Page last updated: 2024-10-28

idebenone and Cardiomyopathy, Hypertrophic

idebenone has been researched along with Cardiomyopathy, Hypertrophic in 6 studies

Cardiomyopathy, Hypertrophic: A form of CARDIAC MUSCLE disease, characterized by left and/or right ventricular hypertrophy (HYPERTROPHY, LEFT VENTRICULAR; HYPERTROPHY, RIGHT VENTRICULAR), frequent asymmetrical involvement of the HEART SEPTUM, and normal or reduced left ventricular volume. Risk factors include HYPERTENSION; AORTIC STENOSIS; and gene MUTATION; (FAMILIAL HYPERTROPHIC CARDIOMYOPATHY).

Research Excerpts

ExcerptRelevanceReference
"This paper reviews the history and pre-clinical development of idebenone and summarises the results of clinical studies, published from 1999 to 2008, on the use of idebenone in the treatment of patients with Friedreich ataxia (FRDA)."8.85Idebenone: an emerging therapy for Friedreich ataxia. ( Buyse, G; Meier, T, 2009)
"Severe hypertrophic cardiomyopathy in a 15 years old child with Friedreich ataxia was treated with idebenone on the basis of a preliminary study reported in the literature."7.72[Successful idebenone therapy of hypertrophic cardiomyopathy in Friedreich ataxia]. ( Kádár, K, 2003)
"The authors carried out a 1-year, randomized, placebo-controlled trial of idebenone in 29 patients with Friedreich ataxia."5.10Idebenone treatment in Friedreich patients: one-year-long randomized placebo-controlled trial. ( Di Donato, S; Fiorentini, C; Marano, L; Mariotti, C; Solari, A; Torta, D, 2003)
"This paper reviews the history and pre-clinical development of idebenone and summarises the results of clinical studies, published from 1999 to 2008, on the use of idebenone in the treatment of patients with Friedreich ataxia (FRDA)."4.85Idebenone: an emerging therapy for Friedreich ataxia. ( Buyse, G; Meier, T, 2009)
"Severe hypertrophic cardiomyopathy in a 15 years old child with Friedreich ataxia was treated with idebenone on the basis of a preliminary study reported in the literature."3.72[Successful idebenone therapy of hypertrophic cardiomyopathy in Friedreich ataxia]. ( Kádár, K, 2003)

Research

Studies (6)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's5 (83.33)29.6817
2010's1 (16.67)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Meier, T2
Buyse, G2
Lagedrost, SJ1
Sutton, MS1
Cohen, MS1
Satou, GM1
Kaufman, BD1
Perlman, SL1
Rummey, C1
Lynch, DR1
Mariotti, C1
Solari, A1
Torta, D1
Marano, L1
Fiorentini, C1
Di Donato, S1
Mertens, L1
Di Salvo, G1
Matthijs, I1
Weidemann, F1
Eyskens, B1
Goossens, W1
Goemans, N1
Sutherland, GR1
Van Hove, JL1
Kádár, K1
Rustin, P1
Rötig, A1
Munnich, A1
Sidi, D1

Clinical Trials (1)

Trial Overview

TrialPhaseEnrollmentStudy TypeStart DateStatus
A Phase III Double-Blind, Randomized, Placebo-Controlled Study of the Efficacy, Safety and Tolerability of Idebenone in the Treatment of Friedreich's Ataxia Patients[NCT00537680]Phase 370 participants (Actual)Interventional2007-12-31Completed
[information is prepared from clinicaltrials.gov, extracted Sep-2024]

Trial Outcomes

Absolute Change From Baseline to Week 24 in Left Ventricular Mass Index (LVMI)

To compare the effects of idebenone on cardiac anatomy and function in patients with Friedreich's ataxia (FRDA) as assessed by echocardiography (NCT00537680)
Timeframe: baseline and 6 months

Interventiong/m2 (Mean)
Mid Dose Idebenone-3.06
High Dose Idebenone-2.56
Placebo-1.26

Absolute Change From Baseline to Week 24 in the Relative Wall Thickness (RWT) of the Left Ventricle

