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We describe four male patients with wasted-leg syndrome, with predominant asymmetric thigh atrophy and weakness that stabilized after a period of slow progression (follow-up 7-18 years). Two patients had an Indian ethnic background and two were Portuguese, without known Indian ancestry. Other mimicking disorders were excluded, but one Indian patient was later diagnosed with CADASIL. Electromyography (EMG) revealed severe chronic neurogenic changes in proximal leg muscles, and mild changes in distal leg muscles, but EMG of the upper limbs was normal. Upper motor neuron signs were absent clinically and on transcranial magnetic stimulation. This seems to represent a variant of the common wasted-leg syndrome presentation.

Introduction Heart failure with mildly reduced ejection fraction (HFmrEF) has been recognized in the last heart failure guidelines of the European Society of Cardiology, replacing the concept of “midrange ejection fraction”. There is limited data regarding mortality outcomes of HFmrEF compared to heart failure with reduced (HFrEF) and preserved (HFpEF) ejection fraction. Purpose We aimed to evaluate whether HFmrEF has a different prognosis after an acute heart failure (AHF) episode, in a real-world contemporaneous southern European population. Methods We retrospectively studied 1026 patients admitted to our emergency department between November 2016 and December 2017 with discharge diagnosis of AHF. Median follow up was 5 months (IQR 3–11 months). Incidence of rehospitalization, cardiovascular (CV) and all-cause mortality were evaluated through multivariable logistic regression models and by Kaplan-Meyer survival curves. Results From all patients, 782 were categorized in HFrEF (34.1%), HFmrEF (19.4%) and HFpEF (46.4%). There was heterogeneity between groups. Compared to HFrEF, HFmrEF patients were older (80 [74–84] vs. 76 [67–82] years, p Conclusions HFmrEF have a similar prognosis to HFrEF and worse than HFpEF, with more readmissions, CV and all-cause mortality, after an AHF episode. Funding Acknowledgement Type of funding sources: None.

Introduction Cardiopulmonary exercise testing (CPET) is recommended in patients with heart failure (HF) to optimize exercise prescription and as part of the evaluation for heart transplantation. Hemodynamic gain index (HGI) derived from CPET has been proposed as a new marker of risk stratification in general population cohorts. Purpose We aimed to evaluate the prognostic value of HGI in patients with HF. Methods We conducted a single-centre study assessing consecutive patients with HF who underwent CPET from 2013 to 2017. HGI was calculated based on heart rate (HR) and systolic blood pressure (SBP): HGI = [(HRpeak × SBPpeak) − (HRrest × SBPrest)] / (HRrest × SBPrest). Classic and recently proposed variables were collected, including peak O2 uptake (pVO2), minute ventilation–CO2 production (VE/VCO2 slope), circulatory power (CP = pVO2 × peak SBP), and ventilatory power (VP = peak SBP/(VE/VCO2) slope). The primary outcome was a composite of HF hospitalization, heart transplant, and all-cause mortality. Results A total of 212 patients (mean age 55.4±10.9, 76.9% male) were included. Most patients had dilated cardiomyopathy (43.9%) followed by ischaemic aetiology (38.7%), with a mean left ventricle ejection fraction of 29±13%. The most used exercise protocol was the modified Naughton (76.6%), followed by the original Naughton (18.7%), and Bruce (4.8%). Mean pVO2 was 16.7±5.9mL O2 kg–1 min–1 and median VE/VCO2 slope was 37.5 [32.7–44.3]. Mean VP was 3.46±1.31mmHg while median CP was 1927 [1427–2697]mmHg min/mL/kg. Mean HGI was 0.90±0.5 bpm/mmHg. Despite weak, there were significant positive correlations between HGI and mean pVO2 (rs=0.55, p1.02). After a median follow-up of 71 [49–81] months, the primary outcome occurred in 66.0% of patients (rehospitalization, heart transplant, and all-cause death occurred in 56.1%, 25.9%, and 32.5%, respectively). In the T1 group, the multivariable-adjusted odds ratio (OR) for the primary outcome was 3.73 (95% CI 1.97–7.06, p Conclusion HGI is inversely associated with the composite of HF hospitalization, heart transplant, and all-cause mortality in patients with heart failure, enhancing the role of CPET in risk stratification. Funding Acknowledgement Type of funding sources: None.

