Incidence and Mortality of Adults With Pulmonary Hypertension and Congenital Heart Disease.

Reports on pulmonary hypertension (PH) in the aging congenital heart disease (CHD) population are few and focused on arterial PH and patients with systemic-to-pulmonary shunts. Our objective was to estimate incidence and mortality of adult-onset PH in the CHD population. Using Danish nationwide registries, we identified all patients diagnosed with CHD from 1963 to 1974 and 1977 to 2012. Patients were matched 1:10 by birth year and gender with general population subjects. Between 1977 and 2013 adults >18 years of age were followed up until PH diagnosis, death, or emigration, whichever came first, using data from the Danish National Registry of Patients. We computed cumulative incidences of PH. Using Cox regression, we compared the mortality rate between CHD subjects with and without PH matched by gender and birth year. We identified 14,860 patients with CHD. At 70 years of age, their overall cumulative incidence of PH was 7.2% (8.3% in those with systemic-to-pulmonary shunts and 5.3% in those without) compared with 0.4% in the general population. The 1-, 5-, and 10-year mortality for adults with CHD and PH was 24%, 44%, and 52%, respectively. This represented a 4-fold (95% confidence interval 3.3 to 5.6) increase in mortality compared with adults with CHD without PH after adjusting for gender, birth year, CHD severity, and presence of extracardiac defects. In conclusion the incidence of PH was substantially increased in adults with CHD relative to the general population. Of note, the increased incidence was not limited to those with a history of systemic-to-pulmonary shunts. PH was associated with increased mortality.

Long-Term Nationwide Follow-Up Study of Simple Congenital Heart Disease Diagnosed in Otherwise Healthy Children.

BACKGROUND: Systematic follow-up is currently not recommended for patients with simple congenital heart disease; however, only a few data exist on the long-term prognosis of simple congenital heart disease. METHODS AND RESULTS: We undertook a nationwide follow-up study of a cohort of 1241 simple congenital heart disease patients, diagnosed from 1963 through 1973, in otherwise healthy children and alive at 15 years of age. We identified 10 age- and sex-matched general population controls per patient. We followed the study population through Danish public registries from the age of 15 years up to January 1, 2013 with respect to mortality, cause of death, morbidity, and medical follow-up. The patients were followed for a total of 58 422 patient-years and had a median age at the end of follow-up of 47.4 years (interquartile range, 43.5-50.9). Mortality was increased compared with the general population, both overall (adjusted hazard ratio [aHR],1.9; 95% confidence interval [CI], 1.5-2.4)] and for patients (79%) without medical follow-up (aHR, 1.7; 95% CI, 1.3-2.2). The most common cause of death (40%) was sudden unexpected death (aHR, 4.3; 95% CI, 2.9-6.5). The incidence of critical cardiac morbidity was 3.9 per 1000 patient-years with the most frequent events being an adult (re)operation and hospitalization for heart failure or ventricular tachyarrhythmia. This corresponded to an aHR of 5.7 (95% CI, 4.6-6.9) when compared with the general population. CONCLUSIONS: Patients diagnosed with simple congenital heart disease in the 1960s have substantially increased long-term mortality and cardiac morbidity compared with the general population. Further studies on the effectiveness of systematic medical follow-up programs appear warranted.