To compare the effects of idebenone on cardiac anatomy and function in patients with Friedreich's ataxia (FRDA) as assessed by echocardiography (NCT00537680)
Timeframe: baseline and 6 months

Interventioncm (Mean)
Mid Dose Idebenone-0.032
High Dose Idebenone0.007
Placebo0.010

Absolute Change From Baseline to Week 24 in the Thickness of the Interventricular Septum (IVS)

To compare the effects of idebenone on cardiac anatomy and function in patients with Friedreich's ataxia (FRDA) as assessed by echocardiography (NCT00537680)
Timeframe: baseline and 6 months

Interventioncm (Mean)
Mid Dose Idebenone-0.014
High Dose Idebenone0.021
Placebo0.063

Absolute Change From Baseline to Week 24 in the Thickness of the Posterior Wall (PW) of the Left Ventricle

To compare the effects of idebenone on cardiac anatomy and function in patients with Friedreich's ataxia (FRDA) as assessed by echocardiography (NCT00537680)
Timeframe: baseline and 6 months

Interventioncm (Mean)
Mid Dose Idebenone-0.018
High Dose Idebenone-0.004
Placebo-0.038

Activities of Daily Living (ADL) of Friedreich's Ataxia Rating Scale (FARS), Change in ADL (Total Score ) From Baseline to Week 24

"To compare the efficacy of 6 months' treatment with 2 different doses of idebenone with that of placebo on Activities of Daily Living (ADL) assessed by the ADL scale of the Friedreich's Ataxia Rating Scale (FARS)~score 0 to 36, higher score indicates greater impairment" (NCT00537680)
Timeframe: baseline and 6 months

InterventionADL points (Mean)
Mid Dose Idebenone0.2
High Dose Idebenone0.6
Placebo1.0

Change in Friedreich's Ataxia Rating Scale (FARS) Scores From Baseline to Week 24

"To compare the efficacy of 6 months' treatment with 2 different doses of idebenone with that of placebo on neurological function as assessed by the Friedreich's Ataxia Rating Scale (FARS)~FARS consists of a 25 manoeuvre exam along with 4 quantitative performance measures. The neurological exam covers bulbar function, upper limb coordination, lower limb coordination, peripheral nervous system function, deep tendon reflexes, stability and gait. The use of FARS has been recently validated as a sensitive scale for this population.~The FARS exam scores can be added to make a total score ranging from 0 to 159. A higher score indicates a greater level of disability.~Calculation details FARS score at week 24 minus FARS score baseline" (NCT00537680)
Timeframe: baseline and 6 months

InterventionFARS score (Mean)
Mid Dose Idebenone-1.6
High Dose Idebenone-1.2
Placebo0.6

Change in the International Cooperative Ataxia Rating Scale (ICARS) From Baseline Assessment to Week 12

"To compare the efficacy of 3 months' treatment with 2 different doses of idebenone with that of placebo on neurological impairment as assessed by the International Cooperative Ataxia Rating Scale (ICARS)~International Cooperative Ataxia Rating Scale (ICARS):~ICARS consists of a one-hundred-point semi-quantitative scale based upon 19 simple testing manoeuvres compartmentalized into postural and stance disorders, limb ataxia, dysarthria and oculomotor disorders and has been previously used in this patient population with good inter-rater reliability.~Minimum score: 0, maximum score 100, higher score indicates greater impairment~Calculation details: ICARS score at week 12 minus ICARS score baseline" (NCT00537680)
Timeframe: baseline and 12 weeks

InterventionICARS points (Mean)
Mid Dose Idebenone-1.5
High Dose Idebenone-1.6
Placebo-3.0

Change in the International Cooperative Ataxia Rating Scale (ICARS) From Baseline Assessment to Week 24

"International Cooperative Ataxia Rating Scale (ICARS):~ICARS consists of a one-hundred-point semi-quantitative scale based upon 19 simple testing manoeuvres compartmentalized into postural and stance disorders, limb ataxia, dysarthria and oculomotor disorders and has been previously used in this patient population with good inter-rater reliability.~Minimum score: 0, maximum score 100, higher score indicates greater impairment~Calculation details: ICARS score at week 24 minus ICARS score baseline" (NCT00537680)
Timeframe: baseline and 6 months

