Is African descent an independent risk factor of peripartum cardiomyopathy?

Risk factors for peripartum cardiomyopathy (PPCM) are controversial. PPCM seems to be more prevalent in women of African descent, the highest observed incidence is in Haiti (1 per 300 live births). Our retrospective study conducted in Martinique showed an incidence of 1 per 5500 live births. This incidence is significantly lower than in Haiti. Women from Martinique and Haiti do not differ for most classical risk factors: African descent, age, pregnancy-associated hypertension, multiple pregnancy and pre-eclampsia. However, the parity rate and the socioeconomic level are different. Thus, African descent could be confounded by high parity rate and socioeconomic status.

Novel SCN5A mutation leading either to isolated cardiac conduction defect or Brugada syndrome in a large French family.

BACKGROUND: The SCN5A gene encoding the human cardiac sodium channel alpha subunit plays a key role in cardiac electrophysiology. Mutations in SCN5A lead to a large spectrum of phenotypes, including long-QT syndrome, Brugada syndrome, and isolated progressive cardiac conduction defect (Lenègre disease). METHODS AND RESULTS: In the present study, we report the identification of a novel single SCN5A missense mutation causing either Brugada syndrome or an isolated cardiac conduction defect in the same family. A G-to-T mutation at position 4372 was identified by direct sequencing and was predicted to change a glycine for an arginine (G1406R) between the DIII-S5 and DIII-S6 domain of the sodium channel protein. Among 45 family members, 13 were carrying the G1406R SCN5A mutation. Four individuals from 2 family collateral branches showed typical Brugada phenotypes, including ST-segment elevation in the right precordial leads and right bundle branch block. One symptomatic patient with the Brugada phenotype required implantation of a cardioverter-defibrillator. Seven individuals from 3 other family collateral branches had isolated cardiac conduction defects but no Brugada phenotype. Three flecainide test were negative. One patient with an isolated cardiac conduction defect had an episode of syncope and required pacemaker implantation. An expression study of the G1406R-mutated SCN5A showed no detectable Na(+) current but normal protein trafficking. CONCLUSIONS: We conclude that the same mutation in the SCN5A gene can lead either to Brugada syndrome or to an isolated cardiac conduction defect. Our findings suggest that modifier gene(s) may influence the phenotypic consequences of a SCN5A mutation.

[Cure of superacute eosinophilic myocarditis treated by temporary circulatory assistance]. / Guérison d'une myocardite suraiguë à éosinophiles traitée par assistance circulatoire temporaire.

The authors report the case of a 31 year old woman, admitted as an emergency for acute myopericarditis, in cardiogenic shock. Echocardiography showed severe left ventricular dysfunction associated with concentric biventricular hypertrophy with increased echogenicity of the myocardial walls. Poor response to treatment with intravenous inotropic drugs led to referral for biventricular circulatory assistance with a Biomedicus pump, which was withdrawn on the 9th day after recovery of normal left ventricular function. Myocardial biopsies showed massive interstitial inflammation with a predominance of eosinophilic cells suggesting a hypersensitivity reaction. Steroid therapy was prescribed when the results of biopsy were known and stopped after 6 months. This case emphasises the value of echocardiography and myocardial biopsy in diagnosis and treatment of acute myocarditis with cardiogenic shock.

[Can one die of sorrow?]. / Peut-on mourir de chagrin?

The authors report the case of a 49 year old woman who, on two occasions four years apart, presented with cardiogenic shock following the same type of intense emotional stress. Acute left ventricular systolic dysfunction in the initial phase regressed completely with drugs. A diagnostic investigation excluded atheromatous coronary artery disease, myocarditis and pheochromocytoma. Two hypotheses remained: prolonged coronary spasm causing myocardial stunning or acute catecholaminergic cardiomyopathy secondary to the stress.

[CardioWest, a complete artificial heart, the French experience]. / Le coeur artificiel total CardioWest, l'expérience française.

The development of implantable electric assist systems for the left ventricle cannot be used in all patients. Approximately one-third of them who could benefit from circulatory support while waiting for transplantation also require a supporting system for the right ventricle. In addition, the small number of available donor organs means that only those patients with the best prognosis can be transplanted. From January 1993 to December 1995, 36 patients (33 men, 3 women) mean age 41 years (range 19-60) were treated with a total artificial heart system, CardioWest at the La Pitié Hospital in Paris and at the Laennec Hospital in Nantes, France. Heart assist lasted 1 to 138 days (mean 31 days). Indications were terminal heart failure due to dilated (n = 21) or ischemic (n = 11) cardiomyopathy and 4 high-risk indications (chronic rejection, primary failure, hereditary heart disease, polyvalve disease). Twenty-two of these patients (61.1%) were transplanted. By using strict selection criteria, the total artificial heart CardioWest was able to maintain satisfactory hemodynamic conditions until transplantation without compromising the small number of donor organs available. The total artificial heart is the best system available for total circulatory support in case of dual ventricular failure. The risk of infection is low and the rate of thrombo-embolic events is reduced when care is taken in controlling coagulation, allowing long-term support. Independently of the experience of the different teams, CardioWest is the only device currently available allowing total heart support; its use is justified in particular when other assist devices are unsuccessful.

