Primary malignant fibrous histiocytoma of the right ventricle and main pulmonary trunk with a review of the literature.

We report the case of a 76-year-old man with a malignant fibrous histiocytoma of the right ventricle and main pulmonary trunk, diagnosed through echocardiography and catheterization. Extensive resection of the right ventricular outflow tract, pulmonary valve apparatus, and main pulmonary trunk was performed, and the defect was repaired with a valveless ventriculo-pulmonary Dacron graft. The patient recovered uneventfully, and was doing well 18 months after surgery. To our knowledge, this is only the 15th case of primary malignant fibrous histiocytoma of the heart that has been documented in the literature since histologic criteria and cases were published in 1977-78, and the 2nd such case of a primary tumor that has arisen in a right cardiac chamber. The case is presented in detail, along with a review of the literature since 1978.

[Clinical, angiographic aspects and 18-month follow-up of 134 cases of left coronary trunk stenosis]. / Caractères cliniques, angiographiques et suivi à 18 mois de 134 cas de sténoses du tronc commun de la coronaire gauche.

A series of 134 patients with left main coronary stenosis was followed up for an average of 18 months. The majority of patients presented with unstable angina and per-critical ECG changes. Coronary angiography showed that left main coronary stenosis is rarely an isolated entity but generally associated with diffuse coronary atherosclerosis. Left ventricular function is usually normal. Treatment is surgical whenever the coronary lesions, left ventricular function and general condition of the patient allow it. The medium-term surgical results are excellent on clinical symptoms and quality of life at the price of low perioperative mortality and morbidity.

[Evaluation of ventricular tachycardia by endocavitary stimulation. Apropos of 46 cases]. / Evaluation des tachycardies ventriculaires par la stimulation endocavitaire. A propos de 46 cas.

Thirty six patients (21 coronary artery disease, 8 cardiomyopathy, 3 mitral valve prolapse and 4 apparently normal) underwent endocavitary stimulation studies. The protocol consisted in delivering one or two right ventricular extrastimuli and twice the diastolic threshold either during spontaneous rhythm (S2 and S2-S3) or a paced ventricular rhythm (S1-S2 and S1-S2-S3). In 9 patients overdrive ventricular pacing at 150-200 bpm was required. Thirteen of the 15 documented sustained ventricular tachycardias could be induced by electrical stimulation (87%). In addition, 9 sustained ventricular tachycardias were induced in patients in whom the symptomatology corresponded to poorly tolerated tachycardia but in whom the documented arrhythmia was non-sustained ventricular tachycardia (4 cases), frequent ventricular extrasystoles with doublets (2 cases) or rare, isolated ventricular extrasystoles (3 cases). The method was less sensitive in non-sustained ventricular tachycardia in which the arrhythmia was induced in 10 of the 17 cases (59%). An antiarrhythmic drug was selected on the basis of these studies in 28 patients, 21 of whom had sustained ventricular tachycardia. There were 6 therapeutic failures with a follow-up of 6 to 24 months, three of which were observed in patients with coronary artery disease and a precarious haemodynamic state. This subgroup is not suitable for this type of evaluation. After reviewing other published series the authors emphasise the value of these investigations in chronic sustained ventricular tachycardia, in ventricular tachycardia with cardiovascular collapse and in the assessment of syncope of unknown origin. However, the systematic investigation of repetitive ventricular responses after ventricular stimulation in patients at high risk of sudden death remains controversial.

[Electrophysiological effects of encainide in man]. / Effets électrophysiologiques de l'encaïnide chez l'homme.

Encainide is an antiarrhythmic agent under evaluation; it is effective in ventricular and supraventricular arrhythmias. Its electrophysiological effects seem to differ according to the route of administration, oral or intravenous, probably because of the effects of active metabolites. Two electrophysiological studies were carried out in 20 patients, under basal conditions, and after 4 to 10 days oral administration at doses ranging from 75 to 300 mg/day. Encainide depressed intra-atrial conduction (prolongation of the P-A interval from 29,7 +/- 2,2 to 36 +/- 4,5 ms, p less than 0,01), slowed conduction in the atrioventricular mode (prolongation of the A-H interval from 74 +/- 14 to 98 +/- 15 ms, p less than 0,01) and the His-Purkinje system (lengthening of H-V from 50 +/- 3 to 70 +/- 6,2 ms, p less than 0,001). The sinus node function was depressed with lengthening of the corrected sinus node recovery time (297 +/- 64 to 387 +/- 71 ms, p less than 0,01) and of the sinoatrial conduction time (173 +/- 25 to 219 +/- 43, p less than 0,01). The atrial and ventricular refractory periods were significantly longer (245 +/- 16 ms to 273 +/- 10 ms, p less than 0,001, and 237 +/- 12 to 266 +/- 19 ms, p less than 0,01, respectively). This new antiarrhythmic agent therefore seems to act at all levels which suggests that it may have wide ranging antiarrhythmic effects.

