From electrophysiological exploration to management of heart arrhythmias: Economic analysis of practices in a high-volume French hospital over two different time periods.

BACKGROUND: Medical devices (MD) used to treat arrhythmias range from electrophysiological exploration catheters to intracardiac ablation catheters, and they are continuously undergoing optimization. The inclusion of innovative MD in Diagnosis Related Groups (DRG) of the French healthcare economic system can lead to financial imbalance for health institutions. The objective of this study was to compare cost-revenue analyses for interventional heart rhythm management in a high-volume French hospital between two time periods. METHODS: For 3 months in 2014 and 3 months in 2017, all of the patients admitted to the interventional rhythmic unit with arrhythmia were included retrospectively in this monocenter study. All arrhythmias were considered. The primary clinical endpoint was the difference between the expenses and incomes, calculated for each patient. The secondary endpoint was the breakdown of costs. RESULTS: 217 patients were included. In 2014 period, the analysis revealed a deficit of 409±1717 euros per patient and an overall deficit for the hospital of 44,635 euros. In 2017 period, the same evaluation indicated a deficit of 446±1316 euros per patient and an overall deficit for the hospital of 48,210 euros. The cost of MD accounts for a significant share of total expenses. CONCLUSION: The profitability for the cardiac rhythm activity at our facility was optimized between 2014 and 2017. The reliance on ambulatory care increased. However, the reduction in the expenses incurred did not increase the profitability for the facility. It was offset by a decrease in DRG tariffs. A flowchart-type structure based on these practices analyses for rhythmic disorder treatments was developed.

Is oxidative stress associated with disease severity, pulmonary function and metabolic syndrome in chronic obstructive pulmonary disease? / ¿Está el estrés oxidativo asociado a la gravedad de la enfermedad, a la función pulmonar y al síndrome metabólico en la enfermedad pulmonar obstructiva crónica?

OBJECTIVE: To investigate associations between oxidant/antioxidant biomarkers with the disease severity, pulmonary function and diagnosis of metabolic syndrome (MetS) in patients with COPD. METHODS: Seventy-four subjects were included, 39 with COPD (age 69±7 years; female 41%) and 35 for control group (age 69±7 years; female 43%). They were diagnosed with MetS and allocated in one of 4 subgroups: COPD and control, with and without MetS, respectively. Advanced oxidation protein products (AOPP), paraoxonase-1, catalase activity, sulfhydryl group and total lipid hydroperoxide were assayed. Pulmonary function was performed with a plethysmograph. RESULTS: COPD severity (GOLD≥3) and pulmonary function were associated with sulfhydryl group and AOPP (P≤.03 for all). The prevalence of MetS was associated with AOPP in COPD (P=.04). Individuals with COPD and MetS showed higher AOPP compared to COPD without MetS (P<.0001). CONCLUSION: COPD severity, worse pulmonary function and presence of metabolic syndrome are associated with oxidative stress in individuals with COPD.

Effects of exercise training in water and on land in patients with COPD: a randomised clinical trial.

OBJECTIVES: To compare the effects of two similar 6-month protocols of high-intensity exercise training, in water and on land, in patients with chronic obstructive pulmonary disease (COPD). DESIGN: Randomised controlled trial. SETTING: University-based outpatient clinic. PARTICIPANTS: Thirty-six patients with predominantly moderate-to-severe COPD completed the study. INTERVENTION: Patients were evaluated at baseline, at 3 months and at the end of the programme (i.e. 6 months). For both groups, the 6-month protocol consisted of high-intensity endurance and strength exercises with gradual increase in time and/or workload, totalling 60 sessions. MAIN OUTCOMES: Objective monitoring of physical activity in daily life (PADL, primary outcome), lung function, peripheral and respiratory muscle strength, body composition, maximal and submaximal exercise capacity, functional status, quality of life, and symptoms of anxiety and depression. RESULTS: After 6 months of training, a significant improvement in PADL was seen for both groups [mean difference (95% confidence interval): land group 993 (358 to 1628) steps/day; water group 1669 (404 to 2934) steps/day]. Significant improvements were also seen in inspiratory, expiratory and peripheral muscle strength; maximal and submaximal exercise capacity; quality of life and functional status for both groups. There were no significant improvements in lung function, body composition, and symptoms of anxiety and depression for either group. No difference was found in the magnitude of improvement between the two types of training for any outcome. CONCLUSION: High-intensity exercise training in water generates similar effects compared with training on land in patients with moderate-to-severe COPD, rendering it an equally beneficial therapeutic option for this population. CLINICAL TRIAL REGISTRATION NUMBER: NCT01691131.