[What are the indications for ambulatory mechanical hart-assist devices for the left ventricle?]. / Quelles indications pour l'assistance mécanique ambulatoire du ventricule gauche?

Ambulatory mechanical left ventricular assistance has become a reality but clinical experience of long-term tolerance is limited for setting up a randomised trial comparing this technique with cardiac transplantation. On the other hand, it is justified in proposing this long-term circulatory assistance to a subgroup of patients on the waiting list for transplantation but who have little chance of being transplanted before irreversible multivisceral failure sets in. Some specific contraindications to left ventricular mechanical assistance should be respected. After implantation of a portable system, the possibility of being reinscribed on the waiting list for cardiac transplantation should be envisaged if the patient so wishes.

[Recent advances in cardiac pacing]. / Progres récents en stimulation cardiaque.

Over recent years, the increasing complexity of pacemakers has discouraged many cardiologists who considered that their role in the surveillance of patients with pacemakers had become minimal. Fortunately, times have changed, as this sophistication, which has how reached maturity, simplifies the cardiologist's task and should renew this interest in pacemaking. For example, improvement of electronic circuits now allows simpler pacing modes (e.g. single electrode VDD mode) and the development of automation of the main pacing parameters: automatic adjustment of the discharge current, the A-V period, protection algorithms against tachycardia due to electronic re-entry and atrial arrhythmias, automatic mode switching. Finally, the incorporation of large capacity memory functions allows the apparatus to act like an implantable "mini Holter" monitor.

Endomyocardial biopsies: a new approach for studying the electrical and mechanical properties of human ventricular myocardium.

The novel endomyocardial biopsy approach described here could facilitate the study of some human pathologies for which tissue specimens are currently unavailable. Electrical and mechanical characteristics such as rat responses, effect of extracellular calcium concentration changes and beta-adrenergic tone were similar to those determined for other human ventricular tissues, indicating that endomyocardial biopsies are suitable for physiological studies. This new source of cardiac tissues should facilitate the investigation of cellular mechanisms involved in the development of previously inaccessible human diseases.

[Detection of residual myocardial ischemia by Thallium-201 myocardial scintigraphy after myocardial infarction. Apropos of 53 patients treated with thrombolytic agents during the acute phase and with coronary transluminal angioplasty]. / Appréciation de l'ischémie résiduelle par scintigraphie myocardique au thallium-201, après infarctus du myocarde. A propos de 53 patients traités par thrombolytiques à la phase aiguë et par angioplastie transluminale coronaire.

The prognosis of silent ischemia after myocardial infarction is similar to that of post-infarction angina. In order to detect this condition two stress myocardial scintigraphies were performed: three weeks after hospital admission for myocardial infarction treated by thrombolytic therapy without any complications or recurrence of chest pain; one month later, after percutaneous transluminal coronary angioplasty on the infarct-related artery in 24 patients or after medical therapy alone when this procedure was not possible (29 patients). Silent ischemia, initially present in two thirds of patients, was less frequently observed in the patients undergoing angioplasty (p < 0.05). In the remaining one third of patients with no silent ischemia, myocardial scintigraphy was unchanged at the follow-up procedure whether or not angioplasty had been performed. These results show that silent ischemia is commonly observed during stress myocardial scintigraphy after acute myocardial infarction treated by thrombolysis, but that this condition can be significantly reduced by coronary angioplasty. When no silent ischemia is observed, coronary angiography and angioplasty do not seem to be indicated.

[Right atrioventricular metastasis of myxoid liposarcoma. Prolonged course after repeated surgery and chemotherapy]. / Métastase auriculo-ventriculaire droite d'un liposarcome myxoïde. Evolution prolongée avec chirurgie itérative et chimiothérapie.

The authors report a case of cardiac metastasis of a myxoid liposarcoma of the thigh in a 54 year old woman. The treatment associated repeated surgical ablation and chemotherapy. The drugs used induced some cardiotoxicity which limited their prescription but nevertheless, the patient survived 5 years. Previous reported cases do not describe survival lasting more than 2 years and associated chemotherapy would therefore seem to be a valuable adjuvant, increasing the life expectancy of such patients.

