[A familial form of conduction defects associated with a PRKAG2 gene mutation]. / Une forme familiale de troubles conductifs associés à une mutation du gène PRKAG2.

Conduction defects are usually secondary to elective aging of the conduction pathways. However, some familial and genetic forms are now being described. Here we report a particular electrocardiographic pattern in four members of the same family over three generations, naturally leading to the suspicion of a hereditary origin. The ECG appearance is very specific and includes conduction defects (RBBB and occasionally left anterior hemiblock), short PR interval, pseudo appearance of atrial hypertrophy, and occasionally sinus dysfunction or supraventricular extrasystole. Gene analysis identified a R302Q mutation of the gamma2 subunit producing AMP protein kinase, coded by the gene PRKAG2. This is a wrong sense mutation present in the heterozygous state in each of those displaying the ECG anomalies, and is transmitted in an autosomal dominant fashion. The clinical picture here appears to constitute a clinical entity distinct from those previously described as being associated with mutations of the PRKAG2 gene, without any left ventricular hypertrophy or Wolff-Parkinson-White syndrome.

[Management of sudden death in a semi-rural district, Seine-et-Marne: the DEFI 77 study]. / Prise en charge de la mort subite dans un département semi rural, la Seine et Marne: étude DEFI 77.

Sudden death is a major problem in public health, affecting around 50 000 people a year in France. The prognosis for cardiac arrest is abysmal because for every minute lost the chances of survival diminish by 10%. The aim of this work was to prospectively evaluate the characteristics of cardiac arrest victims across an entire 6000 km? area, the Seine-et-Marne district, distinguished by the paradox of lying just outside the capital whilst actually being semi-rural, and to determine the current methods of dealing with this emergency. The DEFI 77 prospective epidemiological survey was carried out with the collaboration of the SAMU emergency medical service, the SDIS fire/ambulance service, the general hospitals and the Paris-Ile-de-France cardiological association. Between January 2001 and December 2005 there were 2001 cardiac arrests (mean age 68 +/- 20 years, 67% male) at home in 80% of cases. The arrest was in front of a witness in 72% of cases, but they performed resuscitation in only 14.3% of cases. The SAMU and SMUR emergency medical services attempted cardio-pulmonary resuscitation in 78% of cases. In 29% of cases, one or more external electric shocks were carried out, using a semi-automatic defibrillator 79% of the time. Only 11.5% of patients arrived at the emergency department alive, the overall hospital survival rate being less than 2%. Only eight patients subsequently underwent automatic defibrillator implantation. The results of this observational study are to a large extent explained by an extremely long delay (12 minutes) before help was called for, the delay between the call and the arrival of medical assistance (9.5 +/- 4 min), the low percentage of active witnesses, and the variability in management (invasive in particular). In conclusion, at the dawn of the third millennium the prognosis of cardiac arrest remains very poor and fully justifies educating the general public about calling for help early and about actions that can save lives, particularly external cardiac massage before the arrival of the emergency services, as well as the benefits of using automated external defibrillators.

Heart rate and cardiac rhythm relationships with bisoprolol benefit in chronic heart failure in CIBIS II Trial.

BACKGROUND: beta-Blockade-induced benefit in heart failure (HF) could be related to baseline heart rate and treatment-induced heart rate reduction, but no such relationships have been demonstrated. METHODS AND RESULTS: In CIBIS II, we studied the relationships between baseline heart rate (BHR), heart rate changes at 2 months (HRC), nature of cardiac rhythm (sinus rhythm or atrial fibrillation), and outcomes (mortality and hospitalization for HF). Multivariate analysis of CIBIS II showed that in addition to beta-blocker treatment, BHR and HRC were both significantly related to survival and hospitalization for worsening HF, the lowest BHR and the greatest HRC being associated with best survival and reduction of hospital admissions. No interaction between the 3 variables was observed, meaning that on one hand, HRC-related improvement in survival was similar at all levels of BHR, and on the other hand, bisoprolol-induced benefit over placebo for survival was observed to a similar extent at any level of both BHR and HRC. Bisoprolol reduced mortality in patients with sinus rhythm (relative risk 0.58, P:<0.001) but not in patients with atrial fibrillation (relative risk 1.16, P:=NS). A similar result was observed for cardiovascular mortality and hospitalization for HF worsening. CONCLUSIONS: BHR and HRC are significantly related to prognosis in heart failure. beta-Blockade with bisoprolol further improves survival at any level of BHR and HRC and to a similar extent. The benefit of bisoprolol is questionable, however, in patients with atrial fibrillation.

