[Right intraventricular biopsy. Indications and results. 116 cases]. / Biopsie endoventriculaire droite. Indications et résultats. Cent seize observations.

The indications for endomyocardial biopsy were evaluated from 116 consecutive cases. The diagnostic value of this invasive but well tolerated procedure was in agreement with data from the literature. An accurate diagnosis, unforseeable in 8% of the patients, was established in 12%. The diagnosis of apparently primary myocardiopathy with ventricular dilatation was confirmed in 45 out of 59 cases; there were 3 cases of myocarditis, 3 cases of restrictive cardiopathy (haemochromatosis, fibroplastic endocarditis) and 1 case of hypertrophic cardiopathy. No tissue abnormality was noted in 6 cases. An accurate diagnosis was obtained by biopsy in 1 case of "eosinophilic lung" without overt cardiac involvement. In malignant diseases treated with anthracyclines in doses reaching maximal theoretical total dosage (30 patients), severe tissue lesions were present in 10% of the cases, incipient haemochromatosis in 16.6% and subendocardial fibrosis in 3.3%. However, total doses of up to 600 mg/m2 could be administered to 90% of the patients. Myocardial lesions could be demonstrated in 1 of 2 patients with collagen disease. Endomyocardial biopsy therefore seems to be justified in myocardiopathies with ventricular dilatation, in some collagen diseases with a tendency to cardiac involvement and to monitor treatment with anthracyclines in total doses higher than the theoretical maximum dosage.

[Distribution of lactate dehydrogenase isoenzymes in human myocardium obtained from endomyocardial biopsies]. / Répartition des isoenzymes de la lactico-déshydrogénase du myocarde humain obtenu à partir de biopsies endomyocardiques.

The distribution of lactate dehydrogenase and its isoenzymes in myocardial tissue obtained by biopsies from within the right ventricle can be studied by electrophoresis in a similar manner to that for making serum determinations. Results are comparable to radioimmunologic dosing (Peters) and microelectrophoresis (Schultheiss) and complete the findings obtained from the histological study of biopsied fragments.

[Histological aspects of congestive cardiomyopathy caused by alcohol. Comparison with so-called primary cardiomyopathies]. / Aspects histologiques de la cardiomyopathie congestive d'origine èthylique. Comparaison avec les cardiomyopathies dites primitives.

The endoventricular biopsies of nine patients with alcoholic congestive cardiomyopathy were studied under the light microscope and under the electron microscope. These were compared with the results of nine cases of primary congestive cardiomyopathy, at a similar clinical stage. In the absence of specific signs, the overall appearance on both light and electron microscopy is sufficiently suggestive to allow differentiation of the two forms of cardiomyopathy. Repeated biopsies revealed disappearance of the nuclear abnormalities after withdrawal of alcohol, which introduces a prognostic element.

[Right endoventricular biopsy using the femoral percutaneous approach: a simple procedure, harmless, reliable and repetitive. Modification of the Konno technic]. / La biopsie endoventriculaire droite par voie percutanée fémorale: un geste simple, anodin, fiable et répétitif. Modification de la technique de Konno.

The technique of right endoventricular biopsy by femoral percutaneous path is described based on 300 incident-free samples. Ability to multiply and then repeat sampling supports a method which can be performed at the same time as examination of the conduction channels. This is of diagnostic, prognostic, therapeutic and research interest.

[Isolated intimal stenosis of the renal artery and curable arterial hypertension in Recklinghausen's disease. Association of hypoplastic stenosis of the aortic and pulmonary trunks and sigmoid aortic regurgitation]. / Sténose intimale isolée d'une artère rénale et hypertension artérielle curable dans la maladie de Recklinghausen.

The case of a patient with Von Recklinghausen's disease with multiple localisations, cardiac, aortic, pulmonary and renal artery is reported. Neurofibromatosis was confirmed by the presence of a "royal" tumour on the left elbow and of many "café au lait" spots of over 15 mm diameter. There was no craniofacial dysmorphism or intellectual impairment. The karyotype was normal. The cardiovascular lesions comprised: - stenosis of the left renal artery resulting in renovascular hypertension. This was due to pure intimal hyperplasia; - supravalvular aortic stenosis with hypoplasia of the ascending aorta. There was no craniofacial dysmorphism or intellectual impairment. The karyotype was normal. The cardiovascular lesions comprised: - stenosis of the left renal artery resulting in renovascular hypertension. This was due to pure intimal hyperplasia; - supravalvular aortic stenosis with hypoplasia of the ascending aorta. There was no craniofacial dysmorphism or intellectual impairment. The karyotype was normal. The cardiovascular lesions comprised: - stenosis of the left renal artery resulting in renovascular hypertension. This was due to pure intimal hyperplasia; - supravalvular aortic stenosis with hypoplasia of the ascending aorta, severe aortic regurgitation with dilatation of the aortic ring without rheumatic valvular thickening; - supra valvular pulmonary stenosis which was atypical in site, extension and in the absence of post stenotic dilatation. The pressure gradient was mild (22 mm Hg). There was a striking similarity between the pulmonary and aortic lesions. This new syndrome may be classified amongst the genetic cardiocutaneous syndromes such as the Gorlin or "leopard" syndrome, Watson's syndrome and Noonan's syndrome. Unusual features are the absence of craniofacial abnormalities, normal intelligence, and the left-sided dominance aggravated by hypertension due to curable intimal stenosis of the renal artery.

