Cardiomyopathy, Diastolic Function and CoQ10 – Interview with Giv Heidari-Bateni, MD/MPH

Interview with Giv Heidari-Bateni, MD/MPH
Post-doctoral Research Associate
Washington University in St. Louis
BJC Institute of Health
425 S Euclid Ave., Rm. 7122
St. Louis, MO 63110
(314) 299-1984 / (314) 454-2381 (FAX)
givhb@yahoo.com

Cardiology in the Young

“Coenzyme Q10 Improves Diastolic Function in Children With Idiopathic Dilated Cardiomyopathy,”
Cardiol Young, 2009 Aug 25; 1-6 [Epub ahead of print]. 47408 5/2014

Kirk Hamilton: Can you please share with us your educational background and current position?

Giv Heidari-Bateni: I graduated in Medicine/Master of Public Health dual degree from Tehran
Medical University and I am currently completing a postdoctoral research scholarship in congenital heart
diseases at Washington University in St. Louis. I was doing research in pediatric cardiology at Children’s
Hospital in Tehran before I started my post doctorate training here.

KH: What got you interested in studying the role of coenzyme Q10 (CoQ10) in young cases of
idiopathic dilated cardiomyopathy (IDC)?

GHB: IDC is an unknown disease. Both its etiology and the optimal management of the disease are
controversial. Coenzyme Q10 is a major component of internal membrane of mitochondrion which plays a
crucial role in energy production within the cells. There are several studies which have shown potential benefits
for supplementation of coenzyme Q10 to the medical regimen of adults with heart failure. For the pediatric
population, however, there is lack of data on this topic. The main rationale for this lack of data is the paucity of
research in this field compared to that of adult population. Considering high rates of treatment failure and need
for early cardiac transplantation in this population, optimizing the management for pediatric patients with IDC
is mandatory. I should add that adult data is not necessarily applicable to their pediatric counterparts. For this
reason we decided to conduct a randomized controlled trial to assess the role of coenzyme Q10 in IDC in
children. To the best of my knowledge, there has been no published randomized controlled trial for
supplementation of coenzyme Q10 in pediatric patients since the time we started our study and published it
later. Based on a recent review from Cochrane in late 2013, our study is the only RCT for this topic in children.

KH: What is the biochemistry of CoQ10 that might help correct the pathophysiology of IDC?

GHB: As I mentioned earlier, coenzyme Q10 is a component of inner membrane of mitochondrion.
Needless to say, the mitochondrion is an organelle within nearly every cell in the body. Coenzyme Q10 is a part
of the electron transport chain and is involved in production of ATP within the cells; ATP is the source of energy
for the tissue. The higher the work load of a tissue the more the need for coenzyme Q10. High concentrations of
coenzyme Q10 are found in the mitochondria of cardiac cells as a tissue with high-energy requirements. It is
conceivable that deficiency in the amount of coenzyme Q10 in cardiac tissue would result in energy depletion
and a progression toward decompensation of the ongoing heart failure. Coenzyme Q10 has also been shown to
play a role as an endogenous anti-oxidant; therefore, it can potentially reduce the burden of oxidative stress in
the failing heart. The exact mechanism of usefulness of coenzyme Q10 supplementation in IDC is not yet
understood. Supplementation of coenzyme Q10, however, could presumably compensate for the lack of this
molecule in the heart.KH: Were CoQ10 levels measured before, during or after supplementation with CoQ10? Were
these subjects low in CoQ10 prior to the intervention?

GHB: Several studies have shown a decline in plasma levels of coenzyme Q10 in heart failure.
Medications used for heart failure could also have a side effect of lowering the plasma levels of coenzyme Q10.
Since this concept has been previously shown in literature, we did not measure the plasma level of coenzyme
Q10 either before the administration or after that. Although animal studies have shown uptake of coenzyme
Q10 by the heart after supplementation, there are still controversies on the possible effect of this
supplementation on tissue concentrations of the molecule. We did not measure the tissue concentration of
coenzyme Q10 in our research as it warranted tissue sampling which was unnecessarily invasive for patients.

KH: What dose of CoQ10 was used in these subjects? Was it given in a single dose or divided
dose? With meals or away from meals?

GHB: We started with 2 milligram/kilogram/day in 2 or 3 divided doses which further increased to the
maximum dose of 10 milligram/kilogram/day according to tolerance or the appearance of side effects. For most
of the patients, it was given twice daily with a time interval from the meal unless evidence of gastrointestinal
distress appeared which mandated supplementation with food.

KH: For the sake of comparison if you were using this same dose range of CoQ10 on a 154 lb (70 kg) adult
the low end would be 140 mg/d and the maximum would be 700 mg/d. Correct?

GHB: Yes. That’s correct.

KH: Was it a special type of CoQ10 (ubiquinone or ubiquinol)? Was it highly bioavailable?

GHB: We used ubiquinone. There are some nice articles which have assessed bioavailability of
different types of coenzyme Q10 formulations. Overall, bioavailability of ubiquinol is higher than ubiquinone,
and an oil-based formulation has higher bioavailability.

KH: Can you tell us about your study and the basic results?

GHB: We randomized 38 patients younger than 18 years with IDC to receive either coenzyme Q10,
chosen for 17 patients, or placebo, administered in the remaining 21. Patients were receiving their routine
management for IDC. We compared the indexes of heart failure in both groups six months after
supplementation of coenzyme Q10 and found that those who received coenzyme Q10 showed evidences of
improvement of the performance of myocardium particularly diastolic function of the heart compared to the
control group. This result suggested that hearts of the patients who received coenzyme Q10 for six months were
less stiff and had better capacitance than those who did not receive it.

KH: Were there any side effects with the CoQ10 therapy? How was the patient compliance?

GHB: There were no side effects and patients’ compliance was great. All patients completed the trial.

KH: Who is a candidate for CoQ10 therapy? All patients with cardiomyopathy? Who would you recommend
CoQ10 therapy to?

GHB: In our experience, we used coenzyme Q10 in children with IDC with the New York University
Pediatric Heart Failure Index of at least class 2 in whom medications were stable without hemodynamic
instability. Although it is conceivable that coenzyme Q10 could not be harmful for other types of heart failure in
pediatric population, caution should always be considered when interpreting the results of RCTs. Undoubtedly,
further studies are needed to evaluate usefulness of coenzyme Q10 in other settings. KH: How would you use the findings of this study clinically?

GHB: Currently in Children’s Hospital in Tehran, based on the result of this RCT, coenzyme Q10 is
being administered for children with IDC. In the current era, results of randomized trials work as a guide for
clinicians in order to find the optimal managements for particular diseases; RCTs can be useful at the time of
decision making and treatment selection. It is necessary, however, to consider that treatment plans should
always be individualized for every patient.

KH: Do you have any further comments on this very interesting subject?

GHB: Since currently there is not much data on supplementation of coenzyme Q10 in children, ours
was the only RTC which has assessed its role in IDC. Although the results are convincing so far, further studies
are needed to fully address the question. More investigations are also warranted to reveal the molecular
mechanism behind this functional improvement.

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