Further observations on conduction tissues in univentricular hearts--surgical implications.
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Biomedical subjects
Publications and source records attributed to M C Joseph.
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Net photosynthesis on a leaf area and leaf weight basis increased significantly with ploidy in a 4X, 6X, 8X and 10X allopolyploid series of tail fescue (Festuca arundinacea Schreb.). Total protein did not increase significantly with ploidy. Rocket immunoelectrophoresis was used to quantitate ribulose-1, 5-bisphosphate carboxylase (RuBPCase) protein. RuBPCase content, expressed on both a concentration basis and as a percentage of total protein increased significantly with ploidy in both field and greenhouse experiments. The range of RuBPCase content was 16 to 73% of total protein and 2.8 and 6.5 mg/ml of extract. Specific activity of RuBPCase did not increase significantly with ploidy. Chlorophyll concentration increased as a quadratic function of ploidy, with the mean for 8X genotypes representing maximal chlorophyll content. Evidence is presented that increasing concentrations of RuBPCase are associated with higher net photosynthesis rates in tall fescue. This suggests that RuBPCase may represent a marker for increased net photosynthesis. RuBPCase was extracted in a partially active state or inhibited state and must be fully activated by Mg(2+) and HCO(3) (-) to measure maximal activities. Polyploidization appeared to increase selectively the allocation of total protein for synthesis of RuBPCase; however, there was also a range for carboxylase content among the genotypes within a given ploidy level.
Most univentricular hearts have two chambers in their ventricular mass, only one of which possesses an atrioventricular connection. Categorisation into univentricular heart of right, left, or indeterminate type, using two dimensional echocardiography has been successfully achieved in 122 out of 132 patients to whom this technique has been applied. Thus, right ventricular rudimentary chambers in 84 univentricular hearts of left ventricular type were shown to be anterosuperior and either to the right or left. In contrast, left ventricular rudimentary chambers in 25 univentricular hearts of right ventricular type were posteroinferior and to the right, left, or directly posterior. Thirteen univentricular hearts of indeterminate morphology were characterised by absence of rudimentary chamber on angiography and echocardiography. The trabecular pattern of both main and rudimentary chambers were separately identified in some of the patients with univentricular hearts of right and left ventricular type and two dimensional echocardiography also illustrated the mode of atrioventricular connection, either via two atrioventricular valves, a common valve, or a valve straddling or overriding the trabecular septum. Finally it was also possible to distinguish absent atrioventricular connection from an imperforate valve in 10 patients.
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A case is reported in which acute tricuspid regurgitation developed in a child with a ventricular septal defect resulting in a left ventricular-right atrial shunt. This was successfully treated by closure of the defect and tricuspid valve replacement. The anterior leaflet of the tricuspid valve was almost completely destroyed by endocarditis, though in previous reports of tricuspid valve endocarditis in association with ventricular septal defect in children, it has beem invariably the septal leaflet which is damaged.
Systolic blood pressure was measured at rest and during exercise in 43 children who had undergone operation for correction of coarctation of the aorta, five children awaiting surgery for coaractation, and 22 control children. Ages ranged from 2 to 15 years, mean 7-6 years. The mean blood pressure of children with coarctation in both the pre- and postoperative groups was significantly higher at rest than in the controls. Of 43 postoperative patients, 15 (35%) were hypertensive (systolic blood pressure more than 95th centile), and 12 of these had a gradient between the upper and lower limb. Seven of the 28 normotensive patients also had a gradient postoperatively. Exercise increased the blood pressure more in children with coarctation than in controls, but there was much individual variability and this difference was not significant. Some children with coarctation developed very high blood pressures on exercise, but this was not related to the presence of a gradient. The mean interval after operation was significantly shorter in the hypertensive group, independent of the age at operation. Hypertension with or without a gradient commonly persists despite apparent successful surgical correction, but exercise is of limited value in its assessment in this age group.
A contouring device for measuring the face (Cobb, 1972) was adopted and developed to make it suitable for use with children. Modifications were made to overcome incomplete facial coverage and lack of common head orientation by incorporating a method of optical head positioning which had been invented during the assessment of a stereophotogrammetric technique (Ainsworth and Joseph 1976). The precise alignment of a series of exposures with a common datum enables a set of contour maps to be processed by computer to produce three-dimensional measurements and sections of the complete face.
