Abstract 18398: Long-Term Incidence of Pulmonary Hypertension in Adults With Congenital Heart Disease Compared to the General Population: A Nationwide Cohort Study
Background: The population of adults with congenital heart disease (CHD) is growing and ageing. One potential complication of CHD is pulmonary hypertension (PH), a severe condition that results in limited functional capacity and increased mortality. Few data exist on the incidence of adult onset PH in the CHD population. This study estimates the incidence of PH in the adult CHD-population compared to the general population.
Methods: In two Danish nationwide registries, we identified all individuals diagnosed with CHD in the years 1963-1974 and 1977-2012. Individuals were matched 1:10 by birth year and gender with individuals from the general population. Adults over 18 years were followed during January 1st 1977 to January 1st 2013 for PH-diagnosis, using data from the Danish National Registry of Patients (DNRP), a nationwide hospital discharge registry covering all Danish hospitals. Study subjects with PH-related hospitalization occurring before age 18, or before the CHD diagnosis (if this was made after 18 years of age), were excluded. A unique personal identifier assigned at birth enabled complete follow-up until PH, death, emigration or end of study. We computed incidence rates and cumulative incidences of PH.
Results: We identified 15,263 adult CHD survivors. By 70 years of age the cumulative incidence of PH was 7.8 % in all individuals with CHD, 8.3 % in individuals with systemic-to-pulmonary shunt lesions and 7 % in other heart defects, compared to 0.5 % in the general population comparison cohort. The PH incidence rate was 85 per 100,000 person years in the CHD population aged 18-50 years and it was 441 in the CHD population aged >50 years.
Conclusion: Adult onset PH is a common long-term complication of CHD. The risk is not exclusive to those with a history of systemic-to-pulmonary shunt lesions. The PH incidence rate in the adult CHD population increases with age.
Author Disclosures: M. Olsen: None. N. Madsen: None. S.S. Schwartz: None.
- © 2016 by American Heart Association, Inc.