compera / compera-kids

Conditions: Pulmonary Arterial Hypertension (PAH); Pulmonary Hypertension (PH)

Summary: 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).

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").

Eligibility criteria:
inclusion criteria: * 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. exclusion criteria: * 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).

Keywords: drug treatment treatment pathways clinical routine registry treatment outcomes endothelin receptor antagonist phosphodiesterase-v inhibitor prostanoid sildenafil bosentan macitentan sitaxentan ambrisentan imatinib epoprostenol treprostinil iloprost infusion pump riociguat soluble guanylate cyclase stimulator quality of life patient-related outcomes economic model adverse event safety combination therapy internet europe eisenmenger adults children selexipag baltic states risk