COMPERA / COMPERA-KIDS

Официальное название

Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension

Обзор

In view of the manifold options for mono- and combination therapy that have now emerged for patients with pulmonary (arterial) hypertension (PH/PAH), controlled clinical trials can only provide part of the information needed for optimal management. In order to gather adequate data on PAH/PH treatment in routine clinical care, the ongoing COMPERA registry prospectively documents consecutive patients with newly initiated treatment of PAH/PAH since May 2007. The internet-based registry fulfills high quality standards through several measures (planned minimum centre contribution of at least 10 patients per year, automated plausibility checks of data at entry, queries, monitoring with source data verification in \>50% of participating centers). It can be applied, among further purposes, for quality assurance: individual centers can confidentially compare their results with the combined outcome of other centers and the recommendations from guidelines. It is expected that the register contributes to optimization of specific drug therapy for PAH and PH. Since July 2013, also children of any age can be documented (COMPERA-KIDS).

Подробное описание

COMPERA will report current and comprehensive data on

* Demographics and clinical course of incident and prevalent PAH and PH patients * Patient outcomes including survival, by subgroup, by treatment strategy and other factors * Clinical predictors of short-term and long-term clinical outcomes * Relationship between PAH medications and patient outcomes * Temporal trends in treatments and outcomes for newly diagnosed patients * The state of implementation of current PAH guidelines * Evolving research needs of the PAH community * Patients with PAH associated with congenital heart disease and Eisenmenger physiology who do not receive specific drug therapy for PAH ("COMPERA-Eisenmenger", as stated in the amendment dated 23. January 2012). * Children of any age with PH or PAH (all Dana Point groups), as stated in the amendment dated 1 June 2013 ("COMPERA-KIDS").

Первичные конечные точки

• Number of patients on monotherapy vs combination therapies at baseline and during follow-up (drug utilisation patterns) [Срок оценки: Up to 10 years after inclusion]

Вторичные конечные точки (2)

• Number of patients in the various Dana Point groups (patient characteristics in PAH and non-PAH pulmonary hypertension groups) [Срок оценки: Up to 10 years after inclusion]
• Probability of survival in the various Dana Point groups (PAH and non-PAH pulmonary hypertension groups) by Kaplan-Meier estimate [Срок оценки: Up to 10 years after inclusion]

Критерии участия

Критерии включения

• All age groups (amendment dated 1 June 2013)
• Written informed consent
• Pulmonary hypertension (PH) of either

• PAH: idiopathic form (IPAH) or
• PAH associated with connective tissue diseases (PAH-CTD), with congenital heart defects (PAH-CHD), with HIV infection (PAH-HIV), or the portopulmonary form
• Chronic thromboembolic PH (CTEPH)
• PH in left heart diseases (with isolated diastolic dysfunction; with systolic dysfunction, other)
• PH in pulmonary disease (chronic obstructive pulmonary disease; interstitial fibrosis, etc.)
• "Relative PH" in CHD after cavopulmonary anastomosis or Fontan-type surgery, even without the classical pulmonary pressure criteria of PH.
• Newly initiated (i.e. a maximum of 3 months before documentation for the first time) therapy with endothelin receptor antagonists (ERA), phoshodiesterase-5 (PDE-5) inhibitors, soluble guanylate cyclase (sGC) stimulators or prostacyclins in mono- or combination therapy.

Exceptions: PAH-CHD patients can be included on maintenance or newly initiated PAH therapy (3-month rule dose not apply).

PAH-CHD patients with severe pulmonary vascular disease (e.g. Eisenmenger physiology) irrespective of treatment with any PAH drugs are eligible for inclusion, too.

Критерии исключения

• Patients on maintenance therapy, i.e. previous treatment with any ERA/ PDE-5 inhibitor/prostacyclin/sGC stimulator drug longer than 3 months before documentation for the first time (exception: PAH-CHD patients).

Критерии приведены из реестра в оригинале (на английском). Окончательную оценку соответствия проводит исследовательский центр.