[Septic false aneurysm of the ascending thoracic aorta. Value of covering omentum repair]. / Faux anévrysme septique de l'aorte thoracique ascendante. Intérêt de l'épiplooplastie de couverture.

The incidence of post-sternotomy mediastinitis is 1 to 5% but the mortality is about 40%. The formation of a pseudo-aneurysm is a rare complication and an infectious origin is exceptionally rare. Diagnosis must be early because of the risk of rupture. In this case, a 47 year old patient developed slowly progressive mediastinitis presenting with a pseudo-aneurysm of the aorta. Surgery comprised a retrosternal transposition of the greater omentum.

[Late potentials in chronic alcoholics]. / Potentiels tardifs chez les alcooliques chroniques.

OBJECTIVE: Cardiac arrest is the most frequent cause of death in chronic alcoholics. Detection of late potentials in this population could be helpful in screening from early signs of myocardial disorders and identifying patients at risk of severe ventricular dysrythmia. PATIENTS AND METHODS: A prospective study of late potentials was conducted in 53 subjects (mean age 49 +/- 10 years) with a history of long-standing alcohol abuse (mean 13.6 +/- 8.5 years, mean daily alcohol intake 86 +/- 30 g). After a period of abstinence, the following explorations were performed: liver tests, liver biopsy, electrocardiogram, echocardiography, Holter recording. RESULTS: Among the 53 patients, 37% were positive for 2 of the 3 criteria for late potentials. There was a strong correlation between the duration of alcohol abuse and presence of late potentials (p = 0.006, r = 0.37). The percentage of hepatic steatosis was higher in alcoholic subjects with late potentials (34% versus 23%; p = 0.05) and was correlated with the number of positive criteria for late potentials (p = 0.05, r = 0.328). Finally, the presence of late potentials was also correlated with the following laboratory results: serum gamma glutamyltranspeptidase (p = 0.031), serum aspartate amino transferase (p = 0.033), serum alkaline phosphatases (p = 0.0025). CONCLUSION: Late potentials can be detected easily although their prognostic value remains to be determined. They could be an early marker of infraclinical myocardial lesions.

[Signal averaging electrocardiography in chronic alcoholism]. / Enregistrement de l'électrocardiogramme haute amplification chez les éthyliques chroniques.

Cardiovascular death is the main cause of mortality in chronic alcoholics, perhaps due to a pro-arrhythmogenic effect of alcohol associated with infraclinical myocardial lesions. The authors investigated prospectively 41 patients (average age: 49.7 years) who were chronic alcoholics but had no acute alcoholic episodes for cardiac disease (ECG, signal averaging for late ventricular potentials, echocardiography and Holter ECG monitoring) and hepatic disease (liver biopsy). The history of alcoholism was 14 +/- 9 years, the quantity of alcohol ingested before they stopped drinking being 89 +/- 31 grammes/day. Thirty per cent of patients displayed 2 or 3 criteria of late ventricular potentials (LP). The authors demonstrated a correlation between the daily quantity of alcohol consumed before stopping drinking and the duration of the filtered QRS complex (p = 0.02). Moreover, the frequency of fatty infiltration found on liver biopsy, greater in alcoholics with LP (35% versus 19%, p = 0.025) correlated with the amplitude of the last 40 ms of the average QRS (p = 0.0485), with the duration of potentials of less than 40 microvolts (p = 0.05) and, above all, with the number of criteria of LP (p = 0.02). Finally, the presence of LP was also related to the following biological abnormalities: GGT (p = 0.027), ASAT (p = 0.046), ALAT (p = 0.039). The ECG abnormalities may reflect early infra-clinical myocardial lesions secondary to cellular metabolic abnormalities perhaps analogous to the fatty hepatic changes. However, the prognostic value of these signal-averaging ECG abnormalities remains unknown.

