[Two-dimensional echocardiographic study of Ebstein's disease. Apropos of 16 cases].
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Biomedical subjects
Publications and source records attributed to A Choussat.
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The diagnosis of atrial myxoma, the most common cardiac tumour, has been greatly facilitated by the introduction of echocardiography. The principal echocardiographic features and the diagnostic pitfalls encountered in a personal series of 7 atrial myxomas (5 left and 2 right) studied by M mode and 2-dimensional echocardiography are reported. In M mode, left atrial myxoma is a relatively easy diagnosis when the tumour prolapses into the mitral orifice. On standard mitro-aortic scanning, it is recorded behind the anterior mitral leaflet as a mass of abnormal echos appearing shortly after mitral valve opening. The diagnosis is much more difficult or even impossible in nonprolapsing tumours. Right atrial myxomas, prolapsing into the tricuspid orifice, do not usually pose any diagnotic problems. 2-dimensional echocardiography shows its superiority in the early diagnosis of small, localised, relatively immobile and non-prolapsing tumours. The parasternal (long axis and transverse views), the apical (4 and 2 chamber views) and subxiphoid positions were the most useful incidences and the tumour was visualised as a more or less circumscribed mass of echos. In all cases, 2-dimensional echo provided information on the size, mobility, insertion and the length of the pedicle of the tumour. Atrial myxoma is being recognised more easily and more often nowadays by echocardiography, the diagnostic method of choice, and 2-dimensional studies should be preferred.
Recent studies have shown the high incidence of concealed Bundles of Kent in the reentry circuits of paroxysmal supraventricular tachycardia. Arguments in favour of the nodal or junctional level of reentry were observed in supraventricular tachycardia with complete atrioventricular dissociation. Two such cases under went electrophysiological investigation. In the first case, tachycardia was terminated by a complete infrahisian block. However, during atrioventricular dissociation, tachycardia could be initiated by a single atrial stimulus after an increased nodal conduction time and terminated by a single atrial stimulus or cardiac message. In the second case the supraventricular tachycardia presented with complete atrioventricular dissociation due to a retrograde ventriculo-atrial block. Atrial stimulation at progressively higher rates and premature atrial extra stimuli initiated the tachycardia but could not terminate it, so confirming the non-participation of the atrium in the reentry circuit. These two cases suggest that the ventricle (case I) and the atrium (case II) are not indispensable links in junctional or nodal reentry circuits. Case II was suggestive of a common initial pathway developing retrograde unidirectional block during tachycardia.
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The writers record a severe case of renovascular hypertension resulting from fibromuscular dysplasia which appeared in the only main left renal artery. The first clinical symptoms were noticed at five months. The failure of medical treatment led to exploratory surgery at seven months with renal revascularisation by an aortorenal by-pass using a hypogastric arterial autograft. The writers stress the scarcity of hypertension symptoms among infants of less than one year and conclude the impossibility of prognosis in spite of an apparent cure after six months.
Left ventricular function was studied in 15 patients with rapidly progressive Duchenne de Boulogne muscular dystrophy. Only one patient had a previous history of cardiac failure. All patients had typical electrocardiographical changes. Haemodynamic and angiographic investigation was performed in all patients. Three groups of patients were distinguished according to angiographic criteria : group I comprising 7 normal patients. group II comprising 4 "intermediary" patients, and group III of 4 patients with hypokinetic cardiomyopathy. Segmental ventricular dyskinesis was observed in 6 patients. Mitral incompetence was present in 2 cases and mitral valve prolpse in 1 case. All patients had a normal resting cardiac index. Patients in group II and III had end diastolic left ventricular pressures greater than 15 mmHg. An intravenous angiotensin test was performed in 4 patients from groups I and II. Impaired left ventricular function was demonstrated in patients thought to be normal in the basal state. Finally, myocardial involvement in this group of patients runs a parallel course to the duration and severity of the peripheral muscular disease.
