VasoREACTivity Testing With Intravenous SILdenafil in Patients With Precapillary Pulmonary Hypertension

Vasoreactivity Testing With Intravenous Sildenafil in Patients With Precapillary Pulmonary Hypertension (Treatment Optimisation Study)

Sildenafil is a selective pulmonary vasodilator; in patients with a special kind of pulmonary hypertension it is approved for treatment. The trial seeks to find out, whether the acute response to this treatment (= vasoreactivity testing) given intravenously is effective and allows prediction of therapy success during a following oral treatment.

Inclusion Criteria:

• Precapillary pulmonary hypertension associated with connective tissue disease
• resting mean pressure in the pulmonary artery of > 24 mmHg
• resting mean pulmonary capillary wedge pressure (PCWP) of < 16 mmHg
• age 18 to 80 years
• women of childbearing potential must have a negative pregnancy test (ß-HCG in urine) and must use effective methods of contraception
• women must not be breastfeeding
• ability to understand and sign the informed consent, correctly signed informed consent

Exclusion Criteria:

• pretreatment with Sildenafil

• contraindications for Sildenafil treatment:

  • known intolerance to Sildenafil,
  • optic neuropathy (NAION),
  • known hereditary retina disease,
  • need of nitrate therapy

• advanced liver cirrhosis - CHILD C

• severely reduced renal function with GFR < 30 ml/min/1,73 m²

• stroke or myocardial infarction within the last 6 months