Aims To provide the first systematic analysis of the burden and underlying causes of heart failure (HF) in 195 countries and territories from 1990 to 2017. Methods and results We collected detailed information on prevalence, years lived with disability (YLDs), and underlying causes of HF from the Global Burden of Disease study 2017. Numbers and age-standardized rates of HF prevalence and YLDs were compared by age, sex, socio-demographic index (SDI), and location. The proportions of HF age-standardized prevalence rates due to 23 underlying causes were also presented. Globally, the age-standardized prevalence and YLD rates of HF in 2017 were 831.0 and 128.2 per 100 000 people, a decrease of −7.2% and −0.9% from 1990, respectively. Nevertheless, the absolute numbers of HF prevalent cases and YLDs have increased by 91.9% and 106.0% from 1990, respectively. There is significant geographic and socio-demographic variation in the levels and trends of HF burden from 1990 to 2017. Among all causes of HF, ischaemic heart disease accounted for the highest proportion (26.5%) of age-standardized prevalence rate of HF in 2017, followed by hypertensive heart disease (26.2%), chronic obstructive pulmonary disease (23.4%). Conclusion HF remains a serious public health problem worldwide, with increasing age-standardized prevalence and YLD rates in countries with relatively low SDI. More geo-specific strategies aimed at preventing underlying causes and improving medical care for HF are warranted to reduce the future burden of this condition.

Aims Novel therapies are needed for recurrent pericarditis, particularly when corticosteroid dependent and colchicine resistant. Based on limited data, interleukin-1 blockade with anakinra may be beneficial. The aim of this multicentre registry was to evaluate the broader effectiveness and safety of anakinra in a ‘real world’ population. Methods and results This registry enrolled consecutive patients with recurrent pericarditis who were corticosteroid dependent and colchicine resistant and treated with anakinra. The primary outcome was the pericarditis recurrence rate after treatment. Secondary outcomes included emergency department visits, hospitalisations, corticosteroid use and adverse events. Among 224 patients (46 ± 14 years old, 63% women, 75% idiopathic), the median duration of disease was 17 months (interquartile range 9–33). Most patients had elevated C-reactive protein (91%) and pericardial effusion (88%). After a median treatment of 6 months (3–12), pericarditis recurrences were reduced six-fold (2.33–0.39 per patient per year), emergency department admissions were reduced 11-fold (1.08–0.10 per patient per year), hospitalisations were reduced seven-fold (0.99–0.13 per patient per year). Corticosteroid use was decreased by anakinra (respectively from 80% to 27%; P < 0.001). No serious adverse events occurred; adverse events consisted mostly of transient skin reactions (38%) at the injection site. Adverse events led to discontinuation in 3%. A full-dose treatment duration of over 3 months followed by a tapering period of over 3 months were the therapeutic schemes associated with a lower risk of recurrence. Conclusion In patients with recurrent pericarditis, anakinra appears efficacious and safe in reducing recurrences, emergency department admissions and hospitalisations.

Aims Diabetes mellitus (DM) aggravates the clinical features of ischaemic and hypertensive heart diseases and worsens the prognosis of heart failure patients. Hypertrophic cardiomyopathy (HCM) and diabetes coexist fairly frequently in elderly patients but the impact of DM on the clinical phenotype of HCM is yet unknown. We sought to describe if predominant features of heart failure in DM patients exist independently in HCM. Methods and results We reviewed clinical characteristics of 937 patients, age ≥40, diagnosed with HCM, from two tertiary medical centres in Spain and Israel. A propensity score matched cohort of 294 patients was also analysed. Our cohort comprised 102 HCM patients with diabetes (8.7%). Patients with DM were older at diagnosis {median 56 [interquartile range (IQR) 47–67] vs. 53 (IQR 43–63), P = 0.02} and had a higher prevalence of comorbidities. Hypertrophic cardiomyopathy patients with DM had a higher prevalence of diastolic dysfunction, pulmonary hypertension, significant mitral regurgitation, and pacemaker implantation. Hypertrophic cardiomyopathy patients with DM had a higher New York Heart Association (NYHA) class (P &lt; 0.001) and lower exercise capacity [7.0 METS (IQR 5.0–10.0) vs. 9.0 METS (IQR 6.6–11.0), P = 0.002]. These findings were independent of age, gender, country of origin, hypertension, and coronary artery disease. Patients with diabetes had a significantly higher 15-year mortality (22% vs. 15%, P = 0.03), with no differences in sudden cardiac death, appropriate implanted cardioverter-defibrillator therapy, or heart transplantation. Conclusion Hypertrophic cardiomyopathy patients with diabetes are older and have a higher cardiovascular risk profile. They have a lower functional capacity and more heart failure symptoms due to diastolic dysfunction.

