glycogen and Tetralogy-of-Fallot

Ultrastructural quantitative and qualitative investigations of the myocardium of 7 children with Fallot's disease and 3 children with a ventricular septal defect (VSD) have shown bizarre nuclei and a slight increase in the myofibril share of the volume (Fallot's disease: 51.22%; VSD: 56.85%). This may be interpreted as a sign of a cellular hypertrophy. The mitochondria have a proportion of 35.07% and 33.75%, respectively. The glycogen content is relatively high. The lipofuscin bodies originate mainly from mitochondria having a percentage of 1.43 and 1.67, respectively. Particular signs of a lesion could not be observed. From the ultrastructural findings on the morphology of heart muscle cells it can be deduced that operations of vitia may be safely carried out.

Topics: Adolescent; Cardiomegaly; Child; Child, Preschool; Female; Glycogen; Heart Septal Defects, Ventricular; Humans; Infant; Lipofuscin; Male; Microscopy, Electron; Mitochondria, Heart; Myocardium; Tetralogy of Fallot

In 7 children with Fallot's tetralogy ultrastructural quantitative and qualitative investigations on the heart muscle were carried out. Bizarre nuclei and slightly increased volume of mitochondrial part indicated hypertrophy. The amount of mitochondria was 35.07 per cent. The content of glycogen is relatively high. The lipofuscin bodies, mainly originating from mitochondria, amount to 1.43 per cent. No essential damage is observed. From the ultrastructural findings can be concluded that surgery may be done without suspicion as to cardiac cell morphology.

Topics: Cell Nucleus; Child; Glycogen; Humans; Lipofuscin; Mitochondria; Myocardium; Myofibrils; Sarcoplasmic Reticulum; Tetralogy of Fallot

Ultrastructural studies were made of operatively resected crista supraventricularis muscle in 59 patients with congenital heart diseases, or whom 54 had obstruction to right ventricular outflow. Relationships of anatomic diagnosis, age, peripheral arterial oxygen saturation (PAO2), peak right ventricular systolic pressure gradient and right ventricular end-diastolic pressure (RVEDP) to hypertrophic changes, abnormalities of cellular and myofibrillar orientation, and degenerative alterations were determined. Changes directly related to hypertrophy were: cell diameters greater than 20 mu, irregular cell shape, lobulated nuclei, multiple intercalated discs, dilated T tubules, abnormal Z bands, and increased numbers of ribosomes. Abnormalities of cellular or myofibrillar orientation were focal in distribution and occurred in 12 patients, most of whom had elevated RVEDP, decreased PAO2, markedly enlarged cells, and interstitial fibrosis. Interstitial fibrosis was prominent in 19 patients and was associated with cellular hypertrophy, elevation of RVEDP, and increased age of the patients. Degenerative changes (myofibrillar lysis, abnormally small mitochondria, myelin figure formation, and proliferation of sarcoplasmic reticulum in cardiac muscle cells ocurred in six patients and correlated with increased age, decreased PAO2, and elevated RVEDP. Mitochondria containing glycogen deposits were present in 17 patients, most of whom had decreased PAO2. The variability of morphologic manifestations of chronic cardiac hypertrophy and the relationships of hypertrophic changes to orientation abnormalities and degenerative alterations are discussed.

Topics: Adolescent; Adult; Cardiomegaly; Cell Nucleus; Child; Child, Preschool; Collagen; Cytoplasmic Granules; Glycogen; Heart Defects, Congenital; Heart Septal Defects, Ventricular; Humans; Hypertrophy; Microscopy, Electron; Microtubules; Middle Aged; Mitochondria, Muscle; Myocardium; Myofibrils; Pulmonary Valve; Pulmonary Valve Stenosis; Ribosomes; Sarcoplasmic Reticulum; Tetralogy of Fallot

Topics: Acid-Base Equilibrium; Aminohippuric Acids; Blood Gas Analysis; Blood Volume; Cardiac Output; Exercise Test; Glomerular Filtration Rate; Glycogen; Heart Rate; Lactates; Muscles; Oxygen; Oxygen Consumption; Partial Pressure; Phosphates; Spirometry; Tetralogy of Fallot; Ventilation-Perfusion Ratio

Topics: Adult; Animals; Cardiomegaly; Cell Nucleus; Dogs; Endocardial Fibroelastosis; Glycogen; Heart Arrest; Heart Diseases; Heart Failure; Humans; Hypoxia; Male; Middle Aged; Mitochondria, Muscle; Myocardium; Tetralogy of Fallot; Wolff-Parkinson-White Syndrome

Topics: Coronary Circulation; Eisenmenger Complex; Glycogen; Heart; Humans; Hypoxia; Isoenzymes; Kinetics; L-Lactate Dehydrogenase; Lactates; Myocardium; Oxygen Consumption; Phospholipids; Pyruvates; Tetralogy of Fallot; Triglycerides

Topics: Cell Nucleus; Ductus Arteriosus, Patent; Glycogen; Heart Septal Defects, Ventricular; Heart Ventricles; Humans; Microscopy, Electron; Mitochondria; Myofibrils; Pigments, Biological; Pulmonary Valve Stenosis; Tetralogy of Fallot

Topics: Axons; Biopsy; Child; Child, Preschool; Cytoplasmic Granules; Glycogen; Heart; Heart Conduction System; Heart Defects, Congenital; Heart Septal Defects; Humans; Lysosomes; Microscopy, Electron; Mitochondria; Myocardium; Nerve Endings; Nerve Fibers, Myelinated; Tetralogy of Fallot

Topics: Acid Phosphatase; Adolescent; Angiocardiography; Blood Pressure; Child; Child, Preschool; Glycogen; Heart Ventricles; Hemodynamics; Histocytochemistry; Humans; Injections, Intravenous; Microscopy, Electron; Monoamine Oxidase; Propranolol; Tetralogy of Fallot

Topics: Adolescent; Adult; Aging; Child; Child, Preschool; Endoplasmic Reticulum; Glycogen; Heart Defects, Congenital; Heart Septal Defects, Atrial; Heart Septal Defects, Ventricular; Histocytochemistry; Humans; Lysosomes; Methods; Microscopy, Electron; Mitochondria, Muscle; Myocardium; Myofibrils; Organoids; Tetralogy of Fallot