Background Rotational atherectomy (RA) enables percutaneous coronary intervention (PCI) for complex and calcified coronary lesions, despite limited data regarding long-term outcomes. Purpose We aimed to characterize success rate, safety, and long-term outcomes with this technique in a single-centre with over a decade of experience. Methods We retrospectively analysed all cases of RA performed in a single-centre between January 2009 and December 2020. Angiographic success was defined as the presence of less than 30% residual stenosis and grade 3 TIMI (thrombolysis in myocardial infarction) flow. Complications included coronary perforation or dissection, burr entrapment, wire fracture, slow flow/no-reflow, side branch occlusion, peri-procedural myocardial infarction (MI), or death. Long-term major adverse cardiovascular (CV) events (MACE) included CV and all-cause death, MI, and target vessel revascularization. Results Of the total of 14,527 PCI performed, 410 procedures (2.8%) included RA. Most patients were male (74.0%) with mean age of 72.3±9.3 years. There was high prevalence of hypertension (85.65%) and dyslipidaemia (74.2%) which increased significantly over time (p trend=0.008 and p trend Conclusions RA is an effective and safe technique, with a declining rate of peri-procedural complications along with technical developments. The rate of long-term events is concordant with the CV risk profile and coronary lesion complexity. Funding Acknowledgement Type of funding sources: None.

Objective Studies have been conducted to determine the causal factors and clinical consequences of non-adherence to treatment in systemic lupus erythematosus (SLE). However, no interventions have been performed to increase drug adherence. Our objective was to assess the effectiveness of pharmaceutical care (PC) for drug treatment adherence in lupus nephritis (LN). Methods This was a randomized clinical trial (pragmatic trial) in patients with LN in Rio de Janeiro, Brazil, allocated in two groups: an intervention group (Dader Method for PC) and a control group (institution's usual care). Drug treatment adherence was measured by the combination of five questions normally used in clinical practice. Results A total of 131 patients were randomized, and 122 completed the study, with a mean follow-up of 12.7 months and use of six drugs per day and 10–12 doses per day. Low adherence was observed at baseline (intervention group: 30%; control group: 29%). PC showed 27% effectiveness (95% confidence interval (CI) –6% to 50%) in the intention to treat analysis and 31% (95% CI 0–52%) in per protocol analysis, considering all drugs. As for adherence to specific drugs for SLE, effectiveness of PC was 64% (95% CI 34–80%) with intention-to-treat analysis and 62% (95% CI 32–79%) in per protocol analysis. Conclusions PC was effective for increasing drug treatment adherence in SLE. The detailed account provided by the Dader Method of the difficulties with patients' drug therapy proved invaluable to approach non-adherence.

Background Treatment adherence is a primary determinant of the success and effectiveness of healthcare. Lack of adherence can lead to treatment failure and death. Although studies have shown that pharmaceutical intervention can improve drug treatment for patients with chronic diseases, studies on pharmaceutical care are not only inconsistent, they are scarce and limited to developed countries, include few patients, and are not studied in randomized clinical trials. Systemic lupus erythematosus is an autoimmune disease with high hospitalization and case-fatality rates. The adherence rate is low (31.7 %) in this group of patients in Brazil, and drug treatment for the disease is complex. Our objective is to evaluate the effectiveness of pharmaceutical care in drug treatment adherence in patients with systemic lupus erythematosus treated at a rheumatology outpatient clinic in Rio de Janeiro, Brazil. Methods A randomized clinical trial (pragmatic trial) will be conducted. Adult participants (women) from a public hospital in Rio de Janeiro with a diagnosis of systemic lupus erythematosus will be followed for 12 months. A total of 120 patients will be randomized to two groups: intervention (Dader method for pharmaceutical care) and control (health/dietary counseling and risk reduction). The primary outcome will be drug treatment adherence evaluated by the eight-item Morisky Medication Adherence Scale. Secondary outcomes will be clinical improvement and quality of life. Discussion Patients with systemic lupus erythematosus present with low treatment adherence, thus justifying the mobilization of human resources to optimize their clinical management. Despite the proven effectiveness of pharmaceutical care for various diseases, there are still no studies evaluating its effectiveness in systemic lupus erythematosus. Our hypothesis is that the intervention will also be effective in this patient group. Trial registration ClinicalTrials.gov identifier: NCT02330250. Electronic supplementary material The online version of this article (doi:10.1186/s13063-016-1317-1) contains supplementary material, which is available to authorized users.

The objectives of this study were to measure the prevalence of adherence to drug treatment and analyze associations with characteristics pertaining to the treatment, disease, health professionals and services, and socio-demographic issues in patients with systemic lupus erythematosus (SLE) in the city of Rio de Janeiro, Brazil. A sample of 246 women with SLE was analyzed. The data were collected through individual interviews and a review of patient charts. Adherence was estimated according to the Morisk criteria, and the associated factors were analyzed by hierarchical modeling. The percentage of patients classified as adherent to treatment was 31.7%. The reasons cited for non-adherence were: carelessness with drug administration times (52.43%), forgetfulness (38.21%), adverse drug reaction (13.8%), and interruption of treatment due to improvement in symptoms (7.72%). Factors associated with adherence were: behavior towards the presence of adverse drug reaction, hematological alterations, presence of mucocutaneous manifestations, legibility of the medical prescription, schooling, and family support. