COMPERA is a pulmonary hypertension (PH) registry that was established in 2007. The name stands for “Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension”. Unlike most other PH registries, COMPERA includes patients with all forms of PH if drug therapy is indicated. COMPERA was originally founded in Germany and is the official German PH registry, but centers from numerous other European countries are now participating. At the time, the registry is one of the largest in its indication worldwide, with nearly 11,000 patients.


Press release: Registry data link smoking to the development of a severe form of pulmonary hypertension

The COMPERA registry announces the publication of their study entitled "Phenotyping of idiopathic pulmonary arterial hypertension: a registry analysis" in The Lancet Respiratory Medicine (https://www.thelancet.com/journals/lanres/article/PIIS2213-2600(22)00097-2/fulltext).1 This analysis was conducted in close collaboration with the ASPIRE registry from Sheffield, UK, and focused on patients diagnosed with idiopathic pulmonary arterial hypertension (IPAH). In this study investigators provide evidence that smoking may play an important role in the development of an increasingly recognized form of severe pulmonary hypertension, which responds poorly to medical therapy and has a very high mortality risk.
Pulmonary hypertension (PH), an elevation of blood pressure in the lungs, may affect up to 1% of the world's adult population and is most commonly seen in patients already diagnosed with heart and lung conditions.2 There are many forms of PH, and progressive breathlessness is the most common symptom.  IPAH is a rare and very severe form of PH for which drug therapies are available. It is called "idiopathic" to reflect the absence of an obvious cause. IPAH was previously thought to affect mostly younger women. However, recent studies have shown that this form of PH is increasingly diagnosed in older patients.
In their study, the authors compared patients diagnosed with IPAH with a history of (mostly heavy) smoking who had a severe problem transferring oxygen from the alveoli to the blood vessels in the lungs (diffusion capacity) to those with the "classical" form of IPAH. This diffusion capacity was measured using a simple breathing test. They also compared the outcome of these two separate groups to patients with PH and lung conditions such as COPD, emphysema, and lung fibrosis where lung involvement is thought to damage the blood vessels in the lungs.
The authors noted that smokers with a low diffusion capacity were very similar to patients with pulmonary hypertension and known lung conditions with a similar age and sex distribution, and a very poor survival. This was very different to patients with "classical IPAH" who were younger, more often female, and with a much better response to treatment. The authors propose that those patients previously considered to have IPAH who have problems transferring oxygen into the blood vessels in the lungs and a history of smoking should be distinguished from patients with classical IPAH.
This study lends strong support to the hypothesis that smoking affects not only the airways but can also cause direct damage to the pulmonary blood vessels as it has previously been demonstrated in animal models.3 These data challenge how doctors classify PH. The findings are important not only because they identify a potentially avoidable cause of PH, smoking, but impact on how we treat patients with PH and how we plan for trials of new therapies in the future.
Professor Hoeper from Hannover in Germany who led the study commented "These findings support the theory that smoking can cause a distinct problem with the blood vessels in the lungs and that the pulmonary vascular injury can occur in the presence or absence of typical pulmonary manifestations of smoking, such as COPD or emphysema. This improves our understanding of PH and will help doctors to better counsel patients in addition to helping to design better drug trials in the future".

About COMPERA: COMPERA (Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension) is an academic European PH registry launched in 2007 (www.compera.org). With more than 10,000 patients enrolled, COMPERA is one of the largest PH registries worldwide. COMPERA is led by a group of European PH specialists. The registry is funded by unrestricted grants from pharmaceutical companies, which are not involved in data analysis and scientific publications. Legal sponsor of the registry is GWT-TUD in Dresden, Germany.  ClinicalTrials.gov identifier is NCT01347216.
Contact COMPERA: Prof Marius M Hoeper MD, Department of Respiratory Medicine, Hannover Medical School, Hannover, Germany (hoeper.marius@mh-hannover.de).
About ASPIRE: ASPIRE (Assessing the Severity of Pulmonary Hypertension In a Pulmonary Hypertension REferral Centre) is a PH registry launched in 2001 in Sheffield, UK (www.sheffield.ac.uk/donaldheath/our-research). The ASPIRE registry is supported by Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK.
Contact ASPIRE: Prof David G Kiely MD, Sheffield Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital and Department of Infection, Immunity and Cardiovascular Disease, University of Sheffield, Sheffield, UK (david.kiely1@nhs.net).
About The Lancet Respiratory Medicine: The Lancet Respiratory Medicine (www.thelancet.com/lanres/about) was launched in 2013 as Lancet subspeciality journal. The Lancet Respiratory Medicine has a 2022 Impact Factor >100 and has become the world-leading journal in the field of respiratory journals.

References
1. Hoeper MM, Dwivedi K, Pausch C, et al. Phenotyping of idiopathic pulmonary arterial hypertension: a registry analysis. The Lancet Respiratory Medicine 2022. DOI: 10.1016/s2213-2600(22)00097-2.
2. Hoeper MM, Humbert M, Souza R, et al. A global view of pulmonary hypertension. The lancet Respiratory medicine 2016;4(4):306-22. DOI: 10.1016/S2213-2600(15)00543-3.
3. Seimetz M, Parajuli N, Pichl A, et al. Inducible NOS Inhibition Reverses Tobacco-Smoke-Induced Emphysema and Pulmonary Hypertension in Mice. Cell 2011;147(2):293-305. (In eng). DOI: 10.1016/j.cell.2011.08.035.

