Diagnosis of Pulmonary Hypertension in the Congenital Heart Disease Adult Population: Impact on Outcomes

Study Questions:

What is the impact of the diagnosis of pulmonary hypertension (PH) on mortality, morbidity, and health services utilization in and adult congenital heart disease (ACHD) population?


A retrospective longitudinal cohort study was conducted in an ACHD population from 1983-2005. Provincial databases were searched for ICD-9 codes for PH and the various types of CHD. Mortality, morbidity, and health services utilization outcomes were compared between ACHD patients with and without diagnoses of PH using multivariate Cox regression models for morbidity and mortality, and Poisson regression models for health services utilization.


The prevalence of PH in the ACHD population was 5.8% (2,212 out of 38,439 ACHD patients). Of the PH patients, 59% were women, and the median patient age was 67 years. The all-cause mortality rate of ACHD patients was increased if a diagnosis of PH was present (hazard ratio [HR], 2.69; 95% confidence interval [CI], 2.41-2.99). Heart failure and arrhythmia were also more common in ACHD patients with a diagnosis of PH (HR, 3.01; 95% CI, 2.80-3.22). Health services utilization, including cardiac catheterization and coronary and intensive care hospitalizations, were increased in ACHD patients with PH (rate ratio, 5.04; 95% CI, 4.27-5.93 and rate ratio, 5.03; 95% CI, 4.86-5.20, respectively).


The authors concluded that a diagnosis is associated with significantly increased risk for all-cause mortality and morbidity.


PH is an important complication in the ACHD population. This study demonstrates the prevalence to be almost 6% in the overall ACHD cohort. The study was primarily limited by the use of ICD-9 coding both for the ACHD and PH diagnoses. National registries are currently in place in some countries and under development in others to better understand the prevalence and implications of PH in the ACHD population.

Clinical Topics: Congenital Heart Disease and Pediatric Cardiology, Heart Failure and Cardiomyopathies, Pulmonary Hypertension and Venous Thromboembolism, Congenital Heart Disease, CHD and Pediatrics and Quality Improvement, Acute Heart Failure, Pulmonary Hypertension

Keywords: Registries, Heart Defects, Congenital, International Classification of Diseases, Cardiac Catheterization, Hypertension, Pulmonary, Heart Failure, Hospitalization

< Back to Listings