No abstract

BackgroundThe 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.ConclusionsWe 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.

By using a National Health Insurance database, we investigated the occurrence of Kawasaki disease (KD) shock syndrome in Taiwan. KD shock syndrome occurred most frequently in winter, and the incidence was 1.45 per 100 patients with KD. Compared with KD patients without shock, KD shock syndrome patients were older, more likely to develop coronary lesions (15.9% vs. 7.0%) and had longer hospitalization duration.

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.

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.