An open-label phase IIIb study of riociguat in patients with in-operable CTEPH, or recurrent or persisting pulmonary hypertension (PH) after surgical treatment who are not satisfactorily treated and cannot participate in any other CTEPH trial
The study will be conducted as an open-label, uncontrolled long-term surveillance study to assess safety, tolerability, and clinical effects while providing early access of riociguat to patients with inoperable Chronic Thromboembolic Pulmonary Hypertension (CTePH), or recurrent or persisting PH after surgical treatment that are not satisfactorily treated and cannot participate in any other CTePH trial.
There are no primary variables. Within this open-label long- term surveillance study safety
and tolerability as well as clinical effects will be measured.
Patients must have given their written informed consent to participate in the study
after having received adequate previous information and prior to any study-specific
* 18 to 80 years of age at screening
* Patients with inoperable CTEPH
* The diagnosis is based on (performed max. 1 year prior to screening)
o Historical pulmonary angiogram , and/or
o CT or MR pulmonary angiogram , and/or
o Ventilation /Perfusion (V/Q) Scan AND
o Historical right heart catheter test (performed max. 1 year prior to screening)
confirming mean pulmonary arterial pressure (mPAP) > 25 mmHg and pulmonary
capillary wedge pressure (PCWP) <= 15 mmHg
o Patients on full anti-coagulation therapy for at least 3 months prior to entry into study
o Diagnosis has to be confirmed by a surgeon/physician experienced in diagnosing and
treating CTEPH (see Declaration of Experienced Investigator or Surgeon/Physician in
* Post-PEA patients with recurrent or residual PH having at least 90 days of full anticoagulation
o Right heart catheter (RHC) test measured 180 days after surgery confirming:
o mPAP > 25 mmHg and pulmonary capillary wedge pressure (PCWP) <= 15 mmHg
o Pulmonary vascular resistance (PVR)>300 dyn*sec*cm-5
* No treatment with riociguat or PDE5-inhibitor, endothelin receptor antagonist (ERA)
or prostanoid with a minimum time frame of at least 3 days, or more at the discretion
of the investigator prior to start of riociguat treatment.
* Patients who are able to understand and follow instructions and who should be able to
participate in the study for the entire period.
* Unspecific treatments which may also be used for the treatment of pulmonary
hypertension such as oral anticoagulants, diuretics, digitalis, calcium channel blockers
or oxygen supplementation are permitted. However, treatment with anticoagulants
must have been started at least 90 days before Week 0 (Visit 1)
* Women without childbearing potential defined as postmenopausal women (=
permanent absence of monthly periods for more than 2 years), women with bilateral
tubal ligation, women with bilateral ovarectomy, and women with hysterectomy can
be included in the study. Women with childbearing potential can only be included in
the study if a serological pregnancy test is negative and a combination of safe
contraception methods is used throughout the study.
* Women of childbearing potential and men must agree to use adequate contraception
when sexually active. This applies since signing of the informed consent form until the
time point of safety follow-up of 30 days after the last study drug administration.
Acceptable methods of contraception include (i) condoms (male or female) with or
without a spermicidal agent; (ii) diaphragm or cervical cap with spermicide; (iii) intrauterine
device; (iv) hormone-based contraception.