Idiopathic Pulmonary Arterial Hypertension
Conditions
Keywords
Pulmonary Hypertension, Pulmonary Arterial Hypertension, TheraSorb, immunoadsorption
Brief summary
This clinical investigation is a medical device trial to examine the safety and efficacy of TheraSorb® Ig flex adsorber treatment (as an add-on to conventional treatment) used exclusively with the LIFE 18® apheresis system for extracorporeal application for IA therapy (5 treatments) performed over 5 to 8 consecutive days in patients with Idiopathic Pulmonary Arterial Hypertension and WHO functional classification III.
Interventions
DEVICETheraSorb® - Ig flex
TheraSorb® Treatment with one TheraSorb® Ig flex adsorber pair, used exclusively with the LIFE 18® apheresis system for extracorporeal application for IA therapy. 5 treatments performed over 5 to 8 consecutive days.
Sponsors
Miltenyi Biotec B.V. & Co. KG
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
TREATMENT
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
18 Years to 80 Years
Healthy volunteers
No
Inclusion criteria
* Able to understand and willing to sign the Informed Consent Form * age \> 18 and \< 80 years at time of informed consent * Idiopathic pulmonary arterial hypertension diagnosed by right heart catheter * WHO functional classification III * Application of conventional IPAH therapy, which has been stable for the prior 8 weeks * Able to perform a 6-minute-walk test (MWT) * Negative pregnancy test (β-HCG) at the start of the trial and appropriate contraception throughout the trial for women with child-bearing potential.
Exclusion criteria
* Pregnancy and/or lactation * PAH of any cause other than permitted in the inclusion criteria, e.g. concomitantly to portal hypertension, complex congenital heart disease, reversed shunt, HIV infection, suspected pulmonary veno-occlusive disease * Contraindication for right heart catheterization * Any change in disease-targeted therapy within 8 weeks prior to inclusion * Walking impairment due to causes other than IPAH that would preclude performance of a 6MWT * Any patient who has received any investigational medication within 8 weeks prior to the start of this trial or who is scheduled to receive another investigational drug during the course of the trial between visits V1 and V9 * Known intolerance to immunoadsorption in general or to one of the excipients (e.g. sheep antibodies or agarose) or other supporting agents * Hemoglobin concentration of \< 75% below the lower limit of normal * Systolic blood pressure \< 85 mmHg * Lack of compliance or other similar reason that, in the judgment of the Investigator, precludes satisfactory participation in the trial * Concurrent severe or uncontrolled medical disease (i.e. uncontrolled diabetes, congestive heart failure, myocardial infarction within 6 months prior to the study, unstable and uncontrolled hypertension, chronic renal disease, or active uncontrolled generalized viral, bacterial and/ or mycotic infection) which by assessment of the treating Investigator could compromise patients safety or participation in the study * Drug or alcohol abuse within the last 5 years * Hypercoagulability * Known severe immunodeficiency (e.g. AIDS) * Severe lung disease: FEV1/FVC \< 50%, total capacity \< 60% of normal value * Indications that prohibit transient anticoagulation using Heparin and/or ACD-A-solutions
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Primary endpoint is the change of pulmonary vascular resistance (PVR) at rest before immunoadsorption compared to 3 months after immunoadsorption therapy determined by right heart catheterization (RHC). | Baseline, 3 months after therapy |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| occurrence and number of Adverse Events | ~6 months | The patients will be followed for the duration of the study participation, an expected average of 6 months. |
| Six-minute walk test (6MWT) | Measurements at baseline, ~1 week, ~1 month, ~3 months and ~6 months after start of therapy | — |
| Quality of life questionnaires | Measurements at baseline, and after ~1 month, ~3 months, ~6 months | SF-36 EQ-5D™ |
| all-cause mortality | ~6months | The patients will be followed for the duration of the study participation, an expected average of 6 months. |
| Analysis of Hemodynamics by right heart catheterization: RAP, PAPm, CO, CI, SvO2; sPAP | Measurements at baseline, after ~3 months | (Right atrial pressure, mean pulmonary arterial pressure, pulmonary capillary wedge pressure, cardiac output, cardiac index, venous oxygen saturation, systolic pulmonary arterial pressure.) |
| Reduction of IgG | Measurements at baseline, during each treatment visit, ~1 week, ~1 month and ~3 months after start of treatment | — |
| WHO functional classification | Measurements at baseline, ~1 week, ~1 month, ~3 months and ~6 months after start of therapy | WHO functional classification of pulmonary hypertension (according to the Evian Symposium,1998, modified New York Heart Association (NYHA) Classification |
Countries
Germany
Outcome results
None listed