and 2 years of age (0.9% vs 0.56% vs 0.14%, P = .003). The hospitalization duration, intensive care, ventilator support prevalence, hospitalization cost, and mortality rate were significantly higher in the cyanotic group than in the other 2 groups. Logistic regression revealed that cyanotic CHD was the most significant risk factor for the ventilator support and RSV-associated mortality. In both patients with cyanotic and acyanotic CHD, RSV-associated hospitalization rate was higher in patients aged younger than 1 year and in spring and autumn in Taiwan, a subtropical country. CONCLUSIONS: The results show that patients with cyanotic CHD have a higher risk of severe RSV infection than do those with acyanotic CHD. RSV prophylaxis is more important and may reduce costs more for patients with cyanotic CHD.

Paradigm shift in the intervention for secundum atrial septal defect in an era of transcatheter closure: A national birth cohort study.

BACKGROUND: Secundum atrial septal defect (ASDII) is a common congenital heart defect, but the intervention, either transcatheter or surgical, needs are unclear. This study was to examine the paradigm shift in its intervention in an era of transcatheter closure. METHODS AND RESULTS: The study birth cohort 2000-2008 (2,070,145 live births) with complete postnatal medical data was derived from the national database (2000-2014) of Taiwan, a country with national health insurance and easily accessible high-standard medical care. We found 5,515 patients with simple ASDII (median follow-up 12.2 years, one-third diagnosed after age 6 years, incidence 2.66/1,000 live births). Excluding patients with coexisting ventricular septal defect, ASDII intervention was performed in 1,435 of 4,585 patients: transcatheter closure in 1,080 (23.6%) and surgery in 355 (7.8%). The interventional need is 0.69 per 1,000 live births: 0.52 per 1,000 for transcatheter closure and 0.17 per 1,000 for surgery. In the era of transcatheter closure (July 2004-December 2014), ASDII intervention increased to 3.56-fold, but the number of operations decreased to half. The freedom from intervention was lower, and the age at intervention was younger (freedom from intervention at age 6 years, 0.749 vs 0.805, P < .001). In patients born in the era of transcatheter closure, 19.5% (108/555) of the intervention was surgery. Atrioventricular block occurred in 1.12% of those patients after transcatheter closure and none after surgery (P = .149). CONCLUSIONS: In the era of catheter intervention, the interventional criteria for ASDII are less strict and one-third of the patients may receive intervention, mostly transcatheter closure, at the pediatric ages.

NHI-PharmaCloud in Taiwan--A preliminary evaluation using the RE-AIM framework and lessons learned.

OBJECTIVES: The aim of this article is to present the preliminary impact of a medication monitoring program, PharmaCloud, in Taiwan and analyze the embedded factors that have contributed to the performance thereof. This article also compared PharmaCloud with similar international programs in order to draw lessons learned. METHODS: The five domains of the RE-AIM framework - reach, effectiveness, adoption, implementation, and maintenance - were examined using qualitative and quantitative data. A difference-in-differences model was applied to analyze the quantitative impact of PharmaCloud on drug utilization and drug expenses. The qualitative impact was evaluated by document analysis based on field reports from the participating medical institutions. RESULTS: Reach and adoption: although all of the major hospitals adopted PharmaCloud and some of the hospitals had high inquiry rates, more time and incentives are needed to raise the overall inquiry rate. Effectiveness: during the study period of 3 months, the number of medications per prescription declined in the intervention group was 0.15 more than that of the general population, and the drug expense per person declined in the intervention group was NT $567 (US $18.9) more than that of the general population. The potential savings could be between 2% and 5% of the total pharmaceutical expenditure. Medication duplication was found to have decreased more in the intervention group. IMPLEMENTATION: a variety of innovations in care delivery are being developed in which the pharmacists play a more significant role. Maintenance: the embedded National Health Insurance would lend strong support for PharmaCloud to grow and thrive. CONCLUSION: PharmaCloud owes its effectiveness to the embedded National Health Insurance (NHI) program, which is universal and provides a comprehensive benefit package including more than 16,000 prescription drugs. An effective medication program is one that operates under the principle of universality and comprehensiveness, facilitates innovations, and has a substantial level of interoperability with the intra-hospital health information systems.

Risk of Systemic Hypertension and Cerebrovascular Accident in Patients With Aortic Coarctation Aged <60 Years (from a National Database Study).