 


At a glance

Pulmonary (arterial) hypertension (PH, PAH) occurs when the pressure in the blood vessels leading from the heart to the lungs is too high (CDC). The term PH embraces a variety of diseases that have little in common apart from elevated blood pressure in the pulmonary circulation (Hoeper et al, Dt. Ärzteblatt 2017). For some forms of PH, there are manifold options for mono- and combination therapy, for others, there is no established therapy. Patient organisations on the international (PHA Europe) and the national level (pulmonale hypertonie e.V. und Pulmonale Hypertonie Selbsthilfe) provide information and services to PH patients. 

COMPERA, established in 2007, is a structured, non-interventional (observational) registry in PH expert centers. It documents PH therapy of patients of all age groups, with any form of PH or pulmonary arterial hypertension (PAH). With nearly 11,000 patients and more than 85,000 follow-up visits, the registry is one of the largest in its indication. 

It is fully internet-based, and fulfils high quality standards through several measures like automated plausibility checks of data at entry, queries, and source data verification. Of course, COMPERA has been approved by the ethics committees of all participating centers, and data protection is ensured because all data are completely pseudonymized, so there is no possibility of inferring individual persons outside the clinic.

Current information about the registry can be found at ClinicalTrials.gov under the identifier NCT01347216.

The great value of COMPERA lies in the very detailed description of patient characteristics and their long-term follow-up. The registry serves several purposes: it provides real-world evidence data that complement randomised controlled trials in PH. On a center basis, data are useful for quality assurance: individual centers can confidentially compare their results with the combined outcome of other centers and the recommendations from guidelines. Results may inform planners of clinical studies and authors of clinical guidelines.

People affected by PH cannot register for participation in COMPERA themselves, but may contact one of the participating expert centers (see author list in the publications). Enrollment of additional centers is possible.

The registry has been initiated by investigators (researchers) and is fully independent of the pharmaceutical industry. It is being financed with unrestricted education funds from Acceleron, Bayer, Janssen and OMT.


Publications

Results from COMPERA are published in international scientific journals in regular intervals. The registry has already produced numerous highly published scientific papers that have influenced the way we understand and treat the disease. To date, the following 20 peer-reviewed publications have been published, with the majority of articles being available free-of-charge in Medline

Hoeper et al: Phenotyping of idiopathic PAH: Lancet Resp Med 2022 (in press)
Hoeper et al: Prognostic value of improvement endpoints in PAH trials. J Heart Lung Transplant 2022 (in press)
Kaemmerer et al.: CHD-PAH. Cardiovasc Diagn Ther 2021
Hoeper et al.: COMPERA 2.0 Risk Assessment. Eur Resp J 2021
Olsson et al.: PVR and Risk. Eur Resp J 2021
Hoeper et al.: 10-year temporal trends in PAH. Eur Resp J 2021
Vizza et al.: PAH-COPD. Chest 2021
Kanwar et al.: Risk stratification in pulmonary arterial hypertension using Bayesian analysis. Eur Resp J 2020
Hoeper et al.: Cluster analysis of PAH patients. J Heart Lung Transplant 2020
Kaemmerer et al.: PAH in patients with congenital heart disease. J Clin Med 2020
Delcroix et al.: Risk assessment in CTEPH. Eur Resp J 2018
Zelniker et al.: 6-min walk distance as prognostic tool. Clin Res Cardiol 2018
Kramm et al.: CTEPH in Germany. Clin Res Cardiol 2018
Hoeper et al.: Risk stratification in PAH. Eur Resp J 2017
Opitz et al.: Pre- and postcapillary PH. J Am Coll Cardiol 2016
Hoeper et al.: PAH epidemiology in Germany. Int J Cardiol 2015
Hoeper et al.: PAH associated with chronic fibrosing interstitial lung diseases. PLOS One 2015
Olsson et al.: Anticoagulation in PAH. Circulation 2013
Hoeper et al.: PAH in the elderly. Int J Cardiol 2011
Pittrow et al. COMPERA first results. Dtsch Med Wochenschr 2009


Data protection

In the COMPERA database, patient names or birth dates are not stored. Only physicians at the hospitals know the identity of their patients. Data are entered via secure internet connections, and stored password-protected on secured servers. They are not made availble in online repositories. The General Data Protection Guidelines are closely observed.


Data Sharing

Principally, data from COMPERA are available to other reviewers upon request. Interested researchers may submit requests for minimized anonymous dataset by contacting the study leader. Collaborations require a formal data sharing agreement. Data protection is closely observed.


Contact

Patients cannot apply directly for participation in the registry.
Physicians in PH expert sites who consider participation, kindly send an e-mail to hotline@compera.org.
Researchers who wish to suggest collaborations, please contact Hoeper.Marius@mh-hannover.de .
Journalists kindly contact hotline@compera.org to receive more information.

 

 

 


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