ArticleInternational journal of cardiology2013
Defining cardiac adaptations and safety of endurance training in patients with m.3243A>G-related mitochondrial disease.
Article in International journal of cardiology, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 28 papers, 1 of them a synthesis that pooled it.
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The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
28 citing papers in PubMed, 1 synthesis or guideline pooled it, 51 citations in OpenAlex.
- Quantitative assessment of efficacy and evaluation of feasibility of exercise training protocols in adult and pediatric primary mitochondrial disease.BMC medicine · 2026Pooled it
- Context-specific hypermetabolism in humans with mitochondrial defects.Cell press blue · 2026Article
- Effects of exercise training on skeletal muscle function in patients with mitochondrial myopathy: a systematic review.Frontiers in sports and active living · 2026Review
- Mitochondrial Mutation Leads to Cardiomyocyte Hypertrophy by Disruption of Mitochondria-Associated ER Membrane.Cell proliferation · 2025Article
- Advances in Management of Mitochondrial Myopathies.International journal of molecular sciences · 2025Review
- Outcomes misaligned in mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS): implications for trial design.Brain communications · 2025Article
- OxPhos defects cause hypermetabolism and reduce lifespan in cells and in patients with mitochondrial diseases.Communications biology · 2023Article
- Article
- The ergoreflex: how the skeletal muscle modulates ventilation and cardiovascular function in health and disease.European journal of heart failure · 2021Review
- The long and winding road: perspectives of people and parents of children with mitochondrial conditions negotiating management after diagnosis.Orphanet journal of rare diseases · 2021Article
- Exploring the Ability of LARS2 Carboxy-Terminal Domain in Rescuing the MELAS Phenotype.Life (Basel, Switzerland) · 2021Article
- Metabolomics and cardiovascular imaging: a combined approach for cardiovascular ageing.ESC heart failure · 2021Review
- Current and Emerging Clinical Treatment in Mitochondrial Disease.Molecular diagnosis & therapy · 2021Review
- Optimal Estimate for Energy Requirements in Adult Patients With the m.3243A>G Mutation in Mitochondrial DNA.JPEN. Journal of parenteral and enteral nutrition · 2021Article
- Association of Body Composition, Physical Functioning, and Protein Intake in Adult Patients With Mitochondrial Diseases.JPEN. Journal of parenteral and enteral nutrition · 2021Article
- Current progress in the therapeutic options for mitochondrial disorders.Physiological research · 2020Article
- Exercise Training and Neurodegeneration in Mitochondrial Disorders: Insights From the Harlequin Mouse.Frontiers in physiology · 2020Article
- Physical Exercise and Mitochondrial Disease: Insights From a Mouse Model.Frontiers in neurology · 2019Article
- Diagnosis and Treatment of Mitochondrial Myopathies.Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics · 2018Review
- Monitoring clinical progression with mitochondrial disease biomarkers.Brain : a journal of neurology · 2017Review
Corrections and comments
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Authors and funding
18 authors at 7 institutions in 2 countries.
Funding
Abstract
backgroundCardiac hypertrophic remodelling and systolic dysfunction are common in patients with mitochondrial disease and independent predictors of morbidity and early mortality. Endurance exercise training improves symptoms and skeletal muscle function, yet cardiac adaptations are unknown. METHODS AND
resultsBefore and after 16-weeks of training, exercise capacity, cardiac magnetic resonance imaging and phosphorus-31 spectroscopy, disease burden, fatigue, quality of life, heart rate variability (HRV) and blood pressure variability (BPV) were assessed in 10 adult patients with m.3243A>G-related mitochondrial disease, and compared to age- and gender-matched sedentary control subjects. At baseline, patients had increased left ventricular mass index (LVMI, p<0.05) and LV mass to end-diastolic volume ratio, and decreased longitudinal shortening and myocardial phosphocreatine/adenosine triphosphate ratio (all p<0.01). Peak arterial-venous oxygen difference (p<0.05), oxygen uptake (VO2) and power were decreased in patients (both p<0.01) with no significant difference in cardiac power output. All patients remained stable and completed ≥80% sessions. With training, there were similar proportional increases in peak VO2, anaerobic threshold and work capacity in patients and controls. LVMI increased in both groups (p<0.01), with no significant effect on myocardial function or bioenergetics. Pre- and post-exercise training, HRV and BPV demonstrated increased low frequency and decreased high frequency components in patients compared to controls (all p<0.05).
conclusionPatients with mitochondrial disease and controls achieved similar proportional benefits of exercise training, without evidence of disease progression, or deleterious effects on cardiac function. Reduced exercise capacity is largely mediated through skeletal muscle dysfunction at baseline and sympathetic over-activation may be important in pathogenesis.
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Registered trials
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