Late ventricular potentials and heavy drinking.

OBJECTIVES: To assess the effects of chronic drinking on detection of low amplitude signals, and to determine the relation between late ventricular potentials (LVP) and liver biopsy findings. DESIGN: Prospective study. SETTING: General hospital. PATIENTS: 41 consecutive chronic alcoholics without known pre-existing heart disease. METHODS: About four days after each patient's last alcoholic drink, ECG, echocardiography, signal averaged electrocardiogram, liver biopsy, and blood tests were performed. RESULTS: Twenty eight per cent of patients had evidence of LVP. There was a correlation between the percentage of steatosis of the hepatic biopsy and the amplitude of the last 40 ms of average QRS (P = 0.04), the duration of the terminal low amplitude QRS signal (P = 0.05), and the number of positive criteria of late potentials (P = 0.02). CONCLUSIONS: Chronic drinking sufficient to cause steatosis is associated with positive findings on the signal averaged ECG.

[Do arrhythmias after infarction deter patients from smoking?]. / Les troubles du rythme après un infarctus dissuadent-ils de la poursuite du tabagisme?

Although the short-term outcome of patients treated by thrombolysis during the acute phase of myocardial infarction is now well known, data concerning the repercussions of the coronary accident on smoking are less clearly established. This aspect is particularly important, as cessation of smoking is one of the most effective measures in the context of secondary prevention, with an excellent cost-benefit ratio. Between 1985 and 1991, 218 consecutive patients underwent thrombolysis for myocardial infarction. With a follow-up of 35 +/- 20 months, 11.5% of patients have died, including 6% while in hospital. A questionnaire was sent to 193 surviving patients with a response rate of 97.4%, 27.3% of patients continued to smoke after the myocardial infarction. Smoking patients were younger (p = 0.001) and had generally returned to work (p = 0.05). Continuation of smoking was not influenced by either the patency of the artery or the type of revascularization, and was not correlated with residual symptoms. The frequency of arrhythmias during the acute phase was not related to previous smoking. On the other hand, smoking patients who survived after an infarction appear to quit smoking twice as frequently when they had experienced a cardiac arrhythmia during the acute phase of the infarction (p = 0.005), as confirmed by multivariate analysis. Can the "stress" induced by the arrhythmia, possibly combined with the doctor's reactive anxiety explain cessation of smoking in these patients? If so, it would open new horizons in terms of secondary prevention of myocardial infarction.

[Practical value of the detection of silent myocardial ischemia in patients with coronary disease using Holters]. / Intérêt pratique de la détection de l'ischémie myocardique silencieuse chez le coronarien par les Holters.

The prognosis of painless myocardial ischemia is similar to that of symptomatic ischemia. The Holter technique (and solid memory Holter in particular) is a simple method of detection which, taken together with exercise testing, enables the identification among coronary disease patients of a high risk group in whom effort ischemia is accompanied by episodes of ischemia under everyday conditions and in whom additional investigations (exercise thallium scan then coronary arteriography) and appropriate treatment, including transluminal angioplasty and aorto-coronary bypass if necessary, may be required. The role of drug treatment in silent ischemia has not yet been clearly defined. While electrical ischemia regresses significantly with the majority of standard drug regimens, and beta-blockers in particular, none has yet been confirmed as having a preventive effect against serious events following on from silent myocardial ischemia.

