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Aims The Cardiomyopathy Registry of the EURObservational Research Programme is a prospective, observational, and multinational registry of consecutive patients with four cardiomyopathy subtypes: hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), arrhythmogenic right ventricular cardiomyopathy (ARVC), and restrictive cardiomyopathy (RCM). We report the baseline characteristics and management of adults enrolled in the registry. ................................................................................................................................................................................................... Methods and results A total of 3208 patients were enrolled by 69 centres in 18 countries [HCM (n = 1739); DCM (n = 1260); ARVC (n = 143); and RCM (n = 66)]. Differences between cardiomyopathy subtypes (P < 0.001) were observed for age at diagnosis, history of familial disease, history of sustained ventricular arrhythmia, use of magnetic resonance imaging or genetic testing, and implantation of defibrillators. When compared with probands, relatives had a lower age at diagnosis (P < 0.001), but a similar rate of symptoms and defibrillators. When compared with the Long-Term phase, patients of the Pilot phase (enrolled in more expert centres) had a more frequent rate of familial disease (P < 0.001), were more frequently diagnosed with a rare underlying disease (P < 0.001), and more frequently implanted with a defibrillator (P = 0.023). Comparing four geographical areas, patients from Southern Europe had a familial disease more frequently (P < 0.001), were more frequently diagnosed in the context of a family screening (P < 0.001), and more frequently diagnosed with a rare underlying disease (P < 0.001). ................................................................................................................................................................................................... Conclusion By providing contemporary observational data on characteristics and management of patients with cardiomyopathies, the registry provides a platform for the evaluation of guideline implementation. Potential gaps with existing recommendations are discussed as well as some suggestions for improvement of health care provision in Europe.

Background Although acute coronary syndrome ( ACS ) mainly occurs in patients >50 years, younger patients can be affected as well. We used an age cutoff of 45 years to investigate clinical characteristics and outcomes of “young” patients with ACS . Methods and Results Between October 2010 and April 2016, 14 931 patients with ACS were enrolled in the ISACS‐TC (International Survey of Acute Coronary Syndromes in Transitional Countries) registry. Of these patients, 1182 (8%) were aged ≤45 years (mean age, 40.3 years; 15.8% were women). The primary end point was 30‐day all‐cause mortality. Percentage diameter stenosis of ≤50% was defined as insignificant coronary disease. ST‐segment–elevation myocardial infarction was the most common clinical manifestation of ACS in the young cases (68% versus 59.6%). Young patients had a higher incidence of insignificant coronary artery disease (11.4% versus 10.1%) and lesser extent of significant disease (single vessel, 62.7% versus 46.6%). The incidence of 30‐day death was 1.3% versus 6.9% for the young and older patients, respectively. After correction for baseline and clinical differences, age ≤45 years was a predictor of survival in men (odds ratio, 0.24; 95% confidence interval, 0.10–0.58), but not in women (odds ratio, 1.35; 95% confidence interval, 0.50–3.62). This pattern of reversed risk among sexes held true after multivariable correction for in‐hospital medications and reperfusion therapy. Moreover, younger women had worse outcomes than men of a similar age (odds ratio, 6.03; 95% confidence interval, 2.07–17.53). Conclusion ACS at a young age is characterized by less severe coronary disease and high prevalence of ST‐segment–elevation myocardial infarction. Women have higher mortality than men. Young age is an independent predictor of lower 30‐day mortality in men, but not in women. Clinical Trial Registration URL: http://clinicaltrials.gov/ . Unique identifier: NCT01218776.

