Eligibility NCT00586794 Pulmonary Arterial Hypertension (PAH) Inclusion Criteria Exclusion Criteria pregnancy or lactation Yes
No
women of child-bearing age who are sexually active without practising highly effective methods of contraception Yes
No
any diseases or impairment that, in the opinion of the investigator exclude a subject from participation Yes
No
substance abuse (alcohol, medicines, drugs) Yes
No
other medical, psychological or social circumstances that would adversely affect a patient's ability to participate reliably in the study or increase the risk to themselves or others if they participated Yes
No
insufficient compliance Yes
No
missing willingness to storaging and transferring pseudonymous disease data within this study. Yes
No
subjects who are not able to perform Cardio-Pulmonary Exercise Testing (CPX). Yes
No
pulmonary hypertension secondary to any etiology other than those specified in the inclusion criteria Yes
No
subjects with known intolerance of NO and iloprost or their constituents Yes
No
acute decompensated heart failure within the 7 days before the invasive diagnostic procedure Yes
No
clinically significant haemoptysis within the last 6 months Yes
No
hemodynamic instability which would represent an unjustifiable risk during testing of pulmonary arterial vasoreagibility Yes
No
arterial hypotension (as defined by age-specific values) Yes
No
Anaemia Yes
No
decompensated symptomatic policythemia; (details: 4.2.2. exclusion criteria) Yes
No
Thrombocytopenia Yes
No
secondary impairment of organic function: Yes
No
impairment of renal function (GFR < 30 ml/min/1,73 m2 BSA) Yes
No
impairment of hepatic function (ALT and/or AST > 3 x ULN and bilirubin ≥ 2 mg/dl) Yes
No
other sources of pulmonary blood flow which prohibit measurement of the blood flow into the lungs and therefore of the pulmonary vascular resistance: Yes
No
Glenn shunt, Anastomosis of superior vena cava to pulmonary artery Yes
No
BT - Blalock-Taussig shunt, Subclavian to pulmonary artery shunt operation Yes
No
significant number of MAPCAs; (details: 4.2.2. exclusion criteria) Yes
No
Obstruction of pulmonary outflow tract Yes
No
obstruction of pulmonary venous return Yes
No
mitral valve dysfunction Yes
No
Left heart diseases: Yes
No
aortic or mitral valve disease (more severe than "mild") Yes
No
restrictive or congestive cardiomyopathy Yes
No
PCWP/LVEDP > 15 mmHg Yes
No
symptomatic coronary artery disease Yes
No
Significant valvular diseases other than tricuspid or pulmonary regurgitation (these are not exclusion criteria; details: 4.2.2. exclusion criteria). Yes
No
PERICARDIAL CONSTRICTION Yes
No
History of stroke, myocardial infarction or life-threatening arrhythmia within the 6 months before screening Yes
No
Bronchopulmonary dysplasia (BPD) and other chronic lung diseases Yes
No
History of significant pulmonary embolism Yes
No
Other relevant diseases (e.g. HIV, diabetes mellitus requiring medical treatment) Yes
No
Subjects with trisomy 21 (reproducibility of 6-MWT and CPX doubtful; communication as to side effects and subjective quality of life doubtful) Yes
No
all contraindications against the study medication (see also "4.2.3 concomitant medication") Yes
No
hypersensitivity against the active ingredients as well as supplementaries Yes
No
patients who lost vision on one eye due to a non arteriitic anterior ischaemic neuropathy of the opticus (NAION). Yes
No
Prohibited concomitant medication: Yes
No
Any medication listed below which has not been discontinued at least 30 days prior to screening. Specific pulmonary vasodilators during cardiac catheterization are allowed. Yes
No
Unspecified concomitant medication Yes
No
Other significant medication (a.o. chronic intake of systemic immunosuppression as e.g. systemic glucocorticoids, cytostatic drugs, ciclosporin) Yes
No
Instable medication (details: 4.2.5 prohibited concomitant medication): Yes
No
begin of a new medication regimen within the last 30 days before screening Yes
No
change in the dosage of existing medication within the last 7 days before cardiac catheterization Yes
No
Existing anti-pulmonary hypertensive medication (in any form) with: Yes
No
PDE-5 antagonists (e.g. sildenafil) Yes
No
prostanoids (e.g. iloprost, prostacyclin, beraprost) In case the patient is stable and on Bosentan therapy at least for 6 months. Bosentan (Tracleer) is unprohibited as concomitant medication. Yes
No
Other medication with vascular action: Yes
No
Adrenergic alpha-Antagonists Yes
No
L-arginin (acts through NO axis) Yes
No
ritonavir, nicorandil (act through K+ channels) Yes
No
Medication that is not compatible with sildenafil or interferes with the metabolism: Yes
No
cytochrome P450-CYP2C9 and CYP3A4 inhibitors (e.g. erythromycin/ketoconazole/itraconazole/protease inhibitors) Yes
No
any existing medication that, in the opinion of the investigator, may interfere with sildenafil treatment. Yes
No
