Intramyocardial distribution of hydroquinidine in the dog.

Hydroquinidine concentrations were measured simultaneously in the plasma and in right atrial and ventricular biopsy samples of four dogs in the steady state after eight days of oral sustained release hydroquinidine administration. The right ventricular concentrations were greater than the plasma concentrations in all samples (ratio 5.02(2.2)). In necropsy samples the ventricular concentrations were higher than the atrial concentrations, (ratio 1.54(0.36); p less than 0.01) and than the concentration in the sinus node region (7.72(1.85) ng.g-1 vs 4.21(2.6) ng.g-1). This study shows that intramyocardial pharmacokinetic measurements are possible and may help towards a better understanding of antiarrhythmic agents, particularly those with cumulative myocardial effect.

Blastogenesis dominant 1: a sequence with midline anomalies and heterotaxy.

Lateralization defect is a heterogeneous condition with different modes of transmission (autosomal recessive, dominant or X-linked). Here, we report on 3 additional families that contribute to the description of phenotypic anomalies of the autosomal dominant type. Phenotypic anomalies include: lateralization defects, cardiac malformations, diaphragmatic hernia, urologic and neurologic anomalies. We suggest calling this sequence BGD1 for blastogenesis dominant 1 because the deleterious effect probably occurs during blastogenesis and involves not only lateralization but other defects as well.

Comparison of myoglobin and creatine kinase MB levels in the evaluation of myocardial injury after cardiac operations.

Myoglobin was studied in 40 patients before, during, and after cardiac operations and was compared to the MB isoenzyme of creatine kinase to identify its possible role as a marker of perioperative myocardial damage. Myoglobin reached peak values during cardiac arrest in all patients and was significantly higher immediately after administration of the anesthetics, during cardiac arrest, and until the sixth postoperative hour in eight patients with a perioperative myocardial infarction. By contrast, the MB isoenzyme of creatine kinase reached peak values at the fourth postoperative hour and was significantly higher in patients with perioperative myocardial infarction from the fourth to the tenth postoperative hours. We conclude that myoglobin is a valuable marker of perioperative myocardial damage and is an earlier and more specific marker of perioperative myocardial infarction than creatine kinase MB.

Myosin: a highly sensitive indicator of myocardial necrosis after cardiac operations.

Plasma levels of ventricular myosin fragments, determined with monoclonal antibodies to myosin heavy chains, were studied in 27 patients after cardiac operations (17 aorta-coronary bypass grafts and 10 valve replacements) to assess their possible role as a marker of perioperative myocardial necrosis. Five patients had perioperative myocardial necrosis after aorta-coronary bypass grafts as indicated by changes in the electrocardiogram and elevated levels of the MB isoenzyme of creatine kinase. Six more patients were also studied after thoracic operations performed by the same sternotomy approach. After cardiac operations, myosin levels increased from postoperative day 3 and reached peak values on day 7. Peak myosin values in patients with perioperative myocardial necrosis after aorta-coronary bypass grafting were significantly higher than in patients after an identical operation but without perioperative myocardial infarction (3793 +/- 592 versus 369 +/- 47 ng/ml; p less than 0.001). These results suggest that plasma myosin is a sensitive marker of myocardial necrosis. Furthermore, peak plasma levels of ventricular myosin after coronary bypass grafting without myocardial infarction (mean value 369 +/- 47 ng/ml) were not significantly different from peak levels after thoracic operations (mean value 253 +/- 52 ng/ml), whereas they were significantly higher after valve replacement (mean value 794 +/- 149 ng/ml; p less than 0.01). These results indicate that a certain degree of myocardial necrosis occurs during value replacement that is undetectable by the usual diagnostic criteria for perioperative myocardial infarction. We conclude that the plasma level of ventricular myosin fragments is a more specific and accurate marker of perioperative myocardial necrosis than changes in the electrocardiogram or elevated creatine kinase MB levels. Therefore the detection of myosin fragments, which appear in the serum on the third day after cardiac operations, may be useful for precise comparisons of different techniques of myocardial protection.

