Left atrial appendage thrombosis in a patient with Friedreich Ataxia-related cardiomyopathy, left ventricular systolic dysfunction, and atrial fibrillation.

Friedreich ataxia is the most common form of hereditary ataxia. Heart involvement in Friedreich ataxia is common and can include increased left ventricular wall thickness, atrial fibrillation, and in the later stages, a reduction of left ventricular ejection fraction. We present the case of a 45-year-old man with a history of paroxysmal atrial fibrillation and a congestive heart failure, hypertension, age ⩾ 75 years, diabetes mellitus, stroke, vascular disease, age 65-74 years, and female sex (CHA2DS2-VASc) score of only 1 (because of reduced left ventricular ejection fraction) who presented with pneumonia and was also found to have atrial fibrillation with a rapid ventricular response. Despite already being on long-term therapy with a non-vitamin K-antagonist oral anticoagulant, a transesophageal echocardiogram showed a mobile floating thrombus in the left atrial appendage. In accordance with previous necropsy evidence of thrombosis and thromboembolism in Friedreich ataxia subjects who likely have had only non-sex-related CHA2DS2-VASc score ⩽1, this case suggests that the risk of thromboembolism in Friedreich ataxia subjects with atrial fibrillation may not be adequately predicted by the sole CHA2DS2-VASc score.

Normal weight obesity: a risk factor for cardiometabolic dysregulation and cardiovascular mortality.

AIMS: We hypothesized that subjects with a normal body mass index (BMI), but high body fat (BF) content [normal weight obesity (NWO)], have a higher prevalence of cardiometabolic dysregulation and are at higher risk for cardiovascular (CV) mortality. METHODS AND RESULTS: We analysed 6171 subjects >20 years of age from the Third National Health and Nutrition Examination Survey (NHANES III) and the NHANES III mortality study, whose BMI was within the normal range (18.5-24.9 kg/m(2)), and who underwent a complete evaluation that included body composition assessment, blood measurements, and assessment of CV risk factors. Survival information was available for >99% of the subjects after a median follow-up of 8.8 years. We divided our sample using sex-specific tertiles of BF%. The highest tertile of BF (>23.1% in men and >33.3% in women) was labelled as NWO. When compared with the low BF group, the prevalence of metabolic syndrome in subjects with NWO was four-fold higher (16.6 vs. 4.8%, P < 0.0001). Subjects with NWO also had higher prevalence of dyslipidaemia, hypertension (men), and CV disease (women). After adjustment, women with NWO showed a significant 2.2-fold increased risk for CV mortality (HR = 2.2; 95% CI, 1.03-4.67) in comparison to the low BF group. CONCLUSION: Normal weight obesity, defined as the combination of normal BMI and high BF content, is associated with a high prevalence of cardiometabolic dysregulation, metabolic syndrome, and CV risk factors. In women, NWO is independently associated with increased risk for CV mortality.

Device-guided paced breathing in the home setting: effects on exercise capacity, pulmonary and ventricular function in patients with chronic heart failure: a pilot study.

BACKGROUND: Regular slow breathing is known to improve autonomic cardiac regulation and reduce chemoreflex sensitivity in heart failure. We explored the acceptability and usefulness of a device for paced slow breathing at the home setting. METHODS AND RESULTS: In this open pilot study, 24 patients with chronic heart failure (61% males, mean age, 64+/-9 years; New York Heart Association class, 2.81+/-0.01) were randomized to a control group receiving conventional treatment (n=12) or to a group receiving conventional treatment and device-guided paced breathing (n=12). Groups were comparable for age, therapies, and clinical characteristics. They were evaluated at baseline and again after 10 weeks by Doppler echocardiography, pulmonary function, cardiopulmonary stress test, and quality of life (Minnesota Quality of Life questionnaire). The treatment group was instructed to use the equipment for 18 minutes twice daily. The device is a computerized box connected to a belt-type respiration sensor and to headphones; it generates musical tones (based on the user's breathing rate and inspiration ratio), which guide the user to progressively and effortlessly slow his or her breathing rate <10 breaths/min. The treatment group showed high compliance to the device (90% of the prescribed sessions were completed). Blinded analysis of data demonstrated increased ejection fraction and decreased estimated pulmonary pressure in the echocardiograms of the treated group versus controls and favorable changes in New York Heart Association class, Ve/Vco(2), FEV(1), and a quality of life measure, as well (all P<0.05). CONCLUSIONS: This pilot investigation demonstrates that device-guided paced breathing at home is feasible and results in an improvement in clinically relevant parameters for patients with heart failure and systolic dysfunction.

[Disease management for heart failure patients: role of wireless technologies for telemedicine. The ICAROS project]. / Continuità assistenziale nei pazienti con scompenso cardiaco: ruolo delle tecnologie wireless per la telemedicina. II progetto ICAROS.

Healthcare costs for heart failure are increasing. The need for a better care, however, has to be matched with a policy of cost containment. A way to improve the cost-effectiveness of heart failure care is the disease management approach, in which therapy, education and follow-up are tailored for each patient by a multidisciplinary team. Such a complex intervention can be facilitated by the use of telemedicine, which allows the remote control of considerable amounts of clinical data. In Italy, a few studies with telemedicine have been reported. A recent development in this field is represented by the ICAROS project (Integrated Care vs Conventional Intervention in Cardiac Failure Patients: Randomized Open Label Study), whose aim is to improve the clinical and psychological care of heart failure patients employing advanced wireless telecommunication technology. So far, we randomized 60 patients: 30 in usual ambulatory care, 30 in an intensive treatment group. The latter patients were instructed to use a portable computer to get in touch daily with the heart failure clinic and receive feedback instruction for the management of drug therapy and daily problems. At the first year of follow-up, the treatment group showed good compliance to drug prescriptions, and could easily handle the portable computer. The preliminary results of this ongoing study support the feasibility and appropriateness of new technologies for the management of heart failure, even in elderly patients in whom a limited expertise with these appliances could have been anticipated.