[Value of provocation tests in the evaluation of the treatment of ventricular tachycardias with amiodarone]. / Intérêt des tests de provocation dans l'appréciation du traitement des tachycardies ventriculaires par l'amiodarone.

The role of provocation tests for the assessment of amiodarone therapy in patients with ventricular tachycardia remains a subject of controversy: recent studies seem to show that the ability to initiate VT in patients on amiodarone is compatible with a good long-term result. Eighteen patients, 16 male and 2 female, average age 56 years, were treated with amiodarone (600 mg/day in 15 cases, and 400 mg/day in 3 cases) and submitted to provocative tests before and after treatment. The mean follow-up period was 14 +/- 4 months. In Group I (5 patients), VT could not be initiated after treatment and there were no relapses of the arrhythmia. In Group II (6 patients), non-sustained VT could be initiated and only one relapse was observed after a close reduction from 600 to 400 mg/day; Group III comprised 5 patients with spontaneous recurrences. An identical VT could be initiated during electrophysiological investigation which served as a basis for selection of an effective antiarrhythmic association. Two patients could not be studied after drug impregnation (1 sudden death, 1 exacerbation of VT). The results of this study show that provocative pacing can be useful in evaluating the efficacy of amiodarone, as in Groups I and II (61% of patients) a favourable prognosis could be predicted in 91% of cases. In cases of therapeutic failure with spontaneous recurrences of VT, the same provocation tests enabled a more effective drug combination to be selected.

[Malignant fibrous histiocytoma of the right ventricle and pulmonary artery. Apropos of a case. Review of the literature]. / Histiocytome fibreux malin ventriculaire droit et de l'artère pulmonaire. A propos d'un cas. Revue de la littérature.

The authors report a case of malignant cardiac fibrohistiocytoma involving the right ventricular infundibulum and the pulmonary artery and responsible for a pseudo-thromboembolic right heart failure syndrome. Following discovery of the tumour mass on ultrasonography and catheterisation, wide excision of the pulmonary artery, pulmonary valve and the roof of the infundibulum as far as the middle part of the ventricle was performed under cardio-pulmonary by-pass. Reconstruction was performed with a valveless woven Dacron tube. The postoperative course was uncomplicated and the patient was still alive after 18 months. Histology revealed malignant fibrohistiocytoma. The authors also present a review of the literature of these rare tumours of the right-sided cavities (5 cases reported).

[Oral propafenone in resistant auricular arrhythmia]. / Etude de la propafénone orale dans les arythmies auriculaires rebelles.

Paroxysmal episodes of atrial frequently cause severe functional disturbance because of their recurrent nature. Propafenone (Rythmol) is a very active anti-arrhythmic at the ventricular level which acts by decreasing the rate of atrio-ventricular and intra-ventricular conduction and by prolonging the refractory period of the right atrium and the accessory pathways. The authors conducted an open study of this drug in 20 cases with resistant, recurrent atrial fibrillation. All of the patients were known to have recurrent episodes of atrial fibrillation which could not be prevented by a variety of antiarrhythmic agents. They performed a clinical, electrocardiological and laboratory evaluation of these patients. Holter monitor recordings were performed prior to entry into the study, during the first week of treatment, between the 4th day and the 8th day, on the 20th day, at the 2nd month and between the 3rd and 6th months. Propafenone was prescribed at a dose of 900 mg per day and the initial dose was reduced to 600 mg after the 3rd month of treatment. Five patients can be classified as therapeutic failures, as the arrhythmia recurred. These patients presented a "vagal" atrial fibrillation preceded by an episode of bradycardia. 15 patients can be considered to have obtained a successful result, as no recurrences were detected during the 6 month observation period. The electrical and laboratory tolerance was satisfactory. The most frequent side effects were minor transient gastrointestinal disturbances.