[Diffuse cholesterol embolism after fibrinolysis for myocardial infarction]. / Embolies diffuses de cholestérol après fibrinolyse pour infarctus du myocarde.

The authors report the case of a 57 year old man admitted to hospital 3 hours after the onset of an infero-latero-basal infarct who underwent thrombolytic therapy with intravenous streptokinase, in whom the immediate outcome was complicated by multiple cholesterol embolisation which cause renal failure, mesenteric ischemia, medullary ischemia and livedo reticulosis of the lower half of the body complicated by gangrene of the toes, despite the fact that cardiac catheterisation had not been performed. This case (fifth reported case) of multiple cholesterol embolisation from the abdominal aorta caused by thrombolysis is the earliest presentation of this complication in the literature.

[Hemodynamic study of intravenous milrinone in 26 patients with NYHA class III or IV cardiac failure]. / Etude hémodynamique de la milrinone par voie intraveineuse chez 26 patients en insuffisance cardiaque de classe III ou IV de la NYHA.

The hemodynamic effects of milrinone (WIN 47203) were studied in 26 NYHA Class III or IV patients. The compound was administered intravenously using a protocol including an initial push dose of 50 micrograms/kg in 10 min, followed by a 24 hour infusion at the dose of 0.5 microgram/kg/min. Maximal response was obtained after 15 min and persisted during the infusion: cardiac index increased from 2.08 +/- 0.36 l/min/m2 to 3.09 +/- 0.68 l/min/m2, while capillary pressure fell from 25 mmHg to 16-17 mmHg. These variations were significant (p = 0.01). Heart rate was stable. Mean peripheral blood pressure fell modestly (6%). Systemic vascular resistance fell by 30% and pulmonary vascular resistance by 20%. All these results confirmed the beneficial effect of this inotropic agent administered intravenously. The increase in ventricular premature contractions noted by many justifies the careful surveillance of these patients by monitoring.

[Can solid state Holter monitoring replace endomyocardial biopsy in patients with heart transplantation?]. / Le Holter à mémoire solide peut-il remplacer la biopsie endomyocardique chez le transplanté cardiaque?

Acute cardiac graft rejection after transplantation, the diagnosis of which is based on the findings of endomyocardial biopsy, is associated with a reduction in coronary reserve due to abnormalities of the microcirculation. But this reduction in coronary reserve cause silent myocardial ischaemia (SMI)? In order to assess the frequency of SMI and ventricular arrhythmias during rejection, 53 consecutive Holter recordings were performed in 32 patients (28 men, 4 women, average age 47 +/- 11 years) 11 months after transplantation and within 24 hours of endomyocardial biopsy. The recorder which was used (Monitor One TC) analysed the ST segment in 2 leads in real time: ST segment depression of more than 1 mm lasting over 40 ms, 0.08 s after the J point were considered to be diagnostic of myocardial ischaemia. Although the frequency of SMI is low and not specific for cardiac rejection, its duration was twice as long (80 mn vs 38 mn) in this condition. On the other hand, ventricular arrhythmias are common in cardiac rejection and correlated with its severity according to Billingham's classification (VES p = 0.045; doublets p = 0.035; non-sustained VT p = 0.006).

[Primary isolated dissection of the left anterior descending coronary artery]. / Dissection primitive isolée de l'artère interventriculaire antérieure.

The authors report the case of a 30 year old Senegalese man admitted with an acute antero-septo-apical myocardial infarction which was fibrinolysed at the 5th hour. Coronary angiography showed dissection of the left anterior descending artery. This young patient with no previous medical history or cardiovascular risk factors had an abnormal skin biopsy showing proliferation of the elastic tissues. Eight months after the infarct the patient is asymptomatic and the appearances of dissection have disappeared. This case is original because the dissection occurred in a man, affecting the left anterior descending artery, with a good outcome after fibrinolysis (4th case), the angiographic regression of the arterial lesion and the possible pathogenetic mechanism of disease of elastic tissues.