Agreement of different reference equations to classify patients with COPD as having reduced or preserved 6MWD.

BACKGROUND: Interpretation of the six-minute walk distance (6MWD) is enhanced by using recommended reference equations. Whenever possible, the choice of equation should be region-specific. A potential problem is that different equations for the 6MWD may have been developed for the same population, and it may be complicated to choose the most suitable. OBJECTIVE: To verify the agreement of different reference equations in classifying patients with Chronic Obstructive Pulmonary Disease (COPD) as having reduced or preserved 6MWD. METHODS: 159 patients with COPD performed the six-minute walk test according to international standardization. They were classified as having reduced 6MWD if it was below the lower limit of normal. Five Brazilian equations (Iwama; Britto1; Britto2; Dourado; Soares) and the two non-Brazilian equations most cited worldwide (Troosters; Enright) were used. The agreement for patients classified as reduced or preserved 6MWD was verified by Cohen's Kappa (pair-to-pair) analysis. The proportion of patients classified as having reduced walked distance was compared by the Chi-squared test. RESULTS: Agreement between equations varied largely in classifying subjects as having reduced or preserved 6MWD (Kappa: 0.10-0.82). Brazilian equations with the highest agreement were Iwama, Britto1 and Britto2 (Kappa>0.75). The proportion of patients classified as having reduced 6MWD was statistically similar only between equations in which the agreement was higher than 0.70. CONCLUSION: Even reference equations from the same country vary considerably in the classification of reduced or preserved 6MWD, and it is recommended that the region-specific ones be used as they give with higher agreement for similar and comparable interpretation of the patients' functional exercise capacity.

Circulating PCSK9 levels in acute coronary syndrome: Results from the PC-SCA-9 prospective study.

BACKGROUND: Serum proprotein convertase subtilisin/kexin type 9 (PCSK9) concentrations have been shown to be positively associated with LDL cholesterol (LDL-C), but the relationship between PCSK9 and coronary atherosclerosis lesions remains unclear. OBJECTIVE: This study aims to investigate the correlation between serum PCSK9 levels and coronary damage severity in patients hospitalized for acute coronary syndrome (ACS). METHODS: In this prospective proof-of-concept study, coronary lesions were assessed using SYNTAX scores. Serum PCSK9 concentrations were measured on admission (Day 0) for ACS by Elisa, and on every day of hospitalization. Spearman's correlations were used to determine the association between PCSK9 levels, SYNTAX score and metabolic parameters. RESULTS: A total of 174 patients (mean age: 59±14 years, 79% male) with ACS (on Day 0, 119 patients were not taking statins, but 55 were) were included. After initiation of high-intensity statin therapy, serum PCSK9 concentrations increased significantly, reaching maximum levels on Day 2 (+31% vs. Day 0), and remained stable up to Day 4 (P<0.001, by mixed model). Serum PCSK9 on Day 0 was associated with LDL-C (rho=0.226, P=0.017) and apolipoprotein B (rho=0.282, P=0.005) in the statin-naïve group only, and with triglycerides and non-HDL-C in all groups. More important, PCSK9 levels on Day 0 were positively associated with SYNTAX scores in the statin-naïve group (rho=0.239, P=0.009), but not in the statin-treated group (P=NS). This association was maintained after adjusting for LDL-C (P=0.014) and major CV risk factors (P=0.008). CONCLUSION: Serum PCSK9 levels are positively associated with severity of coronary artery lesions independently of LDL-C concentrations in patients hospitalized for ACS. This reinforces the potential importance of PCSK9 inhibition in the management of ACS.

Smoking status and its relationship with exercise capacity, physical activity in daily life and quality of life in physically independent, elderly individuals.