Здоровые добровольцы: Нет

Дизайн исследования

Модель наблюдения

Когортное

Центры проведения

Германия · 4 центра

• DRK-Klinikum Köpenick — Berlin
• Lung Centre, University of Giessen — Giessen
• Department of Pulmology; Hannover Medical School — Hanover
• German Heart Centre — Munich

Бельгия · 1 центр

• Dept. of Pneumology, University — Leuven

Италия · 1 центр

• Department of Cardiovascular and Respiratory Sciences, University La Sapienza — Rome

Швейцария · 1 центр

• Dept. for Rheumatology, University Hospital — Zurich

Публикации

• Pittrow D, Ghofrani HA, Opitz CF, Huscher D, Hoeper MM. [International, prospective register for the documentation of first-line and maintenance therapy in patients with pulmonary hypertension (CompERA-XL)]. Dtsch Med Wochenschr. 2009 Aug;134 Suppl 5:S173-5. doi: 10.1055/s-0029-1225318. Epub 2009 Aug 28. German. PMID 19718609
• Pausch C, Pittrow D, Hoeper MM, Huscher D. Performance of the ESC/ERS 4-strata risk stratification model for pulmonary arterial hypertension with missing variables. Eur Respir J. 2023 Nov 29;62(5):2301023. doi: 10.1183/13993003.01023-2023. Print 2023 Nov. PMID 37802633
• Hoeper MM, Huscher D, Ghofrani HA, Delcroix M, Distler O, Schweiger C, Grunig E, Staehler G, Rosenkranz S, Halank M, Held M, Grohe C, Lange TJ, Behr J, Klose H, Wilkens H, Filusch A, Germann M, Ewert R, Seyfarth HJ, Olsson KM, Opitz CF, Gaine SP, Vizza CD, Vonk-Noordegraaf A, Kaemmerer H, Gibbs JS, Pittrow D. Elderly patients diagnosed with idiopathic pulmonary arterial hypertension: results from PMID 23164592
• Olsson KM, Delcroix M, Ghofrani HA, Tiede H, Huscher D, Speich R, Grunig E, Staehler G, Rosenkranz S, Halank M, Held M, Lange TJ, Behr J, Klose H, Claussen M, Ewert R, Opitz CF, Vizza CD, Scelsi L, Vonk-Noordegraaf A, Kaemmerer H, Gibbs JS, Coghlan G, Pepke-Zaba J, Schulz U, Gorenflo M, Pittrow D, Hoeper MM. Anticoagulation and survival in pulmonary arterial hypertension: results from the Comparat PMID 24081973
• Kramm T, Wilkens H, Fuge J, Schafers HJ, Guth S, Wiedenroth CB, Weingard B, Huscher D, Pittrow D, Cebotari S, Hoeper MM, Mayer E, Olsson KM. Incidence and characteristics of chronic thromboembolic pulmonary hypertension in Germany. Clin Res Cardiol. 2018 Jul;107(7):548-553. doi: 10.1007/s00392-018-1215-5. Epub 2018 Feb 15. PMID 29450722
• Hoeper MM, Kramer T, Pan Z, Eichstaedt CA, Spiesshoefer J, Benjamin N, Olsson KM, Meyer K, Vizza CD, Vonk-Noordegraaf A, Distler O, Opitz C, Gibbs JSR, Delcroix M, Ghofrani HA, Huscher D, Pittrow D, Rosenkranz S, Grunig E. Mortality in pulmonary arterial hypertension: prediction by the 2015 European pulmonary hypertension guidelines risk stratification model. Eur Respir J. 2017 Aug 3;50(2):1700740 PMID 28775047
• Hoeper MM, Pittrow D, Opitz C, Gibbs JSR, Rosenkranz S, Grunig E, Olsson KM, Huscher D. Risk assessment in pulmonary arterial hypertension. Eur Respir J. 2018 Mar 29;51(3):1702606. doi: 10.1183/13993003.02606-2017. Print 2018 Mar. No abstract available. PMID 29599117
• Zelniker TA, Huscher D, Vonk-Noordegraaf A, Ewert R, Lange TJ, Klose H, Dumitrescu D, Halank M, Held M, Gall H, Pittrow D, Hoeper MM, Frankenstein L. The 6MWT as a prognostic tool in pulmonary arterial hypertension: results from the COMPERA registry. Clin Res Cardiol. 2018 Jun;107(6):460-470. doi: 10.1007/s00392-018-1207-5. Epub 2018 Jan 24. PMID 29368137

Идентификаторы

NCT: NCT01347216 · COMPERA

Первоисточники (государственные реестры)

• ClinicalTrials.gov (NIH, США)

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