[Silent myocardial ischemia during hemodialysis in patients with chronic renal insufficiency]. / L'ischémie myocardique silencieuse au cours de l'hémodialyse des insuffisants rénaux chroniques.

A prospective search for episodes of silent myocardial ischaemia (SMI) was carried out during sessions of haemodialysis in 62 patients with chronic renal failure and was positive in 37.1% of the cases. The occurrence of SMI is correlated with the number of cardiovascular risk factors (p = 0.008) and particularly with diabetes (p = 0.012), smoking (p = 0.007) and age (p = 0.02), as well as with the type of nephropathy that had caused the renal failure (p = 0.02). During a 6-month follow-up two patients died; both had silent myocardial ischaemia on Holter recordings. In these anaemic patients, haemodialysis might sensitize the detection of ischaemia by the concomitant occurrence of hypotensive, hypovolaemic or hypoxic episodes, thus playing a aggravating role. The existence of such episodes characterizes a subgroup of patients at high cardiovascular risk for whom the prognosis and the best therapeutic approach remain to be determined.

[Frequency of painless myocardial ischemia during hemodialysis in 50 patients with chronic kidney failure]. / Fréquence de l'ischémie myocardique indolore au cours de l'hémodialyse de 50 insuffisants rénaux chroniques.

The authors carried out a prospective study to determine the frequency of silent ischemia (SI) in 50 consecutive patients with end stage renal failure during dialysis by Holter monitoring. Twenty patients had SI (40%). This event was related to the number of cardiovascular risk factors (p = 0.0025), principally diabetes, smoking and the underlying renal disease (p = 0.018), and to a history of coronary artery disease (p = 0.0015). Two patients died during the nine months follow-up period and both had SI on Holter monitoring. Dialysis therapy in anaemic patients may predispose to and facilitate the detection of myocardial ischemia by the simultaneous interplay of hypotension, hypovolemia, hypoxia and tachycardia. The detection of these ischemic events may allow identification of a subgroup of dialysis patients with a high cardiovascular risk. The prognosis of these patients and best therapeutic approach require further study.

[Pharmacokinetics of oral propafenone in patients with supraventricular arrhythmia]. / Pharmacocinétique de la propafénone orale chez les patients avec arythmies supraventriculaires.

The efficacy of propafenone (P), a class IC antiarrhythmic drug with weak beta-blocking properties was studied over a four day period in 10 patients with supraventricular arrhythmias (atrial fibrillation 7, flutter 1 and tachycardia 2). Group 1 included five patients (3M, 2F) who received 300 mg of P on days 1 and 4. Group 2 included five patients (4M, 1F) who received 600 mg on days 1 and 4. All the patients received 1200 mg/day on days 2 and 3. Pharmacokinetics parameters were calculated for the first and the final dosing. Half of the patients were converted to sinus rythm after a delay ranging from 12 to 55 h after the first dosing. The duration of arrhythmia was shorter and the left atrial diameter was significantly lower in the responder group than in the non-responders. No relationship was observed between clinical efficacy and dose or plasma concentration of P. After the first administration of P, major interindividual variability in pharmacokinetic parameters was observed. Seven patients correspond to the extensive metabolizer phenotype with t1/2 el less than 10 h (mean: 5.4 +/- 2.2 SD). In this group t1/2 el increased from day 1 to day 4 and the AUC final/AUC initial ratio ranged between 4 to 17.5. Three patients showed the non-extensive metabolizer phenotype with t1/2 el ranging from 12.4 to 13.7 h and a moderate increase in AUC over chronic dosing. Adverse effects (cardiac conduction abnormalities, visual and digestive disturbances) were observed in the 3 oldest patients (70-73 yrs).(ABSTRACT TRUNCATED AT 250 WORDS)