Myocardial lactate dehydrogenase subunit ratio in cardiac allograft recipients.

BACKGROUND: Allograft coronary artery disease (CAD) is a major long-term complication in heart transplanted patients. However, the metabolic basis of allograft CAD remains to be fully elucidated. We analyzed the lactate dehydrogenase heart (H) and muscle (M) isoenzyme pattern in endomyocardial biopsy specimens and the evolution of the H/M ratio to test whether changes in this ratio could be the earliest manifestation of allograft CAD. METHODS: Twenty-four heart transplant recipients were followed up for 12 months. Endomyocardial biopsy was performed at 1, 2, 3, 6, and 12 months after transplantation. Lactate dehydrogenase 1 through 5 isoenzymes were separated by electrophoresis, and the H/M ratio was calculated. Two groups of patients were identified: group 1 (n = 20), patients without allograft CAD; and group 2 (n = 4), patients with poor outcome (three deaths, 1 case of low cardiac output) and angiographic and histologic evidence of allograft CAD. RESULTS: Both groups had similar H/M baseline values. The H/M ratio was higher (p = 0.01) in group 1 at 6 months (3.48 +/- 0.64 versus 2.17 +/- 0.43) and 12 months (3.76 +/- 0.92 versus 2.18 +/- 0.45) when compared with group 2. The H/M ratio increased from 2.78 +/- 0.89 at 1 month to 3.76 +/- 0.92 at 12 months (p = 0.02) in group 1 and decreased in group 2 (2.86 +/- 0.49 versus 2.18 +/- 0.45; not significant). CONCLUSIONS: Changes in H/M ratio reflect an anaerobic shift in the lactate dehydrogenase isoenzyme composition and can be taken as an early indicator of allograft CAD.

Inaugural intra-atrial neoplastic thrombosis in stage II Hodgkin's disease.

We report the first case of intra-atrial neoplastic thrombosis discovered in the initial stage of Hodgkin's disease. Considering the course of the case and the recurrence after 18 months we emphasize the necessity of screening cardiac cavities by CT Scan with a bolus, and of treating such a case as an initial stage IV Hodgkin's disease.

[Cardiotoxicity of anthracyclines]. / Cardiotoxicité des anthracyclines.

The only factor limiting the use of anthracyclines (very powerful antimitotic antibiotics) is their cardiotoxicity. The cardiac involvement is irreversible, dose-dependent and may be detected at an early stage by non-invasive (echocardiography, myocardial scintigraphy) or invasive investigations (endomyocardial biopsy). The mechanism of cardiac toxicity involves the oxidative metabolism of the cardiac myocyte and liberation of oxygen free radicals and is different from the antitumoral effect. Protocols of administration over 6 to 24 hours have enabled the use of higher total doses and the reduction of cardiotoxicity without affecting the therapeutic efficacy. The use of "antioxidants" such as ICRF 187 has given promising results in myocardial protection. The strategy of surveillance (screening) of cardiotoxicity, of the mode of administration of the anthracyclines, results from the essential coordination of the efforts of the chemotherapist, taking into account the pathology, the sensitivity of the tumor to anthracyclines therapy, enabling personalization of the prescription and the abandon of the concept of maximal dosage.

[Long-term course of patients with Smeloff-Cutter aortic valve prosthesis]. / Evolution à long terme des malades porteurs de prothèse de Smeloff-Cutter en position aortique.