OBJECTIVE: To investigate the relationship between smoking status and exercise capacity, physical activity in daily life and health-related quality of life in physically independent, elderly (≥60 years) individuals. DESIGN: Cross-sectional, observational study. SETTING: Community-dwelling, elderly individuals. PARTICIPANTS: One hundred and fifty-four elderly individuals were categorised into four groups according to their smoking status: never smokers (n=57), passive smokers (n=30), ex-smokers (n=45) and current smokers (n=22). MAIN OUTCOME MEASURES: Exercise capacity [6-minute walk test (6MWT)], physical activity in daily life (step counting) and health-related quality of life [36-Item Short Form Health Survey (SF-36) questionnaire] were assessed. RESULTS: Current and ex-smokers had lower mean exercise capacity compared with never smokers: 90 [standard deviation (SD) 10] % predicted, 91 (SD 12) % predicted and 100 (SD 13) % predicted distance on 6MWT, respectively [mean differences -9.8%, 95% confidence intervals (CI) -17.8 to -1.8 and -9.1%, 95% CI -15.4 to -2.7, respectively; P<0.05 for both]. The level of physical activity did not differ between the groups, but was found to correlate negatively with the level of nicotine dependence in current smokers (r=-0.47, P=0.03). The median score for the mental health dimension of SF-36 was worse in passive {72 [interquartile range (IQR) 56 to 96] points} and current [76 (IQR 55 to 80) points] smokers compared with ex-smokers [88 (IQR 70 to 100) points] (median differences -16 points, 95% CI -22.2 to -3.0 and -12 points, 95% CI -22.8 to -2.4, respectively; P<0.05 for both). CONCLUSIONS: Among elderly individuals, current smokers had lower exercise capacity than never smokers. Although the level of physical activity did not differ between the groups, an association was found with smoking. Tobacco exposure was associated with worse scores for the mental health dimension of SF-36 in physically independent, elderly individuals.

Valvular dystrophy associated filamin A mutations reveal a new role of its first repeats in small-GTPase regulation.

Filamin A (FlnA) is a ubiquitous actin binding protein which anchors various transmembrane proteins to the cell cytoskeleton and provides a scaffold to many cytoplasmic signaling proteins involved in actin cytoskeleton remodeling in response to mechanical stress and cytokines stimulation. Although the vast majority of FlnA binding partners interact with the carboxy-terminal immunoglobulin like (Igl) repeats of FlnA, little is known on the role of the amino-N-terminal repeats. Here, using cardiac mitral valvular dystrophy associated FlnA-G288R and P637Q mutations located in the N-terminal Igl repeat 1 and 4 respectively as a model, we identified a new role of FlnA N-terminal repeats in small Rho-GTPases regulation. Using FlnA-deficient melanoma and HT1080 cell lines as expression systems we showed that FlnA mutations reduce cell spreading and migration capacities. Furthermore, we defined a signaling network in which FlnA mutations alter the balance between RhoA and Rac1 GTPases activities in favor of RhoA and provided evidences for a role of the Rac1 specific GTPase activating protein FilGAP in this process. Together our work ascribed a new role to the N-terminal repeats of FlnA in Small GTPases regulation and supports a conceptual framework for the role of FlnA mutations in cardiac valve diseases centered around signaling molecules regulating cellular actin cytoskeleton in response to mechanical stress.

Prevalence, characteristics, and prognosis role of type 1 ST elevation in the peripheral ECG leads in patients with Brugada syndrome.

