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This study aimed at evaluating OS in an amyotrophic quadricipital syndrome with cardiac impairment in a family of 80 members with a mutation in lamin A/C gene. Twelve patients had cardiac involvement (5 cardiac and skeletal muscles impairment). OS was evaluated in blood samples (thiobarbituric acid-reactive substances (TBARS), carbonylated proteins (PCO)) 6 "affected patients" with phenotypic and genotypic abnormalities without heart failure and 3 "healthy carrier" patients. OS was higher in affected patients than in healthy, as shown by the higher TBARS and PCO values. Patients with cardiac and peripheral myopathy exhibited a higher OS than patients with only cardiac disease (TBARS: 1.73 +/- 0.05 vs. 1.51 +/- 0.04 mmol/l (p = 0.051), PCO: 2.73 +/- 0.34 vs. 0.90 +/- 0.10 nmol/mg protein (p = 0.47)), and with healthy carriers patients (TBARS: 1.73 +/- 0.05 vs. 1.16 +/- 0.14 mmol/l (p = 0.05), PCO: 2.73 +/- 0.34 vs. 0.90 +/- 0.20 nmol/mg protein (p = 0.47)). OS may thus contribute to the degenerative process of this laminopathy. ROS production occurs, prior to heart failure symptoms. We suggest that the extent activation may also promote the variable phenotypic expression of the disease.

This study reports a family affected by a new phenotype associated with dilated cardiomyopathy and quadriceps myopathy. 1 Methods: 29 family members underwent a physical and neurological examination, including an electromyogram and biopsy of muscle abnormalities. A cardiac examination was performed in all subjects. 2 Results: The family pedigree (n=72) demonstrated that transmission was autosomal dominant. Eleven subjects had cardiac involvement, only four had quadriceps muscle involvement. Cardiac impairment preceded neurological involvement. The mean age for neurological involvement was 44±0.8 years (range 43–45) and cardiac involvement was 37±7.9 years (range: 24–45). Cardiac involvement consisted of: hypokinetic dilated cardiomyopathy (64%); atrial fibrillation (100%); ventricular arrhythmias (64%); impaired conduction with bundle branch or complete atrio ventricular block (73%). Four patients required pacemakers and anti arrhythmic therapies. Four patients died: two of refractory heart failure and two of sudden death; two patients were resuscitated following cardiac arrest. Three patients required a prophylactic implantable cardiac defibrillator (ICD). Muscle morphological abnormalities were characterized by a variable number of fibers with rimmed vacuoles. The quadriceps deteriorated progressively without impairment of other muscles. Genotypic study showed a lamin A/C gene mutation. 3 Conclusions: This family was affected by a new phenotype composed of an autosomal dominant severe dilated cardiomyopathy with conduction defects or arrhythmias and quadriceps myopathy. Cardiac abnormalities preceded neuromuscular disorders and defined the prognosis of this disease.

Resume Introduction La radiotherapie mediastinale a bouleverse le pronostic de nombre de neoplasies, en particulier les cancers du sein et les lymphomes. Cependant, avec l’augmentation de la survie, des effets secondaires sont apparus parmi lesquels les complications cardiaques, souvent tardives. Observation Il s’agit d’un patient atteint d’une maladie de Hodgkin et traite par radiotherapie. Celle-ci s’est compliquee de troubles conductifs et d’une pericardite chronique, exsudative puis constrictive, 28 ans apres la radiotherapie. Commentaires Comme en temoignent les donnees de la litterature, les complications cardiaques de la radiotherapie peuvent se succeder. Une surveillance attentive et prolongee pour une prise en charge therapeutique adaptee est necessaire.