[Topographic aspects of threatened extension of transmural myocardial infarction. Electrocardiographic and coronarographic data]. / Aspects topographiques de la menace d'extension d'infarctus du myocarde transmural. Données électrocardiographiques et coronarographiques.

The topographical and physiopathological aspects of the danger of extension of infarction of the myocardium are defined with the aid of data collected by electrocardiogram and coronarography in 50 patients. The danger of extension in situ, observed in 64% of the cases, is the most frequent and complicates particularly the progression of anterior infarcts. In fact, it is located in the same area as the initial infarct in 91% of the cases for anterior infarcts and in 40% of the cases for inferior infarcts. It is expressed by an elevation of the ST segment in 84% of the cases and corresponds to a monotruncular attack in 63% of the cases. The downstream bed of the vessel destined for the infarcted area and threatened secondarily remains permeable in the anterograde sense. Apart from infarcts, the danger of extension is less frequent, found in 36% of the cases, and complicates preferentially the progression of inferior infarcts. It finds expression in a depression of the ST segment in 77% of the cases and the coronary attack is always pluritruncular. The mortality in one month is 35% of 17 patients treated medically and 3% of 33 patients who have been equipped with a shunt or angioplasty. In situ the danger of extension denotes the presence of cellular islets, which are still healthy, in the region of an infarcted myocardial zone, the viability of which may be threatened secondarily by a phenomenon of coronary occlusion, which is intermittent and repeated. Except for an infarct, the danger of extension implies the diffusion of an atheromatous effect. The good results of surgical treatment or dilatation argues in favour of an early coronarographic exploration.

[Effects of amrinone administered orally and by injection in heart failure]. / Effets de l'amrinone utilisée par voie orale et injectable dans l'insuffisance cardiaque.

Amrinone is a new positive inotropic agent available in oral and intravenous preparations. Twelve patients with Stage III cardiac failure of ischaemic (6 cases), myocardial (5 cases) or valvular (1 case) origin, were treated with oral amrinone. The protocol included a complete clinical, radiological and biochemical work-up, an exercise stress test, cardiac catheterisation and echocardiography before entering the trial. The patients underwent clinical examination, stress testing and echocardiography at the 4th, 8th and 12th week of treatment with 300 mg daily of amrinone. Two patients had to be withdrawn from the trial because of thrombocytopaenia; one patient deteriorated and eventually died of pulmonary embolism. There was a marked improvement in the 8 patients who achieved the trial, with an average gain of 40 watts on exercise testing, a mean reduction of 16 mm Hg in diastolic pulmonary pressures, and an increase of 11 p. 100 in EF and velocity of circumferential fibre shortening. Four additional patients were given intravenous amrinone (1 cc/kg relayed with an infusion of 1 ng/kg/min). Ventricular end-diastolic pressures fell by 9 mm Hg and cardiac index rose by 1.02 1/min/m2. Tolerance was good with no arrhythmic complications or significant variations in mean arterial pressure or heart rate. Although certain reserves have to be made with regards of tolerance of oral amrinone, the drug would seem to be useful and effective in the intravenous form. Further studies are under way.

[Malignant fibrous histiocytoma of the right ventricle and pulmonary artery. Apropos of a case. Review of the literature]. / Histiocytome fibreux malin ventriculaire droit et de l'artère pulmonaire. A propos d'un cas. Revue de la littérature.

The authors report a case of malignant cardiac fibrohistiocytoma involving the right ventricular infundibulum and the pulmonary artery and responsible for a pseudo-thromboembolic right heart failure syndrome. Following discovery of the tumour mass on ultrasonography and catheterisation, wide excision of the pulmonary artery, pulmonary valve and the roof of the infundibulum as far as the middle part of the ventricle was performed under cardio-pulmonary by-pass. Reconstruction was performed with a valveless woven Dacron tube. The postoperative course was uncomplicated and the patient was still alive after 18 months. Histology revealed malignant fibrohistiocytoma. The authors also present a review of the literature of these rare tumours of the right-sided cavities (5 cases reported).