[Congenital heart diseases in the adult].
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Biomedical subjects
Publications and source records attributed to J Normand.
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Two cases of multiple coronary aneurysms are reported in patients aged 15 and 25 years. The clinical presentation in both cases was myocardial infarction. The diagnosis was confirmed by the demonstration of rounded calcification at the border of the aneurysms on chest X-Ray, and multiple aneurysmal dilatations on selective coronary angiography. These two cases and the other 16 found in the medical litterature allow analysis of the clinical signs of idiopathic coronary aneurysms, generally these of acute coronary insufficiency. Two-dimensional echocardiography seems to be the most effective means of detecting this type of pathology. The incidence of coronary aneurysms during infantile periarteritis nodosa, and in Kawasaki's syndrome, very similar conditions affecting infants and children, suggest that these two diseases may play a role in the formation of coronary aneurysms in adolescents and young adults, which would therefore be sequellae of an inflammatory arteritis of childhood.
From 1973 to 1977, 93 infants (63 newborns) with d-TGA were treated with Blalock-Hanlon operation. All children had Rashkind atrioseptostomy at birth. 65 infants with isolated TGV were operated upon with two deaths (3%) and without any complication. 18 patients with TGV and VSD were treated with Blalock-Hanlon operation and Pulmonary Artery Banding and resulted in 5 deaths (28%). The other 10 cases had complex lesions. The overall operative mortality is 8.6%. The late mortality is 3.5%. The authors conclude that the Blalock-Hanlon operation is a safe procedure even in complex lesions. This kind of surgery does not seem to induce any arythmias but the authors did not perform any 24 hours ECG recording. This type of surgery makes the repair (Mustard operation) easier since the atrial septum is already removed.
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Of a consecutive series of 91 babies aged less than 2 years, operated on for closure of a ventricular septal defect during the last four years, the 61 cases with a minimum post operative follow up period of 18 months were retained for review. Closure of the ventricular septal defect was carried out directly 41 times, and after pulmonary artery banding in the other 20 cases, using deep hypothermia with a short period of circulatory arrest and cardiopulmonary bypass.
The authors report four cases of metabolic cardiomyopathy with lipid infiltration diagnosed by skeletal muscle and myocardial biopsy in children with no clinical signs of muscular dystrophy. Normal or increased serum and urinary carnitine levels excluded a primary carnitine deficiency. A deficiency in muscular-palmityl-carnitine-transferase was demonstrated. This pathogenic mechanism may be an indication for treatment with carnitine, but the results are less spectacular than in primary carnitine deficiency states.
A case of diffuse plane xanthomatosis assoicated with systemic amyloidosis and multiple myeloma at its outset is reported. Plane xanthomatosis is certainly an autonomous entity in comparison with systemic amyloidosis, for there are no amyloid deposits in xanthoma. The patient had lambda type IgG paraproteinemia, with Bence-Jones proteinuria. Lipid tests were considered as normolipemic though some levels recall a type IV hyperlipoproteinaemia. A review of literature about the association "xanthomatosis-multiple myeloma" was made, after the important work of Bazex, Dupré and Mrs. Christol-Jalby. It allows us to distinguish two differnet descriptions: 1. When there is hyperlipoproteinemia, all clinical types of xanthomas may exist; multiple myeloma is generally typical (but sometimes not very progressive). 2. When there is normolipidemia, the main clinical type is diffuse plane xanthomatosis; multiple myeloma is atypical and often only a monoclonal gammapathy is found. 3. However in both cases, the outstanding clinical type is diffuse plane xanthomatosis: whether normo- or hyperlipemic, this therefore indicates a possible underlying disease, and above all a multiple myeloma.
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One hundred and fifty-three men with angina were studied to determine the risk of death over a 5-year period. Multivariate analysis using age, systolic and diastolic pressure, and six electrocardiographic variables (QRS axis, PR interval, the sum of S in V1 and R in V5, T-wave and ST segment anomalies and incomplete left bundle branch block) identified sub-groups having very different prognoses. An analysis using only six variables (omitting diastolic pressure, ST segment anomalies and PR interval) retained a good discriminatory value, and this same discriminant function calculated from half of the sample had good prognostic value in the other half. A prognostic index based on this function (designed to simplify the calculations in clinical use) identified a sub-group (24% of the total group) in which no mortality occurred, while another sub-group (16% of the total group) suffered a mortality of 67%. The logical use of the blood pressure and ECG leads to a more precise prognosis in angina and should help in determining the indications for myocardial revascularisation.
