Transvascular endomyocardial biopsy in infants and small children: Myocardial findings in 10 cases of cardiomyopathy

doi:10.1016/S0046-8177(79)80069-6

Human Pathology

Volume 10, Issue 1, January 1979, Pages 15-30

https://doi.org/10.1016/S0046-8177(79)80069-6 Get rights and content

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Transvascular endomyocardial biopsy specimens from nine children with congestive cardiomyopathy and one with hypertrophic cardiomyopathy were studied by light microscopy using sections 1 μ thick cut from Epon embeded tissue and by electron microscopy. There was a disparity between the severity of the physiologic impairment and the morphologic abnormalities. Interstitial fibrosis was present only in the one case in which significant viral antibody titers were obtained. The sizes of the cardiac muscle cells varied abnormally in all specimens. Cardiac muscle cells in two patients contained abnormal mitochondria, and a leptomeric fibril was found in one patient. Virologic cultures of the tissues were negative and no viral particles were identified by electron microscopy. An attempt was made to correlate the clinical and pathologic findings.

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Safety and utility of endomyocardial biopsy in infants, children and adolescents: A review of 66 procedures in 53 patients
1990, Journal of the American College of Cardiology
The benefits and risks of endomyocardial biopsy in infants, children and adolescents were determined by reviewing the indications for and complications and results of 66 procedures, in 53 patients aged 2 months to 20 years. One patient had a pneumothorax, and three had a right ventricular perforation. Ventricular tachycardia developed in four patients; it was treated with lidocaine in three and was self-limited in one. The procedure was unsuccessful in two patients.
Among 25 patients with a prebiopsy diagnosis of idiopathic dilated cardiomyopathy, microscopic features were consistent with cardiomyopathy in 24 (06%) and were normal in 1. Of nine patients with clinically suspected myocarditis, only two (22%) had microscopic evidence of inflammation, and seven had chronic nonspecific features suggestive of dilated cardiomyopathy. Of eight patients with unexplained arrhythmias, six (75%) had microscopic findings compatible with dilated cardiomyopathy and two had myocarditis. Biopsy tissue samples from seven patients with nondilated forms of cardiomyopathy (four hypertrophic, three restrictive) were consistent with the clinical diagnosis in six and were inadequate in one. Cardiac biopsies were also performed in four patients with other disorders. Among the 51 patients with adequate biopsy specimens, microscopic features were considered diagnostic in 5, confirmatory in 44 and not helpful in 2 with normal tissue.
The results indicate that endomyocardial biopsy is safe in infants, children and adolescents. It is useful for the evaluation of cardiomyopathy and specific secondary forms of myocardial disease. There seems to be little correlation, however, between clinical and tissue diagnoses of myocarditis.
An ultrastructural study of the stretch-induced hypertrophy of skeletal muscle
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The teres minor muscle of the adult chicken was studied ultrastructurally following tonic stretch-induced hypertrophy. The contralateral control muscle fibres showed compact myofibrils and proliferation of normal Z-bands. Myofibrils of the hypertrophied muscle however, showed Z-band alterations as Z-band expansions and Z-band streaming. Thus Z-band is a highly responsive structure to tonic stretch. Since a number of neuro-muscular conditions display Z-band anomolies, the latter occurring in response to a variety of metabolic and physiologic stimuli, including tonic stretch as shown here, represents a non-specific phenomenon.
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An X-linked mitochondrial disease affecting cardiac muscle, skeletal muscle and neutrophil leucocytes
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An X-linked recessive disease is reported in a large pedigree. The disease is characterised by a triad of dilated cardiomyopathy, neutropenia and skeletal myopathy. The untreated patients, all boys, died in infancy or early childhood from septicemia or cardiac decompensation.
Ultrastructural abnormalities were observed in mitochondria in cardiac muscle cells, neutrophil bone marrow cells and to a lesser extent (0–9%) in skeletal muscle cells. Membrane-bound vacuoles were seen in neutrophil bone marrow cells. Intramuscular fat droplets were increased in type I skeletal muscle fibres.
An affected patient had intermittent lactic acidemia, borderline low plasma carnitine, the latter decreasing during periods of illness, and low muscle carnitine (27% pretreatment; 35–40% posttreatment). While on treatment with oral carnitine he had less weakness and no cardiac complaints, but his neutropenia was not affected.
Respiratory chain abnormalities were observed in this patient's isolated skeletal muscle mitochondria. These were:
(1) diminished concentrations of cytochromes c₁ + c, b and aa₃ to 29, 47 and 64% of the averaged controls, and (2) a lowered P : 0 ratio for oxidation of ascorbate + TMPD, with diminished uncoupler stimulated Mg²⁺-ATPase activity. Muscle AMP deaminase was deficient (5 resp. 17%).
Only one previous report (Neustein et al. 1979) on X-linked mitochnodrial cardiomyopathy exists, which probably refers to the same entity. Biochemical studies and haematological abnormalities (neutropenia) are reported for the first time.
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1981, The Journal of Pediatrics
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^*: Study supported in part by National Institutes of Health General Research Support Grant 5 SO1-RR05469-13 and the Michael J. Connell Foundation, Los Angeles, California.