[Kearns' syndrome: a case study (author's transl)]. / Syndrome de Kearns.

An anatomoclinical study of a case of Kearns' syndrome is reported. Neuro-ophthalmic symptoms appeared when the child was 13 year-old. Two and a half years later occurred an episode of paroxystic atrioventricular block, after which the triad characterising the syndrome was completed: retinitis pigmentosa, ophthalmoplegia, disorder of heart conduction. The course was unfavorable despite pacemaker insertion. Study of the central nervous system showed spongiosis of the subcortical white substance, of the basal ganglia and of the cranial nerve nuclei. The specialized heart conduction tissue was the site of apparently primary degeneration. The extension of the visceral involvement is discussed in the light of published data.

[Lymphangioendothelioma. A rare cause of atrioventricular block]. / Lymphangio-endothéliome. Une cause rare de bloc auriculo-ventriculaire.

A new case of lymphangio-endothelioma of the nodal tissue is reported. Complete atrioventricular block had been followed up for 20 years. The histological features are described in detail and the embryological origins of the tumour are discussed. This report underlines the exceptional character of this "smallest benign tumour which can be mortal".

[Cardiological aspect of Kearns' syndrome. Apropos of 3 cases, with histopathological study of the conduction tissue in one of them]. / Aspect cardiologique du syndrome de Kearns. A propos de 3 cas dont 1 avec étude histo-pathologique du tissu de conduction.

Three cases of Kearns syndrome are reported. The neuro-ophthalmological signs were comparable with ophthalmoplegia, pigmentary retinal degeneration and polymorphic neuro-muscular and sensory deficits. The electrocardiological signs were observed 2-4 years after the onset of the condition; the cause of death in each case was related to complete heart block. The post-mortem findings in one of the cases were spongial degeneration of the central nervous system and a seemingly primitive degeneration of the Bundle of His and its branches.

[Arterial and renal parenchymal histological data in the areas on polar and truncal arteries in arterial hypertension of adult patients]. / Données histologiques artérielles et parenchymateuses rénales dans les territoires des artères polaires et tronculaires dans l'hypertension artérielle de l'adulte.

The Cardiac Clinic reports its experience of 16 cases having unilateral curative renal surgery for hypertension in the adult, and has found a particularly high incidence (8 per cent of all cases). A study has been made of the lesions in the malformed and dysplastic renal arteries on the one hand, and of the correspondingly ischaemic and atrophic renal parenchyma on the other. Attention is drawn to the cases of unilateral renal atrophy (50 per cent of cases), the commonest involving gross lymphoplasia of congenital arterial origin. The other cases are of stenotic dysplasias, and special points to notice are the spread of the dysplasia towards the parenchyma, the presence of multiple aneurysms which militate towards nephrectomy, and the relative frequency of dysplasia of the intima (2 cases out of 8).

[Reversible "experimental" human arterial hypertension after fortuitous ligation of an isolated polar renal artery of aortic origin]. / Hypertension artérielle humaine "expérimentale" réversible par ligature fortuite d'une artère polaire rénale autonome d'origine aortique.

During the course of an aorto-femoral bypass in a normotensive young man, an inferior polar artery arising low on the aorta, and supplying the right kidney, was ligated. The limited ischaemia (demonstrated by later arteriography) of the parenchyma at the pole of the kidney was responsible, during the following weeks, for severe hypertension with a raised level of circulating plasma renin, but one that was rapidly treatable. This finding, which was fortuitous but could be ranked as "experimental", demonstrates the important role of abnormal polar arteries and of the renal parenchyma in human arterial hypertension. Such may, for example, be the underlying disorder in cases of hypertension after the transplantation of a kidney with an "uncontrolled" polar artery arising from the aorta, for the so-called "pyelonephritic" hypertension in pregnant patients caused by defective blood supply in an inter-pyramidal artery and corresponding subcortical infarction of the kidney, and for curing hypertension in the adult when isolated polar arteries arising from the aorta and stenosed at their point of origin are reimplanted into the main renal artery.

