COMPERA / 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").

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).