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. 2004 Oct;6(4):1-30.

Right ventricular myocardium in Fallot's tetralogy: a light microscopic, morphometric and ultrastructural study

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Right ventricular myocardium in Fallot's tetralogy: a light microscopic, morphometric and ultrastructural study

S Kuruvilla et al. Images Paediatr Cardiol. 2004 Oct.

Abstract

Aims: To analyze peroperative biopsies of RV myocardium in Tetralogy of Fallot by light microscopy, morphometry and electron microscopy in order to determine the degree of hypertrophy and degenerative changes and to correlate these changes with clinical and haemodynamic parameters.

Materials and methods: Right ventricular myocardium obtained peroperatively during surgical correction of Tetralogy of Fallot along with age-matched control samples were processed for routine light and electron microscopy using standard processing techniques. Mean cell diameter was analyzed using manual morphometric methods and ultrastructural study was carried out using a Philips transmission electron microscope.

Results: The most consistent features of hypertrophy were the mitochondrial changes and increased nuclear convolutions. Majority of the patients had hypertrophy with mild to moderate degenerative changes. Severe degeneration was associated with irreversibility and was related to the severity and chronicity of the disease. There was a significant correlation of the morphological changes with clinical and haemodynamic parameters.

Conclusions: Peroperative histomorphometric and ultrastructural evaluation of the RV myocardium in Tetralogy of Fallot reflects the effect of haemodynamic stresses on the right ventricular muscle and correlates with clinical cardiac dysfunction. It may be a useful adjunct in determining the time for surgical intervention and in predicting clinical outcome.

Keywords: Heart defects; Myocardium; Tetralogy of Fallot; congenital; heart; mitochondria; ventricles.

