Pulmonary Arterial Hypertension Clinical Trial
Official title:
Evaluation of FDG Uptake and Pulmonary Perfusion by Positron Emission Tomography in Patients With Pulmonary Arterial Hypertension Before and After Treatment With Ambrisentan
The investigators are doing this research study to find out how blood flow changes in the lungs of people with pulmonary hypertension before and after treatment with ambrisentan (sold under the brand name Letairis). The investigators hope that knowing about these differences will help us to better understand pulmonary hypertension and find new ways to diagnose it earlier.
The overall goal of this study is to evaluate lung, large vessel, and right ventricular
glucose uptake rate using 18FDG PET and distribution of pulmonary perfusion using 13NN PET
in patients with pulmonary hypertension before and after treatment with ambrisentan. The
investigators propose a pilot study of patients with Group I PAH. The investigators plan to
complete this pilot study with 5 patients (projected enrollment of 8 to allow for drop-out
rate). Taken together, the studies above demonstrate that FDG-PET can be used to detect
changes associated with PAH, and to monitor response to therapy. FDG-PET is a widely
available technology, and is firmly established in the field of clinical oncology.
Ambrisentan is an endothelin receptor antagonist that is FDA-approved to treat patients with
pulmonary arterial hypertension. Patients will be eligible for participation in this study
if they have already made a decision to start treatment with ambrisentan for pulmonary
hypertension. The investigators propose a physiologic study of patients who will already
have made a decision to start ambrisentan therapy. Participation in the study will not alter
or affect participants decisions regarding treatment.
Prior human studies evaluating response to PH-specific therapies focused only on the right
ventricle. Oikawa et al. looked at the RV uptake in patients with PH and evaluated them
before and after treatment with epoprostenol. Fang and colleagues used FDG-SPECT evaluate
FDG uptake in the RV of patients with IPAH and PH before and after treatment with
sildenafil. Both of those studies used SUV (specific uptake variable). The investigators
propose to focus on the lung and the RV uptake rate (Ki) in order to provide a more robust
analysis of the metabolic changes that underlie the development of pulmonary hypertension
both in the right ventricle and the lung parenchyma, and to study the response to
ambrisentan with FDG-PET.
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Intervention Model: Single Group Assignment, Masking: Open Label
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