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

https://doi.org/10.1016/j.jacc.2011.03.033Get rights and content
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Objectives

The aim of this study was to assess the impact of the diagnosis of pulmonary hypertension (PH) on mortality, morbidity, and health services utilization (HSU) in an adult congenital heart disease (CHD) population.

Background

Although PH is a well-recognized complication of CHD, population-based studies of its significance on the survival and functional capacity of patients are uncommon.

Methods

A retrospective longitudinal cohort study was conducted in an adult CHD population with 23 years of follow-up, from 1983 to 2005. The prevalence of PH was measured in 2005. Mortality, morbidity, and HSU outcomes were compared between patients with and without diagnoses of PH using multivariate Cox (mortality and morbidity) and Poisson (HSU) regression models within a subcohort matched for age and CHD lesion type.

Results

Of 38,430 adults alive with CHD in 2005, 2,212 (5.8%) had diagnoses of PH (median age 67 years, 59% women). The diagnosis of PH increased the all-cause mortality rate of adults with CHD more than 2-fold compared with patients without PH (hazard ratio [HR]: 2.69; 95% confidence interval [CI]: 2.41 to 2.99). Morbid complications including heart failure and arrhythmia occurred with a 3-fold higher risk compared with patients without PH (HR: 3.01; 95% CI: 2.80 to 3.22). The utilization of inpatient and outpatient services was increased, especially cardiac catheterization, excluding the index diagnostic study (rate ratio: 5.04; 95% CI: 4.27 to 5.93) and coronary and intensive care hospitalizations (rate ratio: 5.03; 95% CI: 4.86 to 5.20).

Conclusions

A diagnosis of PH in adults with CHD is associated with a more than 2-fold higher risk for all-cause mortality and 3-fold higher rates of HSU, reflecting high morbidity.

Key Words

congenital heart defect
morbidity
mortality
population
pulmonary hypertension

Abbreviations and Acronyms

ASD
atrial septal defect
CHD
congenital heart disease
CI
confidence interval
HR
hazard ratio estimated from Cox proportional hazards regression
HSU
health services utilization
ICD-9
International Classification of Diseases-Ninth Revision
PH
pulmonary hypertension
RR
rate ratio estimated from Poisson regression
VSD
ventricular septal defect

Cited by (0)

Dr. Lowe was supported in part by Actelion Pharmaceuticals Canada and by the Beth Raby Adult Congenital Heart Disease Fellowship of the Jewish General Hospital. Dr. Pilote is supported by the James McGill Research Chair and Fond de la Recherché en Santé du Québec. Dr. Marelli is supported by the Heart and Stroke Foundation of Canada and the Heart and Stroke Foundation of Quebec. All other authors have reported that they have no relationships to disclose.