BACKGROUND: Despite isolated reports of Brugada syndrome (BrS) in the inferior or lateral leads, the prevalence and prognostic value of ST elevation in the peripheral electrocardiographic (ECG) leads in patients with BrS remain poorly known. OBJECTIVE: To study the prevalence, characteristics, and prognostic value of type 1 ST elevation and ST depression in the peripheral ECG leads in a large cohort of patients with BrS. METHODS: ECGs from 323 patients with BrS (age 47 ± 13 years; 257 men) with spontaneous (n = 141) or drug-induced (n = 182) type 1 ECG were retrospectively reviewed. Two hundred twenty-five (70%) patients were asymptomatic, 72 (22%) patients presented with unexplained syncope, and 26 (8%) patients presented with sudden death (12 patients) or appropriated implantable cardioverter-defibrillator therapies (14 patients) at diagnosis or over a mean follow-up of 48 ± 34 months. RESULTS: Thirty (9%) patients presented with type 1 ST elevation in at least 1 peripheral lead (22 patients in the aVR leads, 2 in the inferior leads, 5 in both aVR and inferior leads, and 1 in the aVR and VL leads). Patients with type 1 ST elevation in the peripheral leads more often had mutations in the SCN5A gene, were more often inducible, had slower heart rate, and higher J-wave amplitude in the right precordial leads. Twenty-seven percent (8 of 30) of the patients with type 1 ST elevation in the peripheral leads experimented sudden death/appropriate implantable cardioverter-defibrillator therapy, whereas it occurred in only 6% (18 of 293) of other patients (P < .0001). In multivariate analysis, type 1 ECG in the peripheral leads was independently associated with malignant arrhythmic events (odds ratio 4.58; 95% confidence interval 1.7-12.32; P = .0025). CONCLUSIONS: Type 1 ST elevation in the peripheral ECG leads can be seen in 10% of the patients with BrS and is an independent predictor for a malignant arrhythmic event.

Effects of arm bracing posture on respiratory muscle strength and pulmonary function in patients with chronic obstructive pulmonary disease.

OBJECTIVE: To analyze the effect of arm bracing posture on respiratory muscle strength and pulmonary function in patients with Chronic Obstructive Pulmonary Disease (COPD). METHODS: 20 patients with COPD (11 male; 67 ± 8 years; BMI 24 ± 3 Kg · m⁻²) were submitted to assessments of Maximal Inspiratory and Expiratory Pressures (MIP and MEP, respectively) and spirometry with and without arm bracing in a random order. The assessment with arm bracing was done on standing position and the height of the support was adjusted at the level of the ulnar styloid process with elbow flexion and trunk anterior inclination of 30 degrees promoting weight discharge in the upper limbs. Assessment without arm bracing was also performed on standing position, however with the arms relaxed alongside the body. The time interval between assessments was one week. RESULTS: MIP, MEP and maximal voluntary ventilation (MVV) were higher with arm bracing than without arm bracing (MIP 64 ± 22 cmH2O versus 54 ± 24 cmH2O, p = 0,00001; MEP 104 ± 37 cmH2O versus 92 ± 37 cmH2O, p = 0,00001 and MVV 42 ± 20 L/min versus 38 ± 20 L/min, p = 0,003). Other variables did not show statistical significant difference. CONCLUSION: The arm bracing posture resulted in higher capacity to generate force and endurance of the respiratory muscles in patients with COPD.

Long-term prognosis of patients diagnosed with Brugada syndrome: Results from the FINGER Brugada Syndrome Registry.

BACKGROUND: Brugada syndrome is characterized by ST-segment elevation in the right precordial leads and an increased risk of sudden cardiac death (SCD). Fundamental questions remain on the best strategy for assessing the real disease-associated arrhythmic risk, especially in asymptomatic patients. The aim of the present study was to evaluate the prognosis and risk factors of SCD in Brugada syndrome patients in the FINGER (France, Italy, Netherlands, Germany) Brugada syndrome registry. METHODS AND RESULTS: Patients were recruited in 11 tertiary centers in 4 European countries. Inclusion criteria consisted of a type 1 ECG present either at baseline or after drug challenge, after exclusion of diseases that mimic Brugada syndrome. The registry included 1029 consecutive individuals (745 men; 72%) with a median age of 45 (35 to 55) years. Diagnosis was based on (1) aborted SCD (6%); (2) syncope, otherwise unexplained (30%); and (3) asymptomatic patients (64%). During a median follow-up of 31.9 (14 to 54.4) months, 51 cardiac events (5%) occurred (44 patients experienced appropriate implantable cardioverter-defibrillator shocks, and 7 died suddenly). The cardiac event rate per year was 7.7% in patients with aborted SCD, 1.9% in patients with syncope, and 0.5% in asymptomatic patients. Symptoms and spontaneous type 1 ECG were predictors of arrhythmic events, whereas gender, familial history of SCD, inducibility of ventricular tachyarrhythmias during electrophysiological study, and the presence of an SCN5A mutation were not predictive of arrhythmic events. CONCLUSIONS: In the largest series of Brugada syndrome patients thus far, event rates in asymptomatic patients were low. Inducibility of ventricular tachyarrhythmia and family history of SCD were not predictors of cardiac events.