The long-term results (5 to 12 years) of 77 patients with Smeloff-Cutter aortic valve prostheses are reported. These patients were comparable in age, preoperative clinical condition and type of aortic valve replacement. The postoperative follow-up period of this series was however significantly longer. The 5 year survival rate was of 87%. The causes of death included thromboembolism, infectious endocarditis and cardiac failure. Mortality was higher in the first 5 postoperative years : 2,4% patient-years compared to 1,5% patient-years in the following years. Thromboembolism and neurological complications were particularly rare, representing a risk of 1,04% patient-years but these complications were lethal in half the cases in which they occurred. No haemolytic complications were observed. Infective endocarditis always occurred in patients with a history of infection, the complication usually being late (after 3 years). The life expectancy of patients seen after 5 years is at least 5 additional years in 80% of cases. The haemodynamic profile of the prosthesis did not degrade with time. After 5 years, 58,6% of patients in functional Class IV at operation were in Class I or II afterwards. 84,4% of patients operated in functional Class III were in Class I or II, and 93% operated in functional Class II were in Class I or III. A control of 3 prostheses carried out by the Cutter laboratory after 10 and 11 years' function shows practically no deterioration of the prosthesis. The low incidence of thromboembolism, the absence of haemolysis and long-term deterioration of the prosthesis are particularly valuable characteristics of this prosthesis in the aortic position.

[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).

[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.

[New indications for cardiac pacing]. / Les indications nouvelles de la stimulation cardiaque.

New indications have recently appeared for cardiac pacing with haemodynamic and antiarrhythmic objectives without any symptomatic bradycardia. The best documented indication, though relatively rare, is stimulation of obstructive hypertrophic cardiomyopathy; initially reserved for cases with favorable results of an acute haemodynamic test, it is now used in other cases without this criterion; hypertrophic cardiomyopathy without permanent obstruction, atrial fibrillation or left bundle branch block. The improvement observed during follow-up is always greater as a real remodeling of the myocardium seems to occur with ventricular dilatation and/or septal thinning. However, the position of the atrial, and above all, of the ventricular pacing catheters is critical as is regulation of the pacemaker which should allow complete ventricular capture with an AV delay allowing good filling. The follow-up of these patients must therefore be regular and the effects on longevity are unknown. DDD pacing has also been proposed in dilated cardiomyopathy. The results are contradictory and only very selected cases with left bundle branch block and long PR interval seem justified with, again, optimisation of the pacing sites with high septal or biventricular stimulation. Recurrent atrial tachycardia, special algorithms preventing extrasystoles have been tried with variable results. In cases with inter-atrial block, atrial resynchronisation by bi-atrial stimulation has been assessed with promising results but many technical problems remain unsolved.

[Vasovagal syndromes]. / Syndromes vasovagaux.

Most cases of dizziness or syncope referred to the emergency department or to services of internal medicine are caused by vasovagal syndromes. They comprise relative bradycardia with vasoplegia, the cardiovascular response to a neurological stimulus. It is possible to distinguish vagal or vasovagal syncope which is very common, the very stereotype reflex syncopes, carotid sinus hypersensitivity sometimes associated with sinus node dysfunction and borderline forms such as orthostatic sinus tachycardia and cerebrovascular syncope. The differential diagnosis is vast, from simple hysteria to severe cardiac disease. Tilt testing should be indicated for diagnosis of most cases of syncope with apparently normal hearts. Therapeutic abstention is the rule, providing certain preventive measures are taken, but, should treatment be necessary, cardiac pacing remains an exceptional modality in vasovagal syncope. Strict clinical and physiopathological studies are still required to determine the long-term prognosis and the underlying mechanisms of these syndromes.

[Curable asystole in an adult caused by a diffuse and complete arteriovenous aneurysm of the liver. Cure by ligation of the hepatic artery]. / Asystolie curable de l'adulte par anévrysme artérioveineux diffus et total du foie. Guérison par ligature de l'artère hépatique.

