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Ultrastructural abnormalities of mitochondria and deficiency of myocardial cytochromec oxidase in a patient with ventricular tachycardia

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Summary

A 30-year-old woman presented with lifethreatening ventricular tachycardia without overt heart disease. Ultrastructural investigation of endomyocardial biopsy disclosed abnormally structured and often enlarged mitochondria. Morphometry revealed the ratio of volume density of mitochondria to myofibrils to be markedly increased to 0.667 as compared with five controls (mean: 0.46; range: 0.445–0.479). Investigation of mitochondrial respiratory chain enzymes revealed a 90% reduction in activity of cytochromec oxidase. Our data suggest that mitochondrial cardiomyopathy may induce malignant ventricular arrhythmias.

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References

  • Askanas V, Engel WK, Britton DE, Adornato BT, Eiben RN (1978) Reincarnation in cultural muscle of mitochondrial abnormalities. Two patients with epilepsy and lactic acidosis. Arch Neurol 35:801–809

    Google Scholar 

  • DiMauro S, Bonilla E, Zeviani M, Nakagawa M, DeVivo DC (1985) Mitochondrial myopathies. Ann Neurol 17:521–538

    Google Scholar 

  • DiMauro S, Zeviani M, Servidei M, Bonilla E, Miranda AF, Prelle A, Schon EA (1987) Cytochrome oxidase deficiency: clinical and biochemical heterogeneity. Ann NY Acad Sci 488:19–32

    Google Scholar 

  • DiMauro S, Bonilla E, Zeviani M, Rizzuto R, Lombes A, Nakase H, Miranda A, Schon E (1988) Molecular defects in cytochrome oxidase in mitochondrial diseases. J Bioenerg Biomembr 20:353–364

    Google Scholar 

  • Dunnigan A, Staley NA, Smith SA, Pierpont ME, Judd D, Benditt DG, Benson DW (1987) Cardiac skeletal muscle abnormalities in cardiomyopathy: comparison of patients with ventricular tachycardia or congestive heart failure. J Am Coll Cardiol 10:608–618

    Google Scholar 

  • Dyck PJ (1984) Inherited neuronal degeneration and atrophy affecting peripheral motor, sensory, and autonomic neurons. In: Dyck PJ, Thomas PK, Lambert EH, Bunge R (eds) Peripheral neuropathy, vol II. Saunders, Philadelphia, pp 1600–1655

    Google Scholar 

  • Grigg LE, Chan W, Mond HG, Vohra JK, Downey WF (1985) Ventricular tachycardia and sudden death in myotonic dystrophy: clinical, electrophysiologic and pathologic features. J Am Coll Cardiol 6:254–256

    Google Scholar 

  • Hatefi Y, Rieske JS (1967) The preparation and properties of DPNH-cytochrome c reductase (complex I–III) of the respiratory chain. Methods Enzymol 10:225–231

    Google Scholar 

  • Hatefi Y, Stigall JS (1978) Preparation and properties of succinate: ubichinon oxidoreductase (complex II). Methods Enzymol 53:21–27

    Google Scholar 

  • Hübner G, Grantzow R (1983) Mitochondrial cardiomyopathy with involvement of skeletal muscles. Virchows Arch [A] 399:115–125

    Google Scholar 

  • Hug G, Schubert WK (1970) Idiopathic cardiomyopathy. Mitochondrial and cytoplasmatic alterations in heart and liver. Lab Invest 22:541–552

    Google Scholar 

  • Kadenbach B, Jarausch J, Hartmann R, Merle P (1983) Separation of mammalian cytochrome c oxidase into 13 polypeptides by a sodium dodecyl sulfate-gel electrophoretic procedure. Ann Biochem 129:517–521

    Google Scholar 

  • Kadenbach B, Kuhn-Nentwig L, Büge U (1987) Evolution of a regulatory enzyme: cytochrome c oxidase (complex IV). Curr Top Bioenerg 15:113–161

    Google Scholar 

  • Langes K, Frenzel H, Seitz R, Kluitmann G (1985) Cardiomyopathy associated with Leigh's disease. Virchows Arch [A] 407:97–105

    Google Scholar 

  • Lascelles RG, Baker IA, Thomas PK (1970) Hereditary polyneuropathy of Roussy-Levy type with associated cardiomyopathy. Guy's Hosp Rep 119:253–256

    Google Scholar 

  • Lemery R, Brugada P, Bella PD, Dugernier T, Wellens HJJ (1989) Ventricular fibrillation in six adults without overt heart disease. J Am Coll Cardiol 13:911–916

    Google Scholar 

  • Lowry OH, Rosebrough NJ, Farr AL, Randall RJ (1951) Protein measurement with folin reagent. J Biol Chem 193:265–267

    Google Scholar 

  • Mall G, Schwarz F, Derks H (1982) Clinical pathologic correlations in congestive cardiomyopathy. Virchows Arch [A] 973:67–82

    Google Scholar 

  • Müller-Höcker J (1988) Morphologie, Cytochemie und Immunhistochemie des Cytochrom-c-Oxidase-Mangels. Verh Dtsch Ges Pathol 72:552–565