Background - The X-linked Danon disease (DD) manifests by severe cardiomyopathy, myopathy, and neuropsychiatric problems. We designed this registry to generate a comprehensive picture of clinical presentations and outcome of patients with Danon disease (DD) in cardiomyopathy centers throughout Europe. Methods - Clinical and genetic data were collected in 16 cardiology centers from 8 European countries. Results - The cohort comprised 30 male and 27 female patients. The age at diagnosis was birth to 42 yr. in males and 2-65 in females. Cardiac involvement was observed in 96%. Extracardiac manifestations were prominent in males but not in females. Left ventricular (LV) hypertrophy was reported in 73% of male and 74% of female patients. LV systolic dysfunction was reported in 40% of males (who had LVEF 34±11%) and 59% of females (LVEF 28±13%). The risk of arrhythmia and heart failure (HF) were comparable among genders. The age of first HF hospitalization was lower in males (18 ± 6 vs. 28 ±17 yr., p<0.003). HF was the leading cause of death (10/17, 59%), and LV systolic dysfunction predicted an adverse outcome. Eight males and 8 females (28%) underwent heart transplantation or received a left ventricular assist device (LVAD). Our cohort suggests better prognosis of female compared to male heart transplant recipients. Conclusions - DD presents earlier in males than females and runs a malignant course in both genders, due to cardiac complications. Cardiomyopathy features: heart failure and arrhythmia, are similar among the genders. Clinical diagnosis and management is extremely challenging in females due to phenotypic diversity and absence of extracardiac manifestations.

Background/ObjectiveObjective assessment tools for patients' frailty are lacking. Such tools would have been highly valuable for assessment of candidates for cardiac rehabilitation programs. Low ALT (Alanine aminotransferase) values were recently shown to be a promising parameter for objective, quantitative frailly assessment.MethodsThis was a retrospective study of patients participating in a cardiac rehabilitation program.ResultsPatients with lower ALT activity levels at the initiation of rehabilitation program had lower estimated METs values (6.86 vs. 7.73; p < 0.001), shorter stress test duration (06:41 vs. 07:44 min; p < 0.001), higher resting heart rate (72 ± 13 vs. 70 ± 13 BPM; p = 0.01) and lower heart rate reserve (49 ± 24 vs. 54 ± 24; p < 0.001). Multivariate linear modeling demonstrated that ALT values were Independent determinants of baseline exercise capacity (expressed in METs).ConclusionLower ALT values, measured prior to the initiation of cardiac rehabilitation programs may indicate frailty of patients and be indicative for poor rehabilitation outcomes. Further, prospective studies should assess the potential correlation between ALT values and rehabilitation efficiency. We aimed to assess the potential correlation between the baseline ALT values and the baseline exercise capacity, as expressed in METs (Metabolic equivalent of tasks). 3806 patients were included in our study.