A case of cardiac failure in a 54 year old man with a diffuse total arteriovenous aneurysm of the liver is reported. The aneurysm was arteriolarvenous, without a wide bore localised fistula. It occupied the whole hepatic mass, including the territories of the right subphrenic and anterior collateral of the gastroduodenal artery. The cause of cardiac failure was the elevated cardiac output, 15 1/min, which fell to 6 1/min at each peroperative occlusion of the hepatic artery. After ligature of this vessel, the cardiac output stabilised at 9 1/min and remained at this value 4 months after surgical cure. This case is comparable to the neonatal cardiac failure due to multinodular hepatic angioma with respect to the clinical, angiographic and CAT scan characteristics, and the surgical cure of the high cardiac output syndrome. Some of the histological features were suggestive, in places, of the structure of hepatic cavernoma, which makes the exact pathological classification of this diffuse aneurysm difficult; the closest possibility being an involuted from of diffuse capillary hemangioma of childhood allowing such long survival and, occasionally taking on the appearances of a cavernoma. Permanent surgical cure by ligature of the hepatic artery was an additional rare feature of this case.

[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".

[Congenital arteriopathies and Leo Buerger's disease]. / Artériopathies congénitales et maladie de Leo Buerger.

A homogenous series of 7 consecutive cases of Buerger's disease were investigated and the congenital malformations of the upper and lower limb arterial trunks were classified. These malformations constitute the anatomical basis of Buerger's disease and define an autonomous disease entity. Total supra-malleolar interruption of the main lower limb arteries either by triple vessel agenesis or, more rarely, by femoro-popliteal or popliteal and tibio-peronal agenesis was observed. The appearances of the collateral circulation, already established in utero, are characteristic with long axial spiral-shaped arteries and dependant arterioles maintaining a precarious supply to the distal tissues which may be adequate for many years. The nautral history of the disease is directly related to the malformation of the congenital arterial system of the limbs. Proximal arterial malformative occlusion is the cause of early, inevitable trophic lesions; the spiral-shaped arteries and distal arterioles, which represent the maximal embryonic collateral circulation, limit the area of necrosis and favour healing.

[Arterial malformations and Takayashu disease]. / Malformations artérielles et maladie de Takayashu.

The authors report a recent series of 5 cases with Takayashu's disease and discuss the common pathological presentation of malformations of the arterial trunk, caused by disorderd development of the arterial system or by later segmental arrested development of the large trunks. The disease, which presents in the young adult as a diffuse inflammatory process, is probably auto-immune, but has its foundations in embryonic and foetal development.

[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.

[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.

[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.

[Effect of cardioversion of atrial fibrillation on left ventricular function in dilated cardiomyopathy. A multicenter study]. / Effet de la réduction de la fibrillation auriculaire sur la fonction ventriculaire gauche des cardiomyopathies dilatées. Etude multicentrique.

A group of 73 patients with idiopathic dilated cardiomyopathy were followed up for an average of 22 +/- 7 months to assess the medium term evolution of echocardiographic parameters of left ventricular function and, in particular, the consequences of cardioversion of atrial fibrillation. Seventy nine per cent of patients presented with cardiac failure. Left bundle branch block was observed in 20% and ventricular arrhythmias were frequent in 31%, complex in 62% with episodes of non-sustained ventricular tachycardia in 10% of cases. Left ventricular dilatation was greater in patients with complete left bundle branch block (p less than 0.003). Atrial fibrillation was present in 14 patients (19%) who were generally older than the rest of the study population (p less than 0.02) and was associated with less severe left ventricular dysfunction (p less than 0.01). Return to sinus rhythm was obtained in 9 patients. Echocardiographic data was obtained in 64 patients after an average of 6.2 +/- 1.7 months. Left ventricular function improved during the follow-up period and returned to normal in 12% of cases. Reduction of atrial fibrillation to sinus rhythm was the only predictive factor of normalisation of left ventricular function (p less than 0.02). The changes in left ventricular end diastolic dimension and fractional shortening was less marked in the group of 56 patients in sinus rhythm or chronic atrial fibrillation (normalisation of left ventricular function in 8% of cases) than in the group of 8 patients in which atrial fibrillation was converted to sinus rhythm (normalisation of left ventricular function in 50% of cases).(ABSTRACT TRUNCATED AT 250 WORDS)