Oxidative stress (OS) is a keystone in the pathology of the ischemia reperfusion sequence (acute coronary syndromes, cardiac surgery, transplantation). In heart failure, the implication of OS is less understood. This study was intended to evaluate OS in acute heart failure. Criteria for inclusion were consecutive patients hospitalized in our cardiology department for a first pulmonary edema that revealed a dilated cardiomyopathy (DCM). Exclusion criteria included known cardiomyopathy, smoker, acute coronary syndrome, and treatment with angiotensin converting enzyme inhibitors (ACEI) or angiotensin II receptor blockers (ARAII). OS was evaluated in blood samples: thiobarbituric acid-reactive substances (TBARS), total antioxidant status (TAS), plasma alpha-tocopherol, vitamin A, and beta-carotene. Standard biochemical parameters including CRP, fibrinogen, lipid, and creatinine were assayed. Ten patients (80% men, mean age 55.3 +/- 7.9 years) were included and followed during a 6 month period. The etiologies of DCM were alcohol (n = 3), anti-cancer drugs (n = 2), valvulopathies (n = 2), or idiopathic (n = 3). In acute heart failure, TBARS were elevated (1.69 micromol/L; normal value 0.6-4.2 micromol/L) and TAS status was decreased (0.96 mmol/L; normal value 1.3-1.9 pmol/L). OS was more important when patients had atrial or ventricular arrhythmia. Nevertheless, liposoluble antioxidant parameters (beta-carotene, vitamin A, alpha-tocopherol) had a usual value. At the term of the follow-up, patients returned to a stable condition, OS markers revealed normal values, and every Holter ECG showed no supraventricular or ventricular arrhythmias. In acute heart failure, oxygen-free radicals are increased. We thus hypothetized that a modification in OS could be responsible for arrhythmias and complications of acute heart failure.

Xenotransplantation (XT) reveals a growing interest for the treatment of cardiomyopathy. The major barrier is an acute vascular rejection due to an acute humoral rejection. This pathogenesis is a difficult issue and in order to elaborate means for its prevention, we analysed the implication of oxidative stress (OS) on hearts from mini-pigs followed by reperfusion with either autologous or human blood in an attempt to simulate xenotransplantation. About 14 hearts were studied after a Langendorff blood reperfusion: allografts with autologous blood (n = 7) or xenografts with human blood (n = 7). Blood samples were drawn from the coronary sinus to assess ischemia and OS. In xenografts, arrhythmias occurred more frequently (p0.01, left ventricular systolic pressure decreased more significantly (p0.05), thiobarbituric acid-reactive substances concentrations increased at 30 min (0.7 +/- 0.1 vs. 2.4 +/- 0.3 mmol/l; p0.05) while vitamin A levels decreased (p0.05). XT was associated with a significant increase in ischemic injury and OS production. OS might play an eminent role in hyperacute humoral rejection.

Mediastinal irradiation treatment has drastically improved the prognosis for several neoplasms, mostly particularly breast cancer and lymphoma. Nonetheless, the increase in survival is accompanied by side effects, including cardiac complications that often appear much later.This patient with Hodgkin disease received radiation therapy. Complications developed 28 years later and included conduction disorders and chronic pericarditis, first exudative and then constrictive.As the literature shows, different cardiac complications of radiation therapy can follow one another. Attentive long-term monitoring is necessary for appropriate treatment.

Summary Background Much attention is being paid to the education of and provision of medical information to patients, to optimize their understanding and acceptance of their disease. Aims To ascertain the impact of educating recent recipients of an implantable cardioverter defibrillator (ICD) on their perception and acceptance of a home monitoring (HM) system. Methods Questionnaire 1, completed one month after ICD implantation, was designed to assess: the quality of patient preparation for HM; patient comprehension of HM; and patient anxiety experienced during its installation. The comprehension questions were assigned a score of –2 for an incorrect answer, +1 for a correct answer and 0 for neither (total score ranging from –40 to +20). Questionnaire 2, completed six months after ICD implantation, assessed patient acceptance of and anxiety about HM. Results The registry included 571 patients (mean age 63.9 ± 12.8 years; 83% men; 76% of ICDs implanted for primary prevention) followed by HM for 6.2 ± 1.2 months. Questionnaire 1 was completed by 430 (75.3%) patients and questionnaire 2 by 398 (69.7%) patients. Younger patients had a better comprehension of HM than older patients. High-quality training conditions improved the comprehension score, and a positive association was observed between anxiety and acceptance levels and the comprehension score. The 80 ± 20% mean data transmission rate (days of transmission/days of follow-up ratio) was unrelated to the comprehension scores. Conclusion A clear understanding was associated with a higher acceptance of HM, although it was unrelated to the data transmission rate.