^†: Research Fellow in Pediatric Pathology and Electron Microscopy, Department of Pathology, Children's Hospital of Los Angeles and University of Southern California School of Medicine, Los Angeles, California.

^‡: Professor of Pathology, University of Southern California School of Medicine. Pathologist and Director of Electron Microscopy, Department of Pathology, Children's Hospital of Los Angeles, Los Angeles, California.

^§: Professor of Pediatrics, University of Southern Galifornia School of Medicine. Head, Division of Cardiology Children's Hospital of Los Angeles. Los Angeles. California.

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Transvascular endomyocardial biopsy in infants and small children: Myocardial findings in 10 cases of cardiomyopathy*

Abstaact

J. Ultrastr. Res.

J. Mol. Cell. Cardiol.

J. Mol. Cell. Cardiol.

J. Ultrastr. Res.

Lancet

J. Ultrast. Res.

Human Path.

Am. Heart J.

J. Mol. Cell. Cardiol.

J. Mol. Cell. Cardiol.

Myocardial biopsy: a miniaturized technic for infants and small children—development, testing in animals and clinical application

Circulation

A simple rapid method for staining epoxy-embedded specimens for light micros-copy with the polychrome stain Paragon-1301

Amer. J. Clin. Path.

RD-114 virus infectivity assay by measurement of DNA polymerase activity and virus group specific antigen

Proc. Soc. Exper. Biol. Med.

Propagation and isolation of group A coxsackie viruses in RD cells

J. Clin. Microbiol.

Clinicopathological study of the thickening of parietal endocardium in the adult heart

Jap. Heart J.

Presence of widened and multiple intercalated discs in the hypertrophied canine heart

Circ. REs.

Myofibrillar abnormalities following cardiac muscle injury

Membrane junctions in the intermembrane space of mitochondria from mammalian tissues

J. Cell Biol.

A case of severe hypermetabolism of nonthyroid origin with a defect in the maintenance of mitochondrial respiratory control: a correlated clinical, biochemical and morphological study

J. Clin. Invest.

Light-cored dense particles in mitochondria of a patient with skeletal muscle and myocardial disease

J. Neuropath. Exp. Neurol.

The architecture of the human myocardium

Johns Hopkins Med. J.

Dissociation of the “surface membrane complex” in atrophic muscle fibers

Nature (London)

Ultrastructure of membrane in denervation atrophy

Am. J. Path.

Ultrastructural features of degenerated cardiac muscle cells in patients with cardiac hypertrophy

Am. J. Path.

Ultrastructural changes in the canine myocardium with right ventricular hypertrophy and congestive heart failure

Lab. Invest.

Multiple intercalated discs in hypertrophied human hearts

Circulation

The embryologic development of the mammalian heart

Replacement of myosin during development of cardiac hypertrophy

Circ. Res.