[Recurrent pericardial effusion in infants caused by benign hemangioendothelioma of the pericardium].
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Biomedical subjects
Publications and source records attributed to P Leborgne.
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Metastatic tumors of the heart are often asymptomatic but they may dominate the clinical picture. The location in the interventricular septum can account for conduction disturbances. The authors report a case of esophagus neoplasm with septal metastasis responsible for a complete atrioventricular block. The histologic study of the bundle of His by serial sections showing the destruction of the conduction fibres by malignant elements has not been carried out till now except in some observations. The prognostic of the atrioventricular metastatic block is very poor; the ten months' survival which was observed in the reported case is one of the most extended in literature.
Two cases of thyrotoxicosis including a first-degree heart block are reported; a study of action potentials of the bundle of His was achieved in the second case. The association atrioventricular block-hyperthyroidism is unusual but does not seem to be a pure coincidence. The direct effect of thyroid hormon on the myocardium is supposed to be responsible for the conduction disturbance; its disappearance with the cure of the thyrotoxicosis is a favourable argument for a relation of causality. The therapeutic consequence of this association is to exclude completely the use of betablockers.
The Williams and Beuren's syndrome associates a particuliar facies, a mental debility, a supravalvular aortic stenosis, and frequent vascular anomalies of pulmonary artery as well as aorta and its branches. Relations between this syndrome and congenital idiopathic hypercalcemia seem now determined; the hypertension which has been frequently observed in this last case, seems more unusual in the case of Williams and Beuren's syndrome. The authors report a case about this syndrome in which the hypertension seemed linked to a stenosis of the left renal artery and which has been treated surgically. A year later the result is satisfactory but the evolutive potential of arterial lesions described in this syndrome does not necessory lead to a final recovery. Besides there are in this disease hypertensions which are relevant to an other etiology (more especially aortic hypoplasia) and stenosis of renal arteries without hypertension.
The authors report a case of horseshoe kidney responsible for hypertension through a one-sided renal dysplasia. The part of the renal malformation in the development of the hypertension is attested by the activities of renin from renal veins, by the isotopic nephrogram and by the disappearance of the hypertension following the heminephrectomy. The hypertension seems an exceptionnal complication of the horseshoe kidney, which can be surprising given the frequency of this malformation and associated urologic anomalies.
The authors relate two cases of idiopathic hypertrophic subaortic stenosis detected after orthostatic hypotension with syncope. This type of detection has not been described till now. Relations between idiopathic hypertrophic subaortic stenosis and orthostatic hypotension are debated as well as relation between IHSS and systemic hypertension which has existed previously in the two cases. It seems suitable to call up IHSS systematically before every orthostatic hypotension at least in elderly. Beta-blockers can in such cases considerably improve orthostatic symptoms.
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Ventricular arrhythmias were looked for in 35 patients with mitral valve prolapse. Their frequency is estimated to 35% in the 31 cases studied prospectively. Ventricular tachycardia is observed in 23% of them. Two patients in emergency had to undergo an external electric shock, one of them for ventricular fibrillation. The interest of different methods of detection and investigation of arrhythmia is questioned. Ventricular ectopic depolarizations are usually detected in the first routine ECG when arrhythmia is severe, but the degree of arrhythmia is generally underestimated. The 24 hour electrocardiographic ambulatory monitoring appears as the choice method but it might be inefficient as shown in some observations. In the latter cases, the exercise testing appears as an indispensable complement for the evaluation and long term follow-up of ventricular arrhythmias in mitral valve prolapse.
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