Guia para prática clínica: fisioterapia em pacientes com Doença Pulmonar Obstrutiva Crônica (DPOC): [review] / Clinical practice guideline for physical therapy in patients with Chronic Obstructive Pulmonary Disease (COPD): portuguese version: [review]

INTRODUÇÃO: No contexto da colaboração internacional para desenvolvimento de guias práticos (ou guidelines), a Sociedade Real Holandesa de Fisioterapia (Koninklijk Nederlands Genootschap voor Fysiotherapie, KNGF) se propôs a desenvolver um guia para esclarecimento sobre a prática clínica de Fisioterapia em pacientes com Doença Pulmonar Obstrutiva Crônica (DPOC), assim como também optou por estimular a sua tradução para outras línguas, a fim de torná-lo acessível para públicos internacionais. OBJETIVOS: O presente guia é a versão em língua portuguesa do Guia para Prática Clínica de Fisioterapia em pacientes com DPOC desenvolvido pela KNGF, que teve como objetivo descrever a Fisioterapia baseada em evidências para pacientes com DPOC que apresentam limitação da função pulmonar, da função muscular respiratória e periférica, da capacidade de exercício, da depuração mucociliar e da qualidade de vida, além de limitações em relação à atividade física na vida diária pela dispneia e/ou intolerância ao exercício. CONCLUSÃO: O guia propõe-se principalmente a prover recomendações terapêuticas práticas que auxiliem o fisioterapeuta a oferecer o melhor tratamento possível para pacientes com DPOC, consideradas as evidências científicas disponíveis na atualidade.

INTRODUCTION: In the context of international collaboration for the development of practice guidelines, the Royal Dutch Society for Physical Therapy (Koninklijk Nederlands Genootschap voor Fysiotherapie, KNGF) has developed guidelines for the clinical practice of physical therapy in patients with Chronic Obstructive Pulmonary Disease (COPD). It has also stimulated its translation into other languages to make it accessible to international audiences. OBJECTIVES: The present document brings the Portuguese version of the KNGF Clinical Practice Guidelines for physical therapy in COPD patients. Its purpose was to describe evidence-based physical therapy for COPD patients with impairments in pulmonary function, peripheral and respiratory muscle function, exercise capacity, mucus clearance and quality of life, in addition to limitations in physical activity in daily life due to dyspnea and/or exercise intolerance. CONCLUSION: The guideline provides practical and therapeutic recommendations based on currently available scientific evidence to help the physical therapist provide the best possible treatment to COPD patients.

A clinical practice guideline for physiotherapists treating patients with chronic obstructive pulmonary disease based on a systematic review of available evidence.

PURPOSE: Update of a clinical practice guideline for the physiotherapy management of patients with chronic obstructive pulmonary disease supporting the clinical decision-making process. METHODS: A systematic computerized literature search was performed on different modalities for improving physical exercise capacity, reducing exertional dyspnoea, improving airway clearance and encouraging changes in physical activity behaviour. Methodological quality was scored with the PEDro Scale. Scientific conclusions were graded according to the criteria of the; Dutch Evidence Based Guideline Development Platform'. These, together with practical considerations, were used to formulate recommendations for clinical practice. RESULTS: A total of 103 studies were included in the systematic review, consisting of five meta-analyses of randomized controlled trials, 84 randomized controlled trials and 14 uncontrolled studies. Twenty scientific conclusions supported six recommendations on physical exercise training. Nineteen scientific conclusions supported eight recommendations on interventions for reducing dyspnoea. Five scientific conclusions supported seven recommendations concerning treatment modalities to improve mucus clearance, and two scientific conclusions supported two recommendations on strategies for encouraging permanent changes in physical activity behaviour. CONCLUSIONS: Strong recommendations support the use of physical exercise training to improve health-related quality of life and functional exercise capacity. Future research should investigate whether additional interventions for reducing exertional dyspnoea have a place as adjuncts to physical exercise training in selected patients. In addition, treatment of impaired mucus clearance, especially during acute exacerbations, requires further research. With the advance of new technologies for objective measurements of physical activities in daily life more research is needed concerning interventions to initiate and maintain physical activity behaviour change during and after supervised physical exercise training programmes.