Patients with coarctation of the aorta (CoA) may develop hypertension and cerebrovascular accident (CVA) late after intervention, but the risk is still unclear. Therefore, we studied the epidemiologic profile from a general population aged <60 years to assess the relative risk. Our study population consisted of 126,943 patients with congenital heart disease (CHD) derived from Taiwan's National Health Insurance database from 2000 to 2010 (population 22,765,535). There were 2,295 patients with CoA (50.3% men) (prevalence: 0.116/1000). Associated cardiac lesions in 44% of the patients with CoA included ventricular septal defect (VSD; 23.3%), patent ductus arteriosus (7.5%), atrial septal defect (9.0%), and so forth. Hypertension occurred in 190 patients (8.3%) and was related to age, without associated VSD (odds ratio [OR] 5.90, 95% confidence interval [CI] 2.12 to 16.39, p = 0.001) and male gender (OR 1.59, 95% CI 1.09 to 2.30, p = 0.015). The incidence of hypertension increased rapidly in the young adulthood and was 1.36% in pediatric age group and 28.9% and 45.7% in age group 20 to 29 and 30 to 39, respectively. CVA occurred in 29 patients (1.67%), and the coexisting hypertension was the single predictor (OR 3.19, 95% CI 1.13 to 9.00, p = 0.029). Furthermore, in all patients with CHD, CoA was an independent risk factor for hypertension (OR 15.65, 95% CI 12.44 to 19.68, p <0.001) and CVA (OR 6.55, 95% CI 4.41 to 9.74, p <0.001). Comparing with non-CoA CHD patients, patients with CoA, particularly men, adult, and patients without VSD, have high risk of hypertension, which would further increase the risk of CVA. Early recognition and timely intervention for the hypertension in patients with CoA is mandatory.

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.

Arrhythmic burdens in patients with tetralogy of Fallot: a national database study.

BACKGROUND: Tetralogy of Fallot (TOF) is a common cyanotic congenital heart disease with increasingly recognized late morbidity. OBJECTIVE: The purpose of this study was to explore the long-term outcome by using a national database of Taiwan, a country with national health insurance and easily accessible medical care. METHODS: Data on TOF patients were retrieved from database records from 2000 to 2010. Complications and therapies were identified by their respective codes. RESULTS: We identified 4781 TOF patients (prevalence 0.63/1000 in pediatric patients and 0.06/1000 in adult patients). Arrhythmias were identified in 219 patients (8.3% for adult patients and 2.8% for pediatric patients): 160 tachycardia and 59 bradycardia (4 with tachy-bradycardia syndrome). The occurrence of arrhythmias was associated with higher mortality (excluding cardiac surgical death, 15.6% vs 8.6%, P = .001). Patients with atrial fibrillation were the oldest (median age 44.3 years), followed by those with tachy-bradycardia syndromes (32.4 years) and atrial flutter (31.5 years). The incidence of nonperioperative tachycardia increased with age (1.4%, 1.7%, 3.3%, 5.2%, 10.2%, and 16.9% in age group 0-9, 10-19, 20-29, 30-39, 40-49, and ≥50 years, respectively). Tachycardia therapy (ablation and implantable cardioverter-defibrillator) was administered in 20.4% (annually 2.4%) of patients with nonperioperative tachycardia. In the subgroup born 2000-2010 with complete postnatal data, mortality was 15.1% (296/1960), and 1-, 5-, and 10-year survival was 0.911, 0.826, and 0.788, respectively. Risk of atrioventricular block requiring a pacemaker was 0.6%. CONCLUSION: Arrhythmias are common in TOF patients and increase mortality risk. Medical needs because of tachycardia often appear late in adulthood.

Epidemiological profile of Wolff-Parkinson-White syndrome in a general population younger than 50 years of age in an era of radiofrequency catheter ablation.

BACKGROUND: The prevalence of Wolff-Parkinson-White (WPW) syndrome varies between 0.68 and 1.7/1000. The epidemiological profile may be modified after the introduction of transcatheter interventions. OBJECTIVES: The aim of this study is to investigate the epidemiological trends of the WPW syndrome in a general population during a period with available and reimbursed transcatheter ablation. METHODS AND RESULTS: Data of WPW patients <50 years old were retrieved from our national database (2000-2010). We identified 6086 (61% male) patients, accounting for an overall prevalence of 0.36/1000 with a peak of 0.61/1000 in ages 20-24 years. The risk of death and sudden death was 0.071% and 0.02% per patient-year, respectively. The 42 deaths occurred at a median age of 29 years. Associated congenial heart disease was noted in 158 (2.6%) patients, including 42 with Ebstein's anomaly that increased the mortality risk (P=0.001, OR=8.5). In those without congenital heart disease, myocardial dysfunction occurred in 115 (1.9%) patients and increased the risk of death (P<0.001, OR=10.6) and sudden death. Radiofrequency catheter ablation was performed in 2527 patients at a median age of 25.7 years (4.54% per patient-year, discharge mortality 0.16%); 11 (0.4%) before the age of 5, and 2231 (88%) after the age of 15. Whereas repeated ablation procedures accounted for 6.0% of the procedures, those in Ebstein's patients were 25%. CONCLUSION: Radiofrequency catheter ablation is already a common treatment for WPW patients, particularly during young adulthood, which accounts for a lower prevalence. Myocardial dysfunction and associated congenital heart disease remain as risks of mortality.