[Corrected transposition of the great vessels and preexcitation syndrome (apropos of 2 cases)]. / Transposition corrigée des gros vaisseaux et syndrome de préexcitation (à propos de deux observations)

Two cases with treated transposition of the great vessels and incompetence of the left atrioventricular valve showed a type B preexcitation syndrome. In one case, this consisted of a typical W.P.W. syndrome in which the second PR interval was not shortened, but rather consisted of a delta wave and a widened QRS complex. Post mortem examination showed an abnormal connection between the bundle of His and the ventricular septum, and a low insertion of the inverted tricuspid valve. The published cases of W.P.W. syndrome in cases with treated transposition are reviewed, and the mechanism of preexciation discussed in the light of the anatomical peculiarities of the malformation and of the abnormalities which are a feature of Ebstein's syndrome.

[Renal arteries and renal parenchyma in arterial hypertension in pregnancy]. / Artères rénales et parenchyme rénal dans l'hypertension artérielle gravidique

Hypertensives in pregnancy are not so grossly distinct and different from adult hypertensives if reference is made to the underlying arterial anomalies and malformations of the parenchyma and their incidence. It is, however, in this group that the limited way in which the active remnant of parenchyma, once constrained by these anomalies, can compensate for the changes in pressure and volume sensitivity imposed by pregnancy is most evident; these changes may be caused by pyelonephritis of pregnancy (genuine but rare), eclampsia of the primigravida, progressive hypertension in the multigravida, or certain cases of late hypertension, menopausal hypertension, or hypertension as a late result of a simple juvenile eclampsia. This series contains a particularly high percentage (21%) of cases of hypertension due to unilateral renal arterial ischaemia which are curable.

[Right branch of the bundle of His. From morphology to its function]. / Branche droite du faisceau de His. De sa morphologie à sa fonction

A histological study of serial sections of 71 right branches of the bundle of His has enabled us to confirm the classical findings, but also lay emphasis on certain facts which are not so well known: --the presence or early collateral branches; --the concept of a septal nerve ending; --variations in its course associated with abnormalities in the relationship of the conus with the inferior part of the septum. Certain embryological and functional implications of these facts are noted.

[Ischemic lesions of the left branch of the bundle of His. Their relation of left hemiblock]. / Lésions ischémiques de la branche gauche du faisceau de His. Leur rapport avec les hémiblocs gauches

The term "hemiblock of the left branch of the bundle of His" seems to a histologist to be too precise to be accepted without reservation. Ten cases with septal infarction, with or without left intraventricular conduction problems, were studied by serial sectioning of the heart; these are used as a basis for a discussion of the concepts of electrocardiography, and its implications in the prognosis of patients with myocardial necrosis.

[Kidney parenchymatous hypoplasia and arterial dysplasia in adult arterial hypertension. Data of selective renal arteriography]. / Les hypoplasies parenchymateuses et les dysplasies artérielles des reins dans l'hypertension artérielle de l'adulte. Données de l'artériographie rénale sélective

Analysis of a further series of 125 consecutive unselected adults who were admitted to hospital with hypertension has advanced the study of arterial abnormalities and parenchymal hypoplasia, as demonstrated by selective renal arteriography, further in the direction of the parenchyma. An index of arterioparenchymal thinning is described. The authors list the features and incidence of polar arteries arising from the aorta (46%), polar arteries of non-aortic origin (31%), stenosing dysplasia (26%) and other arterial malformations, as well as biapical hypoplasia (67%), monofocal hypoplasia (37%), and the main types of renal dysgenesis (30%) which they found. The incidence of these abnormalities confirms the previous study of polar arteries arising from the aorta, and gives much more extensive information on the topic of parenchymal hypoplasia in so-called essential hypertension in the adult.

[Embryological duplications of the node-bundle of His : its pathological rôle]. / Dualité embryologique des voies nodo-hisiennes : son rôle en pathologie

A histological study by the method of seriated sections in one case of "double-outlet left ventricle" made it possible to confirm and to describe the embryology of the nodal-His junction: not only the atrio-ventricular node (AV N) but also the His bundle (H) have a double origin, atrial and ventricular. Fusion of these two buds, inexistant in the "lone ventricle" is certainly imperfect. Two macroscopically normal hearts provided examples of nodal buds in one case, of a persisting cleft between the two His buds in the other.

Left posterior hemiblock in acute myocardial infarction.

Fifteen cases of left posterior hemiblock associated with acute myocardial infaction were studied. In 5 cases the left posterior hemiblock was the only intraventricular conduction defect, while in the other 10 cases it was associated with complete right bundle-branch block. Left posterior hemiblock proved to be an early complication, appearing within a few hours from the onset of the acute episode, and an ominous sign, since hospital mortality rate was 87 per cent. Cause of death was mainly pump failure. In most of these cases ther was electrocardiographic evidence of infarction involving both anterior and inferior ventricular walls. Infarction of most or all of the ventricular septum was a common finding in the cases examined anatomically. Histologically, acute changes involving mainly the posterior septal and midseptal fibres were observed in 6 of the 8 cases studied. On the basis of these findings and of other published findings an alternative physiopathological mechanism for so-called left posterior hemiblock is proposed.