Idiopathic Pulmonary Arterial Hypertension Clinical Trial
Official title:
Acute Effects of Benzbromaron on the Pulmonary Circulation
Actual studies suggest that a calcium activated chlorid channel (TMEM16A) may play a
relevant role in the pathogenesis of pulmonary arterial hypertension (PAH). The inhibition
of this channel led to pulmonary vasorelaxation in preclinical studies. Benzbromarone is a
well known inhibitor of the TMEM16A channel and is used in patients with gout.
In this pilot study the investigators plan to investigate if Benzbromarone has an acute
effect on the pulmonary arteries in humans. This will be investigated within the frame of a
right heart catheterization performed in patients with known PAH due to clinical reasons.
The investigators hypothesize that the application of Benzbromarone leads to pulmonary
vasodilation, which can be recognized by the decrease in pulmonary vascular resistance. In
addition, the change in pulmonary and systemic arterial pressure, pulmonary arterial wedge
pressure, heart rate and arterial oxygen saturation will be assessed. Due to clinical
reasons patients will receive NO (15 ppm) during right heart catheterization. Hemodynamic
changes upon NO and Benzbromarone may be compared.
n/a
Endpoint Classification: Safety/Efficacy Study, Intervention Model: Single Group Assignment, Masking: Open Label, Primary Purpose: Treatment
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