ArticleCardiovascular diagnosis and therapy2021
Medical treatment of pulmonary hypertension in adults with congenital heart disease: updated and extended results from the International COMPERA-CHD Registry.
Article in Cardiovascular diagnosis and therapy, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT01347216 (Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension), which is not on this map. Cited by 11 papers.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension
Who cites it
11 citing papers in PubMed, 18 citations in OpenAlex.
- Genetic Syndromes and Multimorbidity in Adults with Congenital Heart Disease and Heart Failure: Insights from the PATHFINDER-CHD Registry.Journal of clinical medicine · 2026Article
- Pulmonary arterial compliance as a long-term prognostic indicator in pulmonary arterial hypertension associated with adult congenital heart disease: results from a National multicenter prospective registry.BMC cardiovascular disorders · 2025Article
- Article
- Establishing a cardiology registry: navigating quality and regulatory challenges with a focus on congenital heart disease.Cardiovascular diagnosis and therapy · 2025Review
- Pulmonary hypertension in adults with congenital heart defects (ACHDs)-in light of the 2022 ESC PAH guidelines-part I: definition, epidemiology, classification, diagnostics, genetics, risk stratification and follow-up, gender aspects.Cardiovascular diagnosis and therapy · 2024Review
- Treatment algorithm for pulmonary arterial hypertension.The European respiratory journal · 2024Review
- Mortality from pulmonary hypertension in Europe 2001-2019.BMC pulmonary medicine · 2024Article
- Unique Pulmonary Hypertensive Vascular Diseases Associated with Heart and Lung Developmental Defects.Journal of cardiovascular development and disease · 2023Review
- Evaluating the role of serum uric acid in the risk stratification and therapeutic response of patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD).Frontiers in pharmacology · 2023Article
- Normal reference values of tricuspid annular dimensions and functional properties in healthy adults using three-dimensional speckle-tracking echocardiography (insights from the MAGYAR-Healthy Study).Quantitative imaging in medicine and surgery · 2023Article
- Long-term course of pulmonary arterial hypertension in adults with congenital heart disease under targeted therapy: a retrospective analysis of a single tertiary center.Cardiovascular diagnosis and therapy · 2022Article
Corrections and comments
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Authors and funding
44 authors at 15 institutions in 4 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundPulmonary arterial hypertension (PAH) is common in congenital heart disease (CHD). Because clinical-trial data on PAH associated with CHD (PAH-CHD) remain limited, registry data on the long-term course are essential. This analysis aimed to update information from the COMPERA-CHD registry on management strategies based on real-world data.
methodsThe prospective international pulmonary hypertension registry COMPERA has since 2007 enrolled more than 10,000 patients. COMPERA-CHD is a sub-registry for patients with PAH-CHD.
resultsA total of 769 patients with PAH-CHD from 62 specialized centers in 12 countries were included into COMPERA-CHD from January 2007 through September 2020. At the last follow-up in 09/2020, patients [mean age 45.3±16.8 years; 512 (66%) female] had either post-tricuspid shunts (n=359; 46.7%), pre-tricuspid shunts (n=249; 32.4%), complex CHD (n=132; 17.2%), congenital left heart or aortic valve or aortic disease (n=9; 1.3%), or miscellaneous CHD (n=20; 2.6%). The mean 6-minute walking distance was 369±121 m, and 28.2%, 56.0%, and 3.8% were in WHO functional class I/II, III or IV, respectively (12.0% unknown). Compared with the previously published COMPERA-CHD data, after 21 months of follow-up, the number of included PAH-CHD patients increased by 91 (13.4%). Within this group the number of Eisenmenger patients rose by 39 (16.3%), the number of "Non-Eisenmenger PAH" patients by 45 (26.9%). Currently, among the 674 patients from the PAH-CHD group with at least one follow-up, 450 (66.8%) received endothelin receptor antagonists (ERA), 416 (61.7%) PDE-5 inhibitors, 85 (12.6%) prostacyclin analogues, and 36 (5.3%) the sGC stimulator riociguat. While at first inclusion in the COMPERA-CHD registry, treatment was predominantly monotherapy (69.3%), this has shifted to favoring combination therapy in the current group (53%). For the first time, the nature, frequency, and treatment of significant comorbidities requiring supportive care and medication are described.
conclusionsAnalyzing "real life data" from the international COMPERA-CHD registry, we present a comprehensive overview about current management modalities and treatment concepts in PAH-CHD. There was an trend towards more aggressive treatment strategies and combination therapies. In the future, particular attention must be directed to the "Non-Eisenmenger PAH" group and to patients with complex CHD, including Fontan patients.
trial registrationwww.clinicaltrials.gov, study identifier: Clinicaltrials.gov NCT01347216.
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