[Genetics of atrial fibrillation]. / Génétique de la fibrillation auriculaire.

Atrial fibrillation is the most common cardiac arrhythmia. The identification of familial forms of atrial fibrillation as well as epidemiological studies have confirmed the presence of a genetic background. Familial forms are rare diseases, and the common form is a multifactorial and probably multigenic disease largely modulated by environmental factors. New research developments, based on GWAS studies of well define large cohorts, should, in the near future, help to better characterize the different genetic profiles of this disease, the first step for the identification of new therapeutic targets.

Sodium channel blocker tests allow a clear distinction of electrophysiological characteristics and prognosis in patients with a type 2 or 3 Brugada electrocardiogram pattern.

BACKGROUND: Patients with a type 2 or 3 Brugada syndrome (BS) pattern and a negative sodium channel blocker challenge (SCBC) are not considered as affected. Their arrhythmic prognosis is generally considered good, but it has never been specifically evaluated. OBJECTIVE: The purpose of this study was to evaluate the arrhythmic prognosis in patients with a type 2 or 3 electrocardiogram (ECG) not converted to type 1 ECG during an SCBC. METHODS: Clinical data, 12-lead ECG, results of the SCBC and electrophysiological study (EPS), and follow-up were collected. RESULTS: Among the 500 patients who underwent an SCBC in our institution, 158 displayed a type 2 or 3 ECG. After the SCBC, 93 (59%) had a type 1 ECG (positive group [PG]), whereas 65 (41%) remained negative (negative group [NG]). An EPS was performed in 31 (33%) PG patients and 15 (23%) NG patients. Ventricular fibrillation was induced in 21 PG patients (67%), whereas no patient in the NG was inducible (P <.001). During a follow-up of 37 +/- 17 months, no sudden death occurred. Three syncopes were observed in the NG versus one syncope, two ventricular tachycardias, and one appropriate shock in the PG. CONCLUSION: This study demonstrates that the presence or absence of coved type ST-segment elevation during the SCBC denotes a profound electrophysiological difference as demonstrated by the absence of inducibility during EPS in the NG that may be responsible for the good prognosis of patients with a type 2 or 3 ECG pattern not converted to type 1.

[Genetic aspects of valvulopathies]. / Aspects Génétiques des Valvulopathies.

Valvular dystrophies due to myxoid degeneration are common and potentially serious cardiac pathologies. They constitute a heterogeneous group of which the most usual is idiopathic mitral valvular prolapse (Barlow's disease). The majority of mitral valvular prolapses are sporadic, but there are several familial forms. Transmission is usually autosomal dominant with incomplete penetrance and variable expression. The first chromosomal location to be identified was on the 16p11-13 chromosome. Since then, two other loci have been identified on the 11p15.4 and 13q31-32 chromosomes. Our team has recently identified the first gene responsible for myxoid valvulopathy linked to the X chromosome, from a large family of 318 members. This is the gene that codes for filamin A, which is a cytoskeleton protein. The frequency of mutations in this gene is still unknown, but out of 7 families in which transmission was compatible with X-linked transmission, mutations were discovered in 4 of the families. Thanks to a genetic epidemiological approach, we have also demonstrated that there are familial forms of aortic stenosis, which are probably common. Identification of the genes implicated in these common forms of valvular pathology is important, as it will allow a better understanding of the pathophysiology of these valvular disorders and could lead to better therapeutic management in the future.

Quantifying physical activity in daily life with questionnaires and motion sensors in COPD.

Accurate assessment of the amount and intensity of physical activity in daily life is considered very important due to the close relationship between physical activity level, health, disability and mortality. For this reason, assessment of physical activity in daily life has gained interest in recent years, especially in sedentary populations, such as patients with chronic obstructive pulmonary disease (COPD). The present article aims to compare and discuss the two kinds of instruments more commonly used to quantify the amount of physical activity performed by COPD patients in daily life: subjective methods (questionnaires, diaries) and motion sensors (electronic or mechanical methods). Their characteristics are summarised and evidence of their validity, reliability and sensitivity is discussed, when available. Subjective methods have practical value mainly in providing the patients' view on their performance in activities of daily living and functional status. However, care must be taken when using subjective methods to accurately quantify the amount of daily physical activity performed. More accurate information is likely to be available with motion sensors rather than questionnaires. The selection of which motion sensor to use for quantification of physical activity in daily life should depend mainly on the purpose of its use.