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Figures

Figure 1
Figure 1
Age distribution
Figure 2
Figure 2
Histomorphometric evaluation of cell diameter vs. no. of cases
Figure 3
Figure 3
Control RV myocardium. Longitudinal section showing cross striations and normal nuclear contours. Semi thin section Toluidine blue LM X400
Figure 4
Figure 4
Electron micrograph control RV muscle. Normal Z bands, smooth nuclear contour. EM magnif 4500X
Figure 5
Figure 5
Electron micrograph control RV muscle. EM magnif 30000X
Figure 6
Figure 6
Electron micrographs control RV muscle. Note the smooth nuclear contours EM magnif 15000X
Figure 7
Figure 7
Cross section TOF RV myocardium showing hypertrophy. Semithin section. Toluidine blue LM X400
Figure 8
Figure 8
Longitudinal section TOF RV myocardium showing hypertrophy and myofibrillar disarray. Semithin section Toluidine blue LM X200
Figure 9
Figure 9
CS TOF RV myocardium showing severe degeneration.The cardiomyocytes show marked crowding and atrophy. Semithin section Toluidine blue LM X200
Figure 10
Figure 10
Longitudinal Section TOF RV myocardium showing hypertrophy, myofibrillar disarray and Z band abnormalities. Semithin section Toluidine blue LM X200
Figure 11
Figure 11
Longitudinal Section TOF RV myocardium showing hypertrophy and myofibrillar disarray. Semithin section Toluidine blue LM X400
Figure 12
Figure 12
Longitudinal Section TOF RV myocardium showing hypertrophy, myofibrillar disarray and increased nuclear convolutions. Semithin section Toluidine blue. LM X400
Figure 13
Figure 13
Longitudinal section: TOF RV myocardium showing hypertrophy, mild myofibrillar disarray, nuclear elongation and irregularity. Semithin section Toluidine blue LM X400
Figure 14
Figure 14
Longitudinal section TOF RV myocardium showing severe degeneration seen as marked interstitial and perivascular fibrosis. The cardiomyocytes appear pale with mild atrophic changes. Semithin section Toluidine blue LM X200
Figure 15
Figure 15
Cross section TOF RV myocardium showing hypertrophy, myofibrillar disarray with interstitial and perivascular fibrosis. Semithin section Toluidine blue LM X200
Figure 16
Figure 16
Electron micrograph TOF RV myocardium showing moderate degeneration, increased mitochondria and myofibrillar disarray. EM magnif: 7000X
Figure 17
Figure 17
Electron micrograph TOF RV muscle. Severe degeneration- intracytoplasmic vacuolation. EM magnif 3000X
Figure 18
Figure 18
Electron micrograph TOF RV muscle Severe degeneration- myelin figure (arrow) EM magnif 7000X
Figure 19
Figure 19
Electron micrograph TOF RV muscle. Markedly dilated, irregular and ruptured mitochondria. EM Magnif 15000X
Figure 20
Figure 20
Electron micrograph TOF RV muscle. Severe degeneration- myelin figures, abnormal Z bands, rough endoplasmic reticulum abnormalities and basement membrane thickening. EM magnif 15000X
Figure 21
Figure 21
Electron micrograph TOF RV muscle. Longitudinal section showing widened intercellular junctions (arrow). EM magnif 7000X
Figure 22
Figure 22
Electron micrograph TOF RV muscle. Longitudinal section: showing widened intercellular junctions (arrow) EM magnif 15000X
Figure 23
Figure 23
Electron micrograph TOF RV muscle. Longitudinal section showing widened intercellular junctions (arrow). EM magnif 30000X
Figure 24
Figure 24
Electron micrograph TOF RV muscle. Hypertrophied cardiomyocytes, convoluted nuclei, abundant mitochondria. EM Magnif 7000X
Figure 25
Figure 25
Electron micrograph TOF RV muscle. Convoluted nuclei with intranuclear tubule formation and abundant mitochondria. EM Magnif 30000X
Figure 26
Figure 26
Electron micrograph TOF RV muscle. Increased nuclear convolutions. EM Magnif 20000X
Figure 27
Figure 27
Electron micrograph TOF RV muscle. Hypertrophied cardiomyocytes, convoluted nuclei with intranuclear tubule formation and abundant mitochondria. EM Magnif 7000X
Figure 28
Figure 28
Electron micrograph TOF RV muscle. Convoluted nuclei with intranuclear tubule formation and abundant mitochondria. EM Magnif 20000X
Figure 29
Figure 29
Key electron microscopic findings vs. no. of cases (%)
Figure 30
Figure 30
Electron micrograph TOF RV muscle. Cross section of hypertrophic muscle fiber and convoluted nucleus (arrow). EM magnif 3000X
Figure 31
Figure 31
Electron micrograph TOF RV muscle. Hypertrophied cardiomyocytes, convoluted nuclei and abundant mitochondria. EM Magnif 4500X
Figure 32
Figure 32
Electron micrograph TOF RV muscle. Longitudinal section of myocyte showing hypertrophy, mild degeneration. Note Z bands and nuclear convolutions. EM magnif 3000X
Figure 33
Figure 33
Longitudinal section TOF RV myocardium showing myofibrillar disarray, nuclear irregularities and interstitial fibrosis. Semithin section Toluidine blue. LM X200
Figure 34
Figure 34
Electron micrograph TOF RV muscle. Moderate degeneration-abnormal Z bands, nuclear convolutions. EM magnif 20000X
Figure 35
Figure 35
TOF RV myocardium showing irreversible degeneration seen as marked interstitial and perimysial fibrosis. The cardiomyocytes appear atrophic. Semithin section Toluidine blue LM X200
Figure 36
Figure 36
Electron micrograph TOF RV myocardium showing severe degeneration. The cardiomyocytes are atrophic with vacuolation and mitochondrial abnormalities. EM magnif: 7000X
Figure 37
Figure 37
Electron micrograph TOF RV myocardium showing severe degeneration. The cardiomyocytes are markedly atrophic.EM magnif: 2000x
Figure 38
Figure 38
Electron micrograph TOF RV muscle. Interstitial fibrosis, fibroblast with nucleus and collagen. EM magnif. 7000X

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