The findings on echocardiography and their anatomical correlations are reported in a group of 15 neonates with the syndrome of hypoplasia of the left side of the heart. The lesions which make up this syndrome could be defined precisely using echocardiography. In the major forms of this syndrome (10 cases), the correlation between the clinical and the echocardiographical findings was sufficient to establish the diagnosis, and to avoid the necessity for cardiac catheterisation without prejudice to the treatment plan. Characteristic findings in the major types were: --an aortic diameter less than or equal to 5 mm; --a left ventricle which was absent or had a diameter of less than 11 mm with an LV/RV ratio of less than 0.6; --a mitral valve which was absent or had a very abnormal form with multiple echoes. The differential diagnosis on echocardiography and the limitations of the method are discussed.
Myocardial involvement in lupus erythematosis takes the form of an interstitial myocarditis with cellular infiltration and fibrinoid necrosis. The most lesions are perivascular, and involve the arterioles. The myocardial fibres are involved secondarily to the vascular lesions, or by grossly, damaging sclerosis. The clinical features are variable:--no clinical features, but haemodynamic evidence of abnormal ventricular function, and perhaps sudden death;--arrhythmias and disorders of atrio-ventricular conduction;--cardiac failure, which may be due to a genuine cardiomyopathy (a part may be played by hypertension, pulmonary hypertension, renal failure, constrictive pericarditis or haemodynamically major valve disorders);--abnormalities of the coronary trunk in a certain number of cases. If anti-nuclear antibodies are present in a cardiomyopathy, the presence of DLE or of a drug-induced lupus syndrome must be suspected. There remain some awkward cases which defy classification, and which systematic use of echocardiography and pericardial and myocardial biopsy may be able to define more accurately.
Between September 1st 1974 and June 1st 1976, 12 infants under the age of 6 months have been operated on by a dacron patch aortoplasty for coarctation of the aorta. The associated lesions were a patent ductus arteriosus in each case, a VSD in 8 cases, and a trans-position of the great arteries in 5 cases. A pulmonary artery banding was performed with the aortoplasty in 8 cases, and a Blalock-Hanlon operation in one instance. There were two operative deaths (17 p. 100), amongst them one in a 1400 g premature infant, and a late death (3rd month). Two infants have a clinical aspect of recoarctation. In four infants, the post-operative hemodynamic and angiographic study carried out before the treatment of associated intra-cardiac lesions, shows a good result of the coarctation repair. This particular technique, although not performed as a routine in the infant with coarctation of the aorta, seems to be of interest in the most severe forms of the disease, with diffuse isthmus narrowing and intra-cardiac defects.
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A case of severe Fallot's tetralogy is presented; there was angiographic evidence of progression towards pulmonary atresia. The relationship between Fallot's tetralogy and pulmonary atresia with open septum is discussed, as is the variability of the pulmonary circulation found in cases of pulmonary atresia with septal defect, and the treatment is also discussed.
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Over the past 3 years, a Mustard operation has been performed in 38 infants aged 30 days to 24 months, among them 29 less than one year of age. There were 27 TGV with intact ventricular septum, 8 TGV and VSD, (isolated in 3, and associated with PS in 2 and with coarctation of the aorta in 3), 2 TGV with isolated PS, and 1 TGV with aorta-pulmonary window. The operation was carried out under deep hypothermia, circulatory arrest and limited by-pass, using a pericardial patch in all cases but one. The operative mortality is 8% (3 cases) for the whole group, 7% in infants less than one year of age, and 3% for all TGV with intact ventricular septum. The long term results are reviewed, with a follow-up of 2 to 36 months (mean 17 months).
This grave complication is a major cause of mortality in ventriculo-atrial shunts in children with hydrocephalus. It occurs at a much higher rate than with long-term indwelling intracavitary pacemakers, which suggests that the shunt procedure is responsible for either chronic infection or the introduction of cerebral thromboplastin.