Pulmonary artery hypertension (PAH) is a chronic and progressive disease that affects 15 persons per million. Although current therapy has improve disease prognosis, PAH still has a poor survival, with a median survival of 2.8 years after diagnosis. In the last few years new key elements in PAH pathogenesis have been discovered, such as the role of metabolism in disease onset and progression. In fact, PAH pulmonary smooth muscle cells switch into a glycolytic phenotype which resembles the metabolism of cancer cells. The investigators hypothesis is that "fatty acid oxidation inhibition reverts the PAH adverse phenotype by restoring mitochondrial function and morphology, decreasing proliferation and restoring apoptosis susceptibility in pulmonary smooth muscle cells "
Study Type
INTERVENTIONAL
Allocation
RANDOMIZED
Purpose
TREATMENT
Masking
QUADRUPLE
Enrollment
25
Hospital Clinico Pontificia Universidad Catolica de Chile
Santiago, Santiago Metropolitan, Chile
RECRUITINGChanges in right ventricular (RV) function
Changes in RV function assessed by echo 3d (strain-strain rate)
Time frame: 3 months
Changes in exercise capacity
Changes in exercise capacity assessed by 6 minute walk test
Time frame: 3 months
Changes in symptoms
Changes in Borg dyspnea index
Time frame: 3 months
Changes in biomarkers
Changes in B-type natriuretic peptide, galectin-3 and rho-kinase activity
Time frame: 3 months
Time to clinical worsening
Time to first PAH related medical event (ER evaluation, hospitalization or death)
Time frame: 3 months
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