Cardiopulmonary stress during exercise training in patients with COPD.

Exercise training is an essential component of pulmonary rehabilitation. However, the cardiopulmonary stress imposed during different modalities of exercise training is not yet known. In the present study, the cardiopulmonary stress of a 12-week exercise training programme in 11 chronic obstructive pulmonary disease (COPD) patients (forced expiratory volume in one second 42+/-12%pred, age 69+/-6 yrs) was measured. Pulmonary gas exchange and cardiac frequency (f(C)) of three training sessions were measured with a portable metabolic system at the beginning, mid-term and end of the programme. Symptoms were assessed with Borg scores. The exercise intensity was compared with the recommendations for exercise training by the American College of Sports Medicine (ACSM). Training effects were significant (maximum change in work: 14+/-11 Watts, 6-min walk test: 44+/-36 m). Whole body exercises (cycling, walking and stair climbing) consistently resulted in higher cardiopulmonary stress (oxygen uptake (V'(O(2))), minute ventilation and f(C)) than arm cranking and resistance training. Dyspnoea was higher during cycling than resistance training. Patients exercised for >70% (>20 min) of the total exercise time at >40% of the V'(O(2)) reserve and f(C) reserve ("moderate" intensity according to the ACSM) throughout the programme. The cardiopulmonary stress resistance training is lower than during whole-body exercise and results in fewer symptoms. In addition, exercise testing based on guidelines using a fixed percentage of baseline peak performance and symptom scores achieves and sustains training intensities recommended according to the American College of Sports Medicine.

[Bi-directional ventricular tachycardia and pheochromocytoma: a case report]. / Tachycardie ventriculaire bidirectionnelle et phéochromocytome: a propos d'un cas.

We present the case of a 48 year old woman who was admitted to our university hospital in cardiogenic shock with bi-directional ventricular tachycardia degenerating into polymorphic venricular tachycardia which resolved spontaneously. Investigation revealed healthy coronary arteries but severe left ventricular dysfunction due to akinesia involving the entire base. There was a rapid improvement within several days. The diagnosis of bilateral phaeochromocytoma was made on the biochemistry and CT scan of the adrenals.

[Genetics and cardiac arrhythmias]. / Génétique des troubles du rythme cardiaque.

The identification of the first gene locus of hereditary arrhythmias was made over 10 years ago. In the last few years, considerable progress has been made and the number of culprit genes for cardiac arrhythmias has rapidly increased. This has been the fruit of close collaboration between clinicians, geneticists and physiologists. This work has demonstrated the heterogenous nature of genetics of diseases. It has led to a better understanding of underlying physiopathological mechanisms by the study of the relationship between gene and clinical abnormalities. In addition, analysis of phenotypes and genotypes has improved our knowledge of the clinical presentation of diseases and opened up new therapeutic approaches. These new diagnostic methods have enabled preventive measures to be taken to avoid potentially serious arrhythmias. The genetics of cardiac arrhythmias is still in its infancy: many culprit genes remain undetected and their identification should led to considerable progress in the understanding of the physiopathology of arrhythmias and their treatment.

[Genetic aspects of cardiac conduction defects]. / Aspect génétique des troubles de la conduction cardiaque.

Degenerative conduction defects are usually considered to be exaggerated ageing affecting the conduction pathways. For several years familial forms have been described, and a first locus on chromosome 19 and then a first gene, SCN5A on chromosome 3 (coding for the sodium channel alpha subunit), have been identified. Mutations of this gene can lead not only to congenital conduction defects but also to progressive forms of conduction defects similar to Lenègre disease. A third locus on chromosome 16 at 16q23-24 has been identified, as have other families not linked to the loci described previously. Although it now seems clear that conduction defects can have a genetic component, the frequency of the familial forms remains to be determined. Important progress could be made in the understanding of this disease if other implicated genes were identified. It would then become possible to elucidate the different pathophysiological mechanisms responsible for conduction defects.