InterventionICARS points (Mean)
Mid Dose Idebenone-2.5
High Dose Idebenone-2.4
Placebo-1.3

Percent Change From Baseline to Week 24 in Left Ventricular Mass Index

To compare the effects of idebenone on cardiac anatomy and function in patients with Friedreich's ataxia (FRDA) as assessed by echocardiography (NCT00537680)
Timeframe: baseline and 6 months

Intervention% change (Mean)
Mid Dose Idebenone-4.5
High Dose Idebenone-4.9
Placebo-1.7

Percent Change From Baseline to Week 24 in the Relative Wall Thickness (RWT) of the Left Ventricle

To compare the effects of idebenone on cardiac anatomy and function in patients with Friedreich's ataxia (FRDA) as assessed by echocardiography (NCT00537680)
Timeframe: baseline and 6 months

Intervention% change (Mean)
Mid Dose Idebenone-3.32
High Dose Idebenone1.53
Placebo2.65

Percent Change From Baseline to Week 24 in the Thickness of the Interventricular Septum (IVS)

To compare the effects of idebenone on cardiac anatomy and function in patients with Friedreich's ataxia (FRDA) as assessed by echocardiography (NCT00537680)
Timeframe: baseline and 24 weeks

Intervention% change (Mean)
Mid Dose Idebenone1.3
High Dose Idebenone2.6
Placebo7.8

Percent Change From Baseline to Week 24 in the Thickness of the Posterior Wall (PW) of the Left Ventricle

To compare the effects of idebenone on cardiac anatomy and function in patients with Friedreich's ataxia (FRDA) as assessed by echocardiography (NCT00537680)
Timeframe: baseline and 6 months

Intervention% change (Mean)
Mid Dose Idebenone-0.075
High Dose Idebenone0.12
Placebo-3.04

Reviews

1 review available for idebenone and Cardiomyopathy, Hypertrophic

ArticleYear
Idebenone: an emerging therapy for Friedreich ataxia.
    Journal of neurology, 2009, Volume: 256 Suppl 1

    Topics: Animals; Antioxidants; Cardiomyopathy, Hypertrophic; Friedreich Ataxia; Humans; Nervous System Disea

2009

Trials

3 trials available for idebenone and Cardiomyopathy, Hypertrophic

ArticleYear
Idebenone in Friedreich ataxia cardiomyopathy-results from a 6-month phase III study (IONIA).
    American heart journal, 2011, Volume: 161, Issue:3

    Topics: Adolescent; Antioxidants; Cardiomyopathy, Hypertrophic; Child; Clinical Trials, Phase III as Topic;

2011
Idebenone treatment in Friedreich patients: one-year-long randomized placebo-controlled trial.
    Neurology, 2003, May-27, Volume: 60, Issue:10

    Topics: Adolescent; Adult; Benzoquinones; Cardiomyopathy, Hypertrophic; Child; Erythrocytes; Female; Frataxi

2003
Idebenone treatment in Friedreich's ataxia: neurological, cardiac, and biochemical monitoring.
    Neurology, 2003, May-27, Volume: 60, Issue:10

    Topics: Adolescent; Adult; Benzoquinones; Cardiomyopathy, Hypertrophic; Child; Erythrocytes; Female; Frataxi

2003

Other Studies

2 other studies available for idebenone and Cardiomyopathy, Hypertrophic

ArticleYear
[Successful idebenone therapy of hypertrophic cardiomyopathy in Friedreich ataxia].
    Orvosi hetilap, 2003, Jun-22, Volume: 144, Issue:25

    Topics: Adolescent; Antioxidants; Benzoquinones; Cardiomyopathy, Hypertrophic; Child; Echocardiography; Elec

2003
Heart hypertrophy and function are improved by idebenone in Friedreich's ataxia.
    Free radical research, 2002, Volume: 36, Issue:4

    Topics: Administration, Oral; Adolescent; Adult; Antioxidants; Benzoquinones; Cardiomyopathy, Hypertrophic;

2002