[Biological myolysis during combined fenofibrate-pravastatin therapy]. / Myolyse biologique survenue à l'occasion d'un relais thérapeutique fénofibrate-pravastatine.

The authors report a case of biological myolysis related to combined fenofibrate-pravastatin therapy in the course of a change in treatment. This case suggests that the risk of myolysis is not specific to the association of some compounds of the two classes but is inherent in possible combinations of drugs from these two families. One must therefore abstain from prescribing these two classes of drugs, since there is no evidence that the combination is preferable to the HMG CoA inhibitor administered alone and the risk of iatrogenic complications is increased. Finally, during the passage from fibrate to HMG CoA reductase a treatment-free interval of about one week should be mandatory.

[Arrhythmogenic effects of sultopride chlorhydrate: clinical and cellular electrophysiological correlation]. / Effets arythmogènes du chlorydrate de sultopride: corrélation clinique et électrophysiologique cellulaire.

This study was designed following the first documented case of torsades de pointes induced by sultopride hydrochloride, a substituted benzamide neuroleptic drug. The patient, a 48 year-old woman with no known cardiovascular disease, had been treated for several years with this drug. She was admitted for severe bronchospasm requiring artificial ventilation. Twenty-one hours after her admission, she developed several episodes of torsades de pointes, which were successfully treated with magnesium sulphate. At that time, the QT interval was 500 ms for a heart rate of 108 b.min-1 (QTc of 668 ms, and theoretical QTc 370 ms). On the fourth day, QTc was 548 ms and theoretical QTc 370 ms. The sultopride was stopped on the fifth day. Two days later, QTc was 397 ms. Six months later, there was no recurrence. Several cases of TdP or sudden death have been reported in patients receiving neuroleptic drugs. The effects of sultopride hydrochloride were therefore tested on isolated ferret Purkinje fibres, using the microelectrode technique. Three concentrations of the drug (D1, D2, D3) were tested, as well as normal Tyrode solution. Maximum diastolic potentials (Vmax) were -88.37 +/- 0.89 mV (control), -89.08 +/- 1.20 mV (D1), -90.00 +/- 1.06 mV (D2), and -90.14 +/- 1.20 mV (D3). Vmax was not affected by sultopride during pacing at 1,000 ms of cycle length. The duration of the action potential increased with the drug concentration. There was no early after-depolarisation (EAD) during control, and 7 out of 9 fibers had EAD and 3 out of 9 triggered activity in D3. The solvent (benzyl alcohol) did not modify the action potential.(ABSTRACT TRUNCATED AT 250 WORDS)

[Wolff-Parkinson-White syndrome. Value of transesophageal atrial stimulation coupled with exercise test for the study of anterograde conduction in the accessory pathway]. / Syndrome de Wolff-Parkinson-White. Intérêt de la stimulation auriculaire transoesophagienne couplée à l'épreuve d'effort pour l'étude de la conduction antérograde dans la voie accessoire.

In patients with Wolff-Parkinson-White syndrome the anterograde conduction properties of the accessory pathway determine the ventricular rate in case of atrial fibrillation (AF). Anterograde conduction in the accessory pathway was evaluated in 20 patients (mean age 31 years) by means of transoesophageal atrial pacing with increasing frequency (up to 460 per minute), first at rest, then during exercise on an ergometric bicycle and upon immediate recovery. The exploration was completed by a search for the disappearance of pre-excitation during exercise and after an intravenous injection of ajmaline 1 mg/kg. The shortest cycle (SC) of atrial pacing with 1:1 conduction by the accessory pathway regularly decreased by 80 +/- 26 ms (n = 18), i.e. 27 p. 100 of its value at rest. At immediate recovery SC increased by 40 +/- 53 ms (n = 9). Atrial fibrillation was induced at rest and/or during exercise in 12 patients. The shortest interval (SI) between two pre-excited ventricular complexes was 290 +/- 80 ms (n = 8) at rest and 244 +/- 53 ms (n = 8) during exercise. With a substantial group of values (n = 12) there was good correlation between SC and SI both at rest and during exercise. With a smaller group of values (n = 3) SI was clearly greater than SC, suggesting a concealed conduction in the accessory pathway during atrial fibrillation. Disappearance of pre-excitation during exercise was observed in 4 patients, 3 of whom had a short (less than 250 ms) SC and/or SI.(ABSTRACT TRUNCATED AT 250 WORDS)