Background Women with ST ‐segment–elevation myocardial infarction ( STEMI ) have higher mortality rates than men. We investigated whether sex‐related differences in timely access to care among STEMI patients may be a factor associated with excess risk of early mortality in women. Methods and Results We identified 6022 STEMI patients who had information on time of symptom onset to time of hospital presentation at 41 hospitals participating in the ISACS ‐TC (International Survey of Acute Coronary Syndromes in Transitional Countries) registry ( NCT 01218776) from October 2010 through April 2016. Patients were stratified into time‐delay cohorts. We estimated the 30‐day risk of all‐cause mortality in each cohort. Despite similar delays in seeking care, the overall time from symptom onset to hospital presentation was longer for women than men (median: 270 minutes [range: 130–776] versus 240 minutes [range: 120–600]). After adjustment for baseline variables, female sex was independently associated with greater risk of 30‐day mortality (odds ratio: 1.58; 95% confidence interval, 1.27–1.97). Sex differences in mortality following STEMI were no longer observed for patients having delays from symptom onset to hospital presentation of ≤1 hour (odds ratio: 0.77; 95% confidence interval, 0.29–2.02). Conclusions Sex difference in mortality following STEMI persists and appears to be driven by prehospital delays in hospital presentation. Women appear to be more vulnerable to prolonged untreated ischemia. Clinical Trial Registration URL : https://www.clinicaltrials.gov/ . Unique identifier: NCT01218776.

Objectives Although most of the adaptive changes in the heart induced by intense and prolonged sporting activity have now been thoroughly described, especially with regard to the left ventricle, variation over time requires clarification, particularly in terms of the response of the myocardium to seasonal variation in training intensity and duration. The aim of this study was to examine whether preparticipation physical examination and echocardiographic screening related to left ventricle (LV) remodellingremodeling in elite international level athletes change during three consequence 6 months follow-up periods. Methods 140 men elite international level athletes were screened in accordance with the recommendations of the ESC with a history and physical examination, electrocardiography (ECG), followed by standard transthoracic echocardiogram. All examination (resting, maximal and recovery heart rate-HR and blood pressure-BP) and echocardiographic parameters related to left ventricle remodellingremodeling (left ventricle mass index-LVMI; endsystolic dimension-ESD; enddyastolic dimension-EDD; septum-IVS; posterior wall-PW and relative wall thickness-RWT) were compared throught three consequence preparticipation check-ups every six months. All echocardiographic findings were adjusted to BSA. Results The players mean age was 23.3 ± 3.2 years. Durring the follow-up period, all athletes had no evidence of cardiovascular disease in medical hystory, physical examination and ECG on preparticipation check-ups. Resting and recovery HR were signifficantly lower after 1.5 yr follow-up (p Conclusions Our data demonstrated that the differences between the first and second assessments are slight, and only after after one year follow-up period there is a signifficant LV remodelling, which can be documented through IVS, PW diameters and LVMI and RWT. Another important conclusion that can be drawn from the study is that additional adaptive changes take place independently from the course of the season, and mostly depending on cumulative effect of previous physical activity. Periodical one year physical and echocardiographical screening of elite athletes is a non-invasive and recommended tool with the potential to increase the screening accuracy for detection of changes in heart dimensions during competitive season. References Ghani S, Papadakis M, Kemp S, Zaidi A, Sheikh N, Gati S, et al. Results of a nationally implemented de novo cardiac screening programme in elite rugby players in England. Br J Sports Med 2016 Jun. [Epub ahead of print] Lavie CJ, Harmon KG. Routine ECG Screening of Young Athletes: Can This Strategy Ever Be Cost Effective? J Am Coll Cardiol 2016 Aug;68(7):712–4.