    Google Scholar 

  • Müller-Höcker J (1989) Cytochrome-c-oxidase deficient cardiomyocytes in the human heart — an age-related phenomenon. Am J Pathol 134:1167–1173

    Google Scholar 

  • Müller-Höcker J, Johannes A, Droste M, Kadenbach B, Hübner G (1986) Fatal mitochondrial cardiomyopathy in Kearns-Sayre syndrome with deficiency of cytochrome oxidase in the cardiac and skeletal muscle. An enzyme-histochemical-ultraimmunocytochemical structural study in the longterm frozen autopsy tissue. Virchows Arch [B] 52:353–367

    Google Scholar 

  • Neustein HB, Lurie PR, Dahms B, Takahashi M (1979) An x-linked recessive cardiomyopathy with abnormal mitochondria. Pediatrics 64:24–29

    Google Scholar 

  • Perloff JK (1972) Cardiac involvement in heredofamilial neuromyopathic diseases. Cardiovasc Clin 4:333–344

    Google Scholar 

  • Schaper J, Meiser E, Stämmler G (1985) Ultrastructural analysis of the myocardium from dogs, rats, hamsters, mice, and from human hearts. Circ Res 56:377–391

    Google Scholar 

  • Schwartzkopff B, Ühre B, Ehle B, Lösse B, Frenzel H (1987) Variability and reproducibility of morphologic findings in endomyocardial biopsies of patients with hypertrophic obstructive cardiomyopathy. Z Kardiol 76 [Suppl 3]: 26–32

    Google Scholar 

  • Schwartzkopff B, Frenzel H, Breithardt G, Deckert M, Lösse B, Toyka KV, Borggrefe M, Hort W (1988) Ultrastructural findings in endomyocardial biopsy of patients with Kearns-Sayre syndrome. J Am Coll Cardiol 12:1522–1528

    Google Scholar 

  • Segal BL, Iskandrian AS, Kotler MN (1985) Sudden cardiac death. In: Morganroth J, Horowitz LN (eds) Sudden cardiac death. Grune and Stratton, Orlando, pp 1–23

    Google Scholar 

  • Stumpf DA (1982) Friedreichs's disease: a metabolic cardiomyopathy. Am Heart J 104:887–888

    Google Scholar 

  • Tisdale HD (1967) Preparation and properties of succinic-cytochrome reductase (complex II–III). Methods Enzymol 10:213–215

    Google Scholar 

  • Urie PM, Billingham ME (1988) Ultrastructural features of familial cardiomyopathy. Am J Cardiol 62:325–327

    Google Scholar 

  • Weibel ER (1969) Stereological principles for morphometry in electron microscopic cytology. Int Rev Cytol 26:235–248

    Google Scholar 

  • Welsh J, Lynn TN, Haase GR (1963) Cardiac findings in 73 patients with muscular dystrophy. Arch Intern Med 112:199–206

    Google Scholar 

  • Wharton DC, Tszagoloff A (1967) Cytochrome oxidase from beef heart mitochondria. Methods Enzymol 10:245–250

    Google Scholar 

  • Zeviani M, Van Dyke DH, Servidei S, Bausermann SC, Bonilla E, Beaumont ET, Sharda J, Van der Laan K, DiMauro S (1986) Myopathy and fatal cardiomyopathy due to cytochrome c oxidase deficiency. Arch Neurol 43:1198–1202

    Google Scholar 

  • Zierz S, Jahns G, Jerusalem F (1989) Coenzyme Q in serum and muscle of 6 patients with Kearns-Sayre syndrome and 12 patients with ophthalmoplegia plus. J Neurol 236:97–101

    Google Scholar 

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Authors and Affiliations

  1. Department of Cardiology, Angiology and Pneumology, Heinrich-Heine University, Moorenstrasse 5, W-4000, Düsseldorf, Federal Republic of Germany

    B. Schwartzkopff, M. Block & B. E. Strauer

  2. Department of Pathology, Heinrich-Heine University, Düsseldorf, Federal Republic of Germany

    H. Frenzel

  3. Department of Neuropathology, Heinrich-Heine University, Düsseldorf, Federal Republic of Germany

    E. Neuen-Jacob

  4. Department of Neurology, Heinrich-Heine University, Düsseldorf, Federal Republic of Germany

    K. Reiners

  5. Department of Neurology, University of Bonn, Bonn, Federal Republic of Germany

    S. Zierz

Authors
  1. B. Schwartzkopff
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  2. S. Zierz
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  3. H. Frenzel
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  4. M. Block
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  5. E. Neuen-Jacob
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  6. K. Reiners
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  7. B. E. Strauer
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Dedicated to Professor Waldemar Hort on the occasion of his professor emeritus

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Schwartzkopff, B., Zierz, S., Frenzel, H. et al. Ultrastructural abnormalities of mitochondria and deficiency of myocardial cytochromec oxidase in a patient with ventricular tachycardia. Vichows Archiv A Pathol Anat 419, 63–68 (1991). https://doi.org/10.1007/BF01600154

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  • DOI: https://doi.org/10.1007/BF01600154

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