Coenzyme Q-10 and Pulmonary Arterial Hypertension

Abnormalities in the blood vessels in the lung are the hallmark of pulmonary hypertension.
Links between increased free radical production, mitochondrial dysfunction and pulmonary
hypertension have been studied but are poorly understood. The mitochondria of cells is the
location where cellular energy is created and free radicals are atoms or groups of atoms
with an odd (unpaired) number of electrons and can be formed when oxygen interacts with
certain molecules. Once formed these reactive radicals can start a chain reaction, like
dominoes. Their chief danger comes from the damage they can do when they react with
important cellular components. Cells may function poorly or die if this occurs. The body
produces free radicals in the normal course of energy production and in pulmonary
hypertension, free radical production is found to be increased. To prevent free radical
damage the body has a defense system of antioxidants. Coenzyme Q-10 is an antioxidant and
it helps to protect cells from damage caused by the body's own free radicals. By providing
oral supplementation of coenzyme Q-10, free radical levels will be decreased and cellular
functioning in the pulmonary blood vessels may improve and even return to near normal
functioning.

The purpose of this study is to evaluate the effects of coenzyme Q-10, an antioxidant, in
the treatment of pulmonary hypertension. We will assess coenzyme Q-10 supplementation in
the treatment of pulmonary hypertension by clinical measurements and blood levels of certain
cellular components. We would like to assess the effects of coenzyme Q-10 on the pulmonary
vessels by measuring the lung diffusing capacity (a breathing test) and exhaled Nitric Oxide
(NO) (a substance in the body that relaxes or dilates blood vessels). We will also measure
endothelial progenitor cells (cells from the bone marrow) from a blood sample; these cells
are markers of measure of blood vessel formation and repair. We will also measure the
activity of superoxide dismutase (a protein in cells that executes the breakdown of a free
radical into oxygen and hydrogen peroxide) in the blood. In addition, we will measure
levels of coenzyme Q-10 in the blood. Other markers of disease response to therapy will be
done including physical exam, BNP level (a blood marker that correlates with heart
function), 6-minute walk and echocardiography (ultrasound of the heart). A total of 60ml (5
tablespoons) of blood will be drawn at each visit.

Inclusion Criteria:

- Males and Females age equal to or greater than 18 not to exceed 65.

- Patients with PAH Class 1 (Venice 2003)

- PAH medications must not have changed for the last two months.

- Women of child-bearing age must use a double-barrier local contraception until
completion of study.

- Subjects must demonstrate understanding of study and sign informed consent and have a
reliable method of communication for contact and the ability to comply with the study
requirements.

Exclusion Criteria:

- Participation in any other studies at the time of enrollment

- History of any significant illness within four weeks of starting Coenzyme Q-10

- Hepatic insufficiency (transaminase levels >4 fold the upper limit of normal or
bilirubin >2 fold the upper limit of normal).

- Renal insufficiency (creatinine >2)

- Pregnancy,breast-feeding or lack of safe contraception.

- Acute heart failure

- Known allergy to the study drug or drugs similar to the study drug

- History of drug or alcohol abuse within last 12 months