Objectives This study aimed to evaluate the association of ACE insertion/deletion (I/D) and ACTN3 R577X polymorphisms with resting, maximal and recovery blood pressure (BP) and left ventricular hypertrophy measured by left ventricular mass index (LVMI) in elite athletes. Methods A group of 107 white, healthy, elite male athletes, aged between 20 and 35 yr, enrolled in this study. All of them participated either in sprint/power sports (n = 17), endurance (n = 36) or in mixed sports (n = 54). HRM method has been developed to differentiate between variant alleles of ACE and ACTN3 genes (Figures 1 and 2). Results No significant difference was found in the ACE and ACTN3 genotypes or allele frequencies distribution between sprint/power, endurance or mixed sports athletes (p > 0.05). Also, neither insertion in the ACE gene, nor nonsense mutation in the the ACTN3 gene had a significant effect on resting and maximal BP. Sport activity, but not the analysed polymorphisms influence resting diastolic, maximal systolic and recovery systolic BP (p 0.05). In relation to maximal BP, decrease in systolic BP at 3 min of recovery was higher in ACE II/ID group compared to ACE DD in all sport groups (endurance, sprint/power and mixed: 78 vs. 86%, 79 vs. 83%, 67 vs. 78%, respectively, p 0.05). Conclusion These data show that LVMI as a marker of LVH depends significantly on the interaction between ACE polymorphism and the type of sport activity. References Gunel T, Gumusoglu E, Hosseini MK, Yilmazyildirim E, Dolekcap I, Aydinli K. Effect of angiotensin I-converting enzyme and α-actinin 3 gene polymorphisms on sport performance. Mol Med Rep 2014 Apr;9(4):1422–6. Ma F, Yang Y, Li X, Zhou F, Gao C, Li M, Gao L. The association of sport performance with ACE and ACTN3 genetic polymorphisms: a systematic review and meta-analysis. PLoS One 2013;8(1):e54685. Saber-Ayad MM, Nassar YS, Latif IA. Angiotensin-converting enzyme I/D gene polymorphism affects early cardiac response to professional training in young footballers. J Renin Angiotensin Aldosterone Syst 2014;15(3):236–42.

Introduction: The use of digitalis has been plagued by controversy since its initial use. Previous observations have shown that digoxin can be safely used in patients with chronic heart failure (HF), but concern remains about the effects of digoxin on mortality in patients with incident HF in the acute setting, especially in those with myocardial infarction. Hypothesis: We aimed to determine the relationship between digoxin use and outcomes in hospitalized patients with acute coronary syndromes (ACS) complicated by HF accounting for sex difference and prior heart diseases. Methods: We analyzed information on 88,774 patients presenting with acute HF (Killip class ≥2) in the International Survey of Acute Coronary Syndromes (ISACS)-Archives (NCT04008173) registry, 4,722 (18.7%) who received digoxin on hospital admission. Patients with pre-existing nonischemic congestive cardiomyopathy and without HF were excluded, leaving a final study population of 25,187 patients. The main outcome measure was all-cause 30-day mortality. Estimates were evaluated by inverse probability of treatment weighting models. Results: Women who received digoxin had a higher rate of death than women who did not receive it (33.8% vs. 29.2 %; relative risk [RR] ratio:1.24;95 % confidence interval [CI]: 1.12-1.37). Similar odds for mortality with digoxin were observed in men (28.5 % vs. 24.9 %; RR ratio 1.20; 95% CI:1.10-1.32). Comparable results were obtained in patients with no prior coronary heart disease (RR ratios:1.26; 95 % CI: 1.10 to 1.45 in women and RR:1.21; 95 % CI: 1.06 to 1.39 in men) and those in sinus rhythm at admission (RR ratios:1.34; 95% CI 1.15 to 1.54 in women and 1.26; 95% CI 1.10 to 1.45 in men). Conclusions: Digoxin therapy is associated with an increased risk of early death among women and men with ACS complicated by HF. This finding highlights the need for re-examination of digoxin use in the clinical setting of ACS

Background: Limited data are available on the outcome of primary percutaneous coronary intervention (PCI) in octogenarian patients, as the elderly are under-represented in randomized trials. This study aims to provide insights on clinical characteristics, management and outcome of the elderly and very elderly presenting with STEMI. Methods: 2225 STEMI patients >= 70 years old (mean age 76.8 +/- 5.1 years and 53.8% men) were admitted into the network of the ISACS-TC registry. Of these patients, 72.8% were >= 70 to 79 years old (elderly) and 27.2% were >= 80 years old (very-elderly). The primary end-point was 30-day mortality. Results: Thirty-day mortality rates were 13.4% in the elderly and 23.9% in the very-elderly. Primary PCI decreased the unadjusted risk of death both in the elderly (OR: 0.32, 95% CI: 0.24-0.43) and very-elderly patients (OR: 0.45, 95% CI 0.30-0.68), without significant difference between groups. In the very-elderly hypertension and Killip class >= 2 were the only independent factors associated with mortality; whereas in the elderly female gender, prior stroke, chronic kidney disease and Killip class >= 2 were all factors independently associated with mortality. Factors associated with the lack of use of reperfusion were female gender and atypical chest pain in the very-elderly and in the elderly; in the elderly, however, there were some more factors, namely: history of diabetes, current smoking, prior stroke, Killip class >= 2 and history chronic kidney disease. Conclusions: Age is relevant in the prognosis of STEMI, but its importance should not be considered secondary to other major clinical factors. Primary PCI appears to have beneficial effects in the octogenarian STEMI patients. (C) 2016 Published by Elsevier Ireland Ltd.

