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A Multicenter, Double-blind, Randomized, Placebo-controlled, Parallel group, Event driven, Phase III study to assess the effects of ACT-064992 on Morbidity and Mortality in patients with Symptomatic Pulmonary Arterial Hypertension - SERAPHI

Conditions
Pulmonary Arterial Hypertension
MedDRA version: 9.1Level: LLTClassification code 10037405Term: Pulmonary hypertension primary
Registration Number
EUCTR2007-002440-14-IT
Lead Sponsor
Actelion Pharmaceuticals Ltd.
Brief Summary

Not available

Detailed Description

Not available

Recruitment & Eligibility

Status
ot Recruiting
Sex
All
Target Recruitment
525
Inclusion Criteria

Signed informed consent prior to initiation of any study mandated procedure.Patients with symptomatic pulmonary arterial hypertension (PAH) in modified WHO functional class II to IV.Patients with the following types of PAH belonging to groups 1.1 to 1.3 of the Venice classification:a. Idiopathic (IPAH); b. Familial (FPAH); or c. Related to: i. Collagen vascular disease;ii. Simple, congenital systemic-to-pulmonary shunts at least 1 year post surgical repair;iii. HIV infection; oriv. Drugs and toxins.PAH diagnosis confirmed by hemodynamic evaluation performed prior to randomization and showing all of the following: a. Mean pulmonary artery pressure (mPAP) = 25 mmHg at rest,b. Pulmonary capillary wedge pressure (PCWP) or leftventricular end diastolic pressure (LVEDP) < 15 mmHg,andc. Pulmonary vascular resistance (PVR) at rest= 240 dyn·sec/cm5.
Are the trial subjects under 18? yes
Number of subjects for this age range:
F.1.2 Adults (18-64 years) yes
F.1.2.1 Number of subjects for this age range
F.1.3 Elderly (>=65 years) yes
F.1.3.1 Number of subjects for this age range

Exclusion Criteria

PAH associated with portal hypertension, thyroid disorders, glycogen storage disease, Gaucher??s disease, hereditary hemorrhagic telangiectasia, hemoglobinopathies, myeloproliferative disorders and splenectomy.PAH associated with non corrected simple congenital systemic-to-pulmonary shunts, and combined and complex systemic-to-pulmonary shunts, corrected or non corrected.PAH Associated with significant venous or capillaryinvolvement (PCWP > 15 mmHg), Pulmonary veno-occlusive disease and Pulmonary capillary hemangiomatosis.Persistent pulmonary hypertension of the newborn.Pulmonary Hypertension belonging to groups 2 to 5 of the Venice classification.Pulmonary hypertension associated with moderate to severe obstructive lung disease: forced expiratory volume in one second/forced vital capacity (FEV1/FVC) < 70% and FEV1 < 65% of predicted value after bronchodilator administration.Pulmonary hypertension associated with moderate to severe restrictive lung disease: total lung capacity (TLC) < 60% of predicted value.Moderate to severe hepatic impairment, i.e., Child-Pugh Class B or C.Aspartate aminotransferase (AST) and/or alanine aminotransferase (ALT) > 1.5 times the upper limit of normal.Hemoglobin < 75% of the lower limit of the normal range.Systolic blood pressure < 100 mmHg.Acute or chronic physical impairment (other than dyspnea), limiting the ability to comply with study requirements.Pregnant or breast-feeding.Known concomitant life-threatening disease with a lifeexpectancy < 12 months.Body weight < 40 kg.Any condition that prevents compliance with the protocol or adherence to therapy.Recently started (< 8 weeks prior to randomization) or planned cardio-pulmonary rehabilitation program based on exercise.Treatment with endothelin receptor antagonists (ERAs) within 3 months prior to randomization.Systemic treatment within 1 week prior to randomization with cyclosporine A or tacrolimus, everolimus, sirolimus (calcineurin or mTOR inhibitors).Known hypersensitivity to drugs of the same class as the study drug, or any of their excipients.Planned treatment, or treatment, with another investigational drug within 1 month prior to randomization.

Study & Design

Study Type
Interventional clinical trial of medicinal product
Study Design
Not specified
Primary Outcome Measures
NameTimeMethod
Main Objective: To demonstrate that ACT-064992 prolongs the time to thefirst morbidity or mortality event in patients with symptomatic pulmonary arterial hypertension.;Secondary Objective: To demonstrate the effect of ACT-064992 on exercisecapacity in patients with symptomatic pulmonary arterialhypertension.To evaluate the safety and tolerability of ACT-064992 inpatients with symptomatic pulmonary arterial hypertension.;Primary end point(s): Time from baseline to first morbidity or mortality event, defined as follows:1. Death, or2. Atrial septostomy, or3. Lung transplantation, or4. Initiation of intravenous or subcutaneous prostanoids (e.g., epoprostenol, treprostinil), or5. Treatment emergent worsening of pulmonary arterialhypertension
Secondary Outcome Measures
NameTimeMethod
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