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The haemodynamic action of I-V Cordarone have been studied in 20 subjects over a 15 minute period. Over the 15 minute period we studied variations in left pulmonary and ventricular pressures, in cardiac output, and in the paramaters of contraction VEC max, V max and Taylor's index. At a dose of 5 mg/kg there is a fall in peripheral resistance (1274 +/- 232 to 915 dynes/s/cm-5), and the measurements of contraction hardly vary. At a dose of 10 mg/kg, after an initial increase in output and a fall in peripheral resistance there occurs, after 4 minutes, an increase in left ventricular end-diastolic pressure (from 6 +/- 2 to 13 +/- 4 mmHg, P less than 0.01), and a lowering of contractility (VEC max reduced from 49.7 +/- 12.4 to 27.1 +/- 3.8, P less than 0.001). We have shown that the first phase of this response is due to the solvent (Tween 80), while the fall in contractility is due to the amiodarone. Doses above 10 mg/kg must be used with care, and only if there is no evidence of impaired cardiac function.
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A retrospective study of 100 cases of obstructive cardiomyopathy of the left ventricle has allowed us to predict a mean survival of 30 years after the murmur has been discovered. A comparative angiographic and haemodynamic study was carried out on 50 cases using the NYHA classification into four functional stages. The lowering of functional status, at rest, seems to occur: -With the advent of a permanent intraventicular gradient (6 +/- 5 mmHg in stage 56 +/- 38 mmHh in stages III and IV, p less than 0.001). Despite preservation of the indices of contractility (VECmax 1.81 +/- 0.66 c/s at stage I, 1.71 +/- 0.7 C/S at stages III and IV). -With a progressive change in the ventricular complicance (dV/dP/VTD 0.029 +/- 0.016 at stage I, 0.017 +/- 0.01 at stage III and IV). A progressive change in venticular complicance seems to be secondary to an increase in the parietal diastolic thickness and to lesions visible histologically. It governs the natural history of the condition and the results of surgical treatment.
Two new cases of ventricular tachycardia (VT) in the infant are reported, and reviewed in the light of the 23 case histories found in the literature. The diagnosis rests upon eliminating a pre-excitation syndrome, which is so common in this age group. The VTs found in infants are rapid, irregular, and take many different forms. They often necessitate urgent treatment with electric shocks. Preventive treatment consists of a combination of procainamide and beta-blockers in relatively large doses. The search for an aetiological agent should include a haemodynamic and angiocardiographic study of all the chambers of the heart to exclude cardiomyopathy, tumours, papyraceous right ventricle and congenital heart defects. Where no cause can be demonstrated, preventive treatment should be given, with regular electrical testing and other follow-up investigations. An attempt to reduce the drug dosage should be made every 6 months, in hospital. In cases which prove resistant despite adequate treatment, it seems justifiable to carry out a pericardial exploration with the aim of diathermising the ectopic focus; this approach is suggested because of the poor natural history of this type of case.
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The left ventricular kinaetics of 29 coronary patients (pure angina and with sequela of myocardial infarction) was studies by biplane angiocardiography. Their contractility was assessed by measurement of the Vmax and VECmax indices derived from the relationship between contractile elements shortening speed-overall wall tension, in isovolumetric phase. An excellent relationship links the hypokinaetic area with decrease of the ejection fraction (SV/LSV): when the hypokinaetic area exceeded 20% of the overall endocardial surface, the ejection fraction deveased below 0.40, and signs of cardiac failure were manifest. Pure anginal patients at rest kept normal kinaetics, late diastolic volume, ejection fraction and myocardial mass. A myocardial hypertrophy develops in the areas adjacent to the fibrous scar. In some cases (group I) it compensates for the ventricular dysfunction; in other cases, it is not sufficient to compensate for the ejection fraction reduction. One must then admit the presence of diminished contractility in the areas adjacent to the fibrous scar, as is suggested by the increase of the late diastolic pressure, the decrease of the externel work of the left ventricle and of the contractility indices. Analysis of both the natural and post-operative courses in these patients shows that Vmax the ejection fraction and the hypokinaetic areas afford excellent criteria for prognosis and operability.