Background: There is conflicting information about sex differences in presentation, treatment, and outcome after acute coronary syndromes (ACS) in the era of reperfusion therapy and percutaneous coronary intervention. The aim of this study was to examine presentation, acute therapy, and outcomes of men and women with ACS with special emphasis on their relationship with younger age ( lt = 65 years). Methods: From January 2010 to June 2015, we enrolled 5140 patients from 3 primary PCI capable hospitals. Patients were registered according to the International Survey of Acute Coronary Syndrome in Transitional Countries (ISACS-TC) registry protocol (ClinicalTrials.gov: NCT01218776). The primary outcome was the incidence of in-hospital mortality. Results: The study population was constituted by 2876 patients younger than 65 years and 2294 patients older. Women were older than men in both the young (56.2 +/- 6.6 vs. 54.1 +/- 7.4) and old (74.9 +/- 6.4 vs. 73.6 +/- 6.0) age groups. There were 3421 (66.2%) patients with ST elevation ACS (STE-ACS) and 1719 (33.8%) patients without ST elevation ACS (NSTE-ACS). In STE-ACS, the percentage of patients who failed to receive reperfusion was higher in women than in men either in the young (21.7% vs. 15.8%) than in the elderly (35.2% vs. 29.6%). There was a significant higher mortality in women in the younger age group (age-adjusted OR 1.52, 95% CI: 1.01-2.29), but there was no sex difference in the older group (age-adjusted OR 1.10, 95% CI: 0.87-1.41). Significantly sex differences in mortality were not seen in NSTE-ACS patients. Conclusions: In-hospital mortality from ACS is not different between older men and women. A higher short-term mortality can be seen only in women with STEMI and age of 65 or less.

Background: Although coronary heart disease (CHD) mainly occurs in patients over the age of 50, younger patients can be affected as well. Most studies have used an age cut-off of 45 years to define “young” patients with CHD or acute coronary syndrome (ACS). We used this definition to investigate clinical characteristics and outcomes of ACS young patients in a large international cohort. Methods: Between 2010 and April 2016, 14931 ACS patients were enrolled in the ISACS-TC registry (ClinicalTrials.gov NCT01218776). Of these patients, 1182 (8%) were aged ≤ 45 years old (mean age 40.3 yrs, 15.8% female). The primary end-point was STEMI as index event and 30 day all cause mortality. Percent diameter stenosis of 50% or less were defined as insignificant coronary disease. Results: ST segment elevation myocardial infarction (STEMI) is the most common clinical manifestation of ACS in the younger cases (68 vs 59.6%). Younger patients had a higher incidence of insignificant coronary disease (7.6 vs 5.4%) and single vessel disease (67 vs 52.5%). Conversely, three-vessel disease was less common (7.7 vs 16.3%) in the younger patients. Smoking was the most important risk factor in this population (61.9 vs 34.9%). Predictors of ACS in the young population included male sex (OR 1.84), smoking habit (OR 2.29), family history of CHD (OR 1.72) and higher BMI (OR 1.05). Thirty day unadjusted day survival rates were 98.6 vs 93.1% for young and older patients. After adjusting for baseline characteristics, medications at admission and invasive procedures, age ≤ 45 years old was a predictor of survival in men (OR 0.19, 95% CI 0.07-0.48), but not in women (OR 0.85, 95% CI 0.36-2.04). Younger women had worse outcomes than men of a similar age (OR 5.85, 95% CI 1.92-17.77). Conclusions: ACS at a young age is characterized by less severe coronary disease and worse clinical presentation. Women have higher mortality than men. Factors underlying ACS in young patients and higher mortality rates in female sex warrant further investigation.