[Continuous nodal reciprocal rhythm and Steinert's disease: treatment by double chamber stimulation. A case report]. / Rythme réciproque nodal incessant et maladie de Steinert: traitement par stimulation double chambre. A propos d'un cas.

We report a case of continuous supraventricular tachycardia in a patient affected by Steinert's myotonic dystrophy. The investigation of this tachycardia showed that there was a "slow-fast" common nodal re-entry, rendered continuous by the existence of significant conduction defects in the fast pathway and the slow anterograde pathway. Implantation of a double chamber cardiac stimulator, necessary for conduction defects present in the basal state in this patient allowed, with the evolution of the conduction defects, the complete eradication of reciprocal rhythm entry, without resorting to ablation.

A "dysautonomic" head-up tilt test pattern in elderly patients with neurocardiogenic syncope.

The characteristics of neurocardiogenic syncope (NCS) in elderly patients remain unclear. We compared the hemodynamic profiles of young and older patients with consecutive and positive head-up tilt tests (HUT). Continuous, noninvasive, and reliable monitoring of arterial pressure (AP) and heart rate (HR) was done throughout 46 consecutive positive HUTs of symptomatic patients. The population (12-82 years old) was divided into two groups: younger patients, Y (n = 25, < or = 65 years), and older patients, O (n = 21). Changes in AP and HR after the first minute of tilting, during the stable orthostatic phase and during syncope were compared. Except for systolic pressure, baseline hemodynamic parameters were similar in Y and O. No difference appeared in the mean time elapsed before syncope (19+/-9 vs 22+/-2 min). Asymptomatic hypotension was observed, only in O, 1 minute after tilting, followed by a progressive fall in the mean AP before syncope (0+/-0.9 vs -1+/-0.7 mmHg/min) without HR increase (0.7+/-1 vs 0+/-0.6 beats/min). This pressure slope was strongly related to age (r = 0.54, P < 0.001). Hemodynamic recording during HUT identifies a dysautonomic pattern in elderly patients with NCS and the abnormal AP/HR responses to orthostasis may be a feature specific to this population. Although the central mechanism of NCS is common to all ages, the age-related characteristics of the trigger event may indicate the need for specific management at different ages.

[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.

Chronic left main coronary artery occlusion: a complication of radiofrequency ablation of idiopathic left ventricular tachycardia.

We describe in this report the development of chronic left main coronary artery (LMCA) occlusion in a young patient 2 years after an uncomplicated, successful ablation of idiopathic left ventricular tachycardia. This complication appears to be a late consequence of trauma to the LMCA during the procedure rather than an acute or subacute embolic event.

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.

[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.

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.

[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.

[French familial multicenter survey of hypertrophic cardiomyopathy. Initial Doppler echocardiographic results]. / L'enquête multicentrique familiale française de la cardiomyopathie hypertrophique. Résultats échocardiographiques Doppler préliminaires.

A French multicentre study of hypertrophic cardiomyopathy has recruited 260 subjects belonging to 18 families. At least 3 persons from each family included had a hypertrophic cardiomyopathy. A Doppler echocardiographic examination was performed in all members of these 18 families. The diagnosis of hypertrophic cardiomyopathy was based on M mode and/or 2D observations of parietal hypertrophy: wall thickness over 13 mm. Asymmetrical forms were distinguished from symmetrical forms by a septum/posterior wall ratio of over 1.3 in M mode or 2D study. The patients had an average age of 40 +/- 18 years, 127 were men and 133 women. One hundred and sixty eight were considered to be normal, 87 had hypertrophic cardiomyopathy and 5 were border line. The maximal diastolic wall thickness on M mode recording of pathological cases was 19.2 +/- 4.8 mm compared with 9.5 +/- 1.9 mm in healthy subjects (p < 0.001). The septum/posterior wall ratio was 1.8 +/- 0.62 in pathological cases and 1.1 +/- 0.8 in normal ones (p < 0.001). Eighty five per cent of the cardiomyopathic cases were asymmetrical (74/87) and only 15% were considered to be symmetrical (13/87) on M mode study whereas 5% were concentric on 2D echocardiography. By Maron's classification, type I hypertrophy was observed in 6% of cases (4/64), type II in 30% (19/64), type III in 58% (37/64), and type IV in 6% (4/64). Signs of obstruction were looked for and systolic anterior motion of the mitral valve was observed in 52% of pathological cases (45/86) and mid-systolic aortic valve closure in 30% (25/83).(ABSTRACT TRUNCATED AT 250 WORDS)

Detection of enteroviruses in endomyocardial biopsy by molecular approach.

Enteroviruses are considered to be the most common agents implicated in myocarditis and cardiomyopathy. Recent studies have suggested persistent enterovirus infection in chronic disease showing the presence of enteroviral RNA in the myocardium. We used gene amplification by PCR which can demonstrate directly the presence of enteroviral sequences in endomyocardial biopsies. The primers were chosen in the 5' non-coding region of the genome representing highly conserved sequences among enteroviruses and therefore allowed the amplification of the majority of enteroviruses. The hybridization of the amplified products was effected with specific general riboprobe derived from 5' non-coding sequences internal of the amplified fragments. The results include 105 patients distributed in 6 groups: 45 idiopathic dilated cardiomyopathies with 66.7%, 17 alcoholic cardiomyopathies with 52.9%, 10 myocarditis with 30%, 5 multifactorial cardiomyopathies with 40%, 5 patients with immunosuppressive therapy with 100%, and 23 control group without viral etiology with 39.1% positive samples. The study suggested a positive link between viral infection and cardiomyopathies, but did not allow a direct relation between enterovirus infection and idiopathic dilated cardiomyopathy to be established.

[Cardiomyopathy in the peripartum period: current aspects. A multicenter study. 11 cases]. / La myocardiopathie du péripartum: aspects actuels. Etude multicentrique: 11 observations.

Eleven of 17 case histories recensed after an inquiry addressed to all the French university hospital cardiological departments were attributed to peripartum cardiomyopathy. Three patients (27%) needed cardiac transplantation. Predisposing factors were multiparity and twin pregnancies. Forty five per cent of patients were under 25 years of age. Hypoproteinemia was present in all serious forms of the condition. Hypoproteinemia, a cardio-thoracic ratio of over 0.60, echocardiographic left ventricular fractional shortening of under 15 per cent and presistance of symptoms after one month of medical treatment were poor prognostic factors. The relatively high incidence of myocarditis (1 out of 11 cases) justifies endomyocardial biopsy.

Effects of ACE-inhibition on glucose metabolism.

ACE inhibition is widely used for treatment of arterial hypertension or congestive heart failure. No change occurs in glucose metabolism either in diabetic or non diabetic subjects. No change occurs in glucose metabolism in patients with chronic renal failure. Glucose intolerance induced by diuretics is attenuated when ACE inhibitor is associated with thiazides. In some very rare circumstances (with high plasmatic levels of norepinephrine), insulin sensitivity seems to be enhanced by captopril. Then, in clinical use, no adverse effect occurs with ACE inhibition in non diabetic or diabetic subjects. Under thiazide treatment, ACE inhibitors protect against glucose intolerance.

[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)

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.

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.

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.