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, <0.0001). Percentage of severe ACHD also increased over time (<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.