A case is described of corrected transposition in a situs solitus individual, in which, despite the presence of atrioventricular discordance (1-bulboventricular looping), the aorta was right-sided. A ventricular septal defect and pulmonary stenosis were also present, and the case had been erroneously diagnosed during life as complete transposition. The true diagnosis was not appreciated at surgery, and correction of the anomaly was attempted using a modification of Fontan's operation. The criss-cross atrioventricular connections were only fully appreciated at autopsy. The patient also exhibited ventricular preexcitation of Wolff-Parkinson-White variety type A. Histopathological examination revealed the presence of two atrioventricular nodes in the right atrium, each contacting the ventricular conduction tissue which was distributed to the morphologically appropriate ventricles. In addition, a left-sided accessory atrioventricular connection perforated the anulus of the left-sided, morphologically tricuspid, valve.
Forty-two children with eczema were studied for exercise-induced asthma (EIA), skin sensitivity to prick testing, blood eosinophil count, and immunoglobulins. 29 had a fall in peak expiratory flow rate after exercise greater than 20% and of these, 23 had symptoms of wheezing. 13 of the eczematous children showed a fall of less than 20%. The children with EIA showed greater cutaneous sensitivity (p less than 0.001) and a higher total serum IgE (p less than 0.025). 3 of the group with a fall of less than 20% had allergic rhinitis with skin sensitivity to grass pollen. The remaining 10 had no clinical evidence of allergic disease, other than eczema and skin sensitivity, and total IgE fell within the normal range. It is suggested that in a proportion of chilren with eczema there is little evidence of reaginic allergy.
In 100 children with persistent hypertension seen over the past 5 1/2 years the commonest causes of hypertension were chronic glomerulonephritis, reflux nephropathy, coarctation of the aorta, and obstructive uropathy, accounting for some 70% of cases. 17 children have died, but in the remainder hypertension has been controlled by surgery, chronic haemodialysis, or by the use of pharmacological agents. Methyldopa was the commonest drug used, and the children appeared relatively resistant to the side effects of this and of other drugs, even when large doses were used. The improvment is the prognosis of severe hypertension in childhood indicated in this survey is largely due to the availability of chronic haemodialysis and transplantation for end-stage renal disease, but the advances in diagnositc methods and surgical techniques and the introduction of new drugs have also contributed.
Arterial oxygen tension was measured from radial artery samples in 276 infants referred for cardiological investigation. Values obtained during air breathing in infants with congenital heart disease showed considerable overlap between 'cyanotic' and 'acyanotic' groups, and are of limited diagnostic use. By contrast, values obtained while breathing oxygen in concentrations of over 80%, measured in 182 infants, allowed clear differentiation between these groups. All infants with acyanotic, but only 2 of 109 with cyanotic lesions, achieved an arterial oxygen tension of more than 150 mmHg. In the cyanotic group the response to oxygen breathing was significantly greater in common mixing situations and in the hypoplastic left heart syndrome than with either pulmonary outflow tract obstruction or transposition of the great arteries. Infants with transposition had a significantly lower mean arterial oxygen tension in air than infants with other forms of cyanotic congenital heart disease. Of 23 infants whose final diagnosis was primary lung disease but in whom cyanotic congenital heart disease had been suspected, 7 achieved arterial oxygen tensions of more than 150 mmHg during oxygen breathing, and on this basis cardiac catheterization was not performed. We therefore conclude that measurement of the arterial oxygen tension while breathing high concentrations of oxygen should be routinely performed in the initial assessment of sick infants with suspected congenital heart disease.
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This report represents the Brompton Hospital experience of infants and neonates with critical pulmonary valve stenosis and aortic valve stenosis. There were 25 patients with critical pulmonary valve stenosis and 8 with critical aortic valve stenosis. Critical pulmonary stenosis: Among the 25 patients up to one year of age, 11 were girls and the commonest presenting symptom was cyanosis seen in 15, the commonest physical sign being a systolic ejection murmur with a single 2nd sound. A more detailed analysis of the symptoms, signs and findings at special investigation will be presented. The evolution of a satisfactory technique of operation will be indicated in these critically ill patients, and the late follow-up will be presented. Critical aortic stenosis: There were 8 patients in this group all of whom were male. Dyspnoea was the commonest presenting symptom with a systolic ejection murmur and hepatomegaly as the commonest signs. All patients in this group underwent open aortic valvotomy and/or valvuloplasty with 3 deaths. A late follow-up of the survivors will be presented. In summary, a series of 33 patients up to the age of one year with critical obstruction of either the pulmonary or aortic valves will be presented. (his is one of the larger series to be reported in this age group.
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