No abstract

Corrigendum to: 2014 ESC Guidelines on the diagnosis and treatment of aortic diseases [Eur Heart Journal (2014) 35, 2873–2926,doi:10.1093/eurheartj/ehu281]. In Table 3, the radiation for MRI is “0” and not “-“. The corrected table is shown below.

Aims: This prospective, non-randomised, observational study conducted in Europe was designed in order to assess the long-term safety and efficacy of the Parachute device in ischaemic heart failure subjects as a result of left ventricle remodelling after anterior wall myocardial infarction. Methods and results:One hundred subjects with New York Heart Association Class II-IV ischaemic heart failure (HF), ejection fraction (EF) between 15% and 40%, and dilated akinetic or dyskinetic anteriorapical wall without the need to be revascularised were enrolled. The primary safety endpoint was procedural-or device-related major adverse cardiac cerebral events (MACCE). The secondary safety endpoint was the composite of mortality and morbidity. Secondary efficacy endpoints included haemodynamic measurements determined by echocardiography, LV volume indices, and assessment of functional improvement measured by a standardised six-minute walk test. Of the 100 subjects enrolled, device implantation was successful in 97 (97%) subjects. The one-year rates of the primary and secondary safety endpoints were 7% and 32.3%, respectively. The secondary endpoints, LV volume reduction (p<0.0001) and six-minute walk distance improvement (p<0.01), were achieved. Conclusions:The favourable outcomes observed in this high-risk population provide reassuring safety and efficacy data to support adoption of this technology as a therapeutic option for HF subjects. Clinical registration: www.clinicaltrials.gov numbers are NCT01297296 (PARACHUTE III) and NCT01286116 (cohort B subjects). KEYWORDS• ischaemic heart failure • left ventricle remodelling • structural heart

In patients with recurrent unexplained syncope psychiatric alteration is common. However, patients seldom accepted a psychiatric evaluation and treatment.

We report six patients with Emery-Dreifuss muscular dystrophy (EDMD) and four patients including one female with EDMD phenotype (EDMDP). This series includes one sporadic case who had previously been reported in this journal under the diagnosis of "rigid spine syndrome" in 1977. Time of observation ranged from three to ten years. Detailed cardiological assessment was performed in all patients, skeletal muscle biopsies were obtained from 9 out of 10 and cardiac muscle biopsies from 2 out of 10 patients. One patient showed evidence of cardiomyopathy in the absence of clinically apparent neuromuscular disease and one sibling of another EDMD patient reportedly had a similar combination of symptoms which, to our knowledge, has not yet been reported. Cardiac involvement was found to consist of four independent, albeit often combined features: 1) impairment of impulse generating cells; 2) conduction defects with atrial preponderance; 3) increased atrial and ventricular heterotopia; and 4) functional impairment of ventricular myocardium. Ventricular involvement as apparent from ventricular heterotopia, abnormal enddiastolic diameter, decrease of contractility and/or morphological evidence of ventricular myocardial disease was found in 7 out of 10 patients and confirmed by myocardial histopathology in two EDMD patients. In one myocardial biopsy extensive accumulations of intermediate filaments were observed, a rare finding, which has not been linked to EDMD before. Skeletal muscle biopsies showed evidence of myopathy throughout but several equivocal features such as fibre type grouping in EDMD and fibre type disproportion in EDMDP were also observed. The variability of clinical manifestation of both cardiac and neuromuscular disease encompassed a broader spectrum than apparent from the literature. The consequences for the inherent differential diagnosis are discussed.