To evaluate cystourethrography in the era of fetal screening, we evaluated retrospectively the clinical and imaging findings of all children up to the age of 1 year who underwent a cystourethrogram in a 5-year period (1987-1992). There were 292 children, 64 neonates (< 1 month, 51 boys, 13 girls) and 228 infants (1 month-1 year, 111 boys, 117 girls). Hydronephrosis detected prenatally and confirmed after birth by US was the indication for cystourethrography in 88 children (72 boys, 16 girls). This 4.5 to 1, male to female ratio is very unusual compared to children with urinary tract infection, the great majority of whom are girls. The findings based on imaging studies in these 88 children with hydronephrosis included 31 with vesicoureteral reflux (VUR) (in 4 this was secondary), 23 with obstruction at the ureteropelvic junction (UPJ), 13 with multicystic dysplastic kidney, 2 with obstruction at the ureterovesical junction, 1 with ectopic ureterocele and 1 with posterior urethral valves, both the latter without reflux. Eleven children had normal postnatal studies and six children were lost to follow-up. Urinary tract infection (UTI) was the indication in 163 children (62 boys, 101 girls). Forty-one children were examined for other causes. Although most cases of hydronephrosis were detected on fetal screening, UTI was still the most common indication for cystourethrography and some significant abnormalities were found in these symptomatic children.

QTc duration anomalies are associated with worse short- and medium-term prognosis and may act as a marker for more severe clinical sequelae.

Objective: To estimate the burden of myocarditis (MC), alcoholic cardiomyopathy (AC), and other cardiomyopathy (OC) for 195 countries and territories from 1990 to 2017.Methods: We collected detailed information on MC, AC, and OC between 1990 and 2017 from the Global Burden of Disease study 2017, which was designed to provide a systematic assessment of health loss due to diseases and injuries in 21 regions, covering 195 countries and territories. Estimates of MC, AC, and OC burden were produced using a standard Cause of Death Ensemble model and a Bayesian mixed-effects meta-regression tool, and included prevalence, deaths, years lived with disability (YLDs), and years of life lost (YLLs). All estimates were presented as counts, age-standardized rates per 100,000 people and percentage change, with 95% uncertainty intervals (UIs).Results: Worldwide, there were 1.80 million (95% UI 1.64–1.98) cases of MC, 1.62 million (95% UI 1.37–1.90) cases of AC and 4.21 million (95% UI 3.63–4.87) cases of OC, contributing to 46,486 (95% UI 39,709–51,824), 88,890 (95% UI 80,935–96,290), and 233,159 (95% UI 213,677–248,289) deaths in 2017, respectively. Furthermore, globally, there were 131,376 (95% UI 90,113–183,001) YLDs and 1.26 million (95% UI 1.10–1.42) YLLs attributable to MC, 139,087 (95% UI 95,134–196,130) YLDs and 2.84 million (95% UI 2.60–3.07) YLLs attributable to AC, and 353,325 (95% UI 237,907–493,908) YLDs and 5.51 million (95% UI 4.95–5.99) YLLs attributable to OC in 2017. At the national level, the age-standardized prevalence rates varied by 10.4 times for MC, 252.6 times for AC and 38.1 times for OC; the age-standardized death rates varied by 43.9 times for MC, 531.0 times for AC and 43.3 times for OC; the age-standardized YLD rates varied by 12.4 times for MC, 223.7 times for AC, and 34.1 times for OC; and the age-standardized YLL rates varied by 38.4 times for MC, 684.8 times for AC, and 36.2 times for OC. Between 1990 and 2017, despite the decreases in age-standardized rates, the global numbers of prevalent cases, deaths, YLDs, and YLLs have increased for all the diseases.Conclusion: Accurate assessment of the burden of MC, AC, and OC is essential for formulating effective preventative prevention and treatment programs and optimizing health system resource allocation. Our results suggest that MC, AC, and OC remain important global public health problems with increasing numbers of prevalent cases, deaths, YLDs, and YLLs over the past decades, and there are significant geographic variations in the burden of these diseases. Further research is warranted to expand our knowledge of potential risk factors and to improve the prevention, early detection and treatment of these diseases.