Congenital Heart Disease-Associated PAH vs Idiopathic PAH Survival Rates

congenital heart disease
Overall survival in congenital heart disease-associated pulmonary hypertension has been shown to be significantly better than in idiopathic pulmonary arterial hypertension.

The overall survival in individuals with congenital heart disease-associated pulmonary hypertension (PH) depends on their underlying diagnosis and treatment status, but has been shown to be significantly better than the overall survival in those with idiopathic pulmonary arterial hypertension (PAH).

A subgroup of patients from the international Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA) with PH related to congenital heart disease (COMPERA-CHD) were analyzed. Results of the study were published in the Journal of Clinical Medicine.

Investigators sought to describe current management strategies and outcomes in adults with PH relative to different types of congenital heart disease and based on real-world data. Overall, 680 (8.3%) of the 8200 adult patients with PH enrolled in COMPERA with a diagnosis of PAH related to congenital heart disease were eligible for the current analysis. The patients were included between 2007 and 2018 in 49 specialized centers for PH related to congenital heart disease, located in 11 European countries.

The median patient age was 44 years at enrollment. Overall, 66.6% of the participants were women. The patients had pre-tricuspid shunts, post-tricuspid shunts, complex congenital heart disease, congenital left heart or aortic disease, or miscellaneous other types of congenital heart disease. At study inclusion, the following targeted therapies for PAH were being used by the participants: endothelin receptor antagonists, phosphodiesterase type-5 inhibitors, prostacyclin analogues, and soluble guanylate cyclase stimulators.

All of the participants underwent a complete noninvasive evaluation upon study enrollment. Cardiac catheterization was performed in 71.6% (n=487) of the patients. In those patients who did not undergo cardiac catheterization, their PAH diagnosis was determined via the use of noninvasive imaging and clinical evaluation.

Of 680 patients in COMPERA-CHD with congenital heart disease-associated PAH, 47.1% (n=320) had a proven Eisenmenger syndrome, 24.6% (n=167) had “non-Eisenmenger PAH,” and 1.0% (n=7) had a Fontan circulation. An additional 27.4% (n=186) of the participants were receiving targeted PAH medication but could not be clearly categorized based on the data entered. Of the 320 patients with Eisenmenger syndrome, 80 were treatment-naive.

At inclusion, the primary PAH treatment for the cohort was monotherapy in 70% of the patients, whereas 30% of the patients were receiving combination therapy. Following a median observation time of 45.3 months, the number of patients receiving combination therapy increased significantly, to 50%. Oral anticoagulant or antiplatelet use depended on the patient’s underlying diagnosis or comorbidities.

Of the patients who received targeted PAH therapy, 91 with congenital heart disease-PAH and 419 with idiopathic PAH died over the course of the 5-year follow-up. Survival in patients with congenital heart disease who received targeted PAH treatment was significantly superior to the survival in patients with idiopathic PAH, with a 5-year Kaplan-Meier survival estimate of 76% for those with congenital heart disease-PAH vs 54% for those with idiopathic PAH (P <.001).

The investigators concluded that their findings suggest that the characteristics of congenital heart disease must be taken into consideration in the diagnosis and treatment of those with the disease, since the management of PAH-congenital heart disease often differs from that of other types of PAH.


Kaemmerer H, Gorenflo M, Huscher D, et al. Pulmonary hypertension in adults with congenital heart disease: real-world data from the international COMPERA-CHD registry [published online May 13, 2020]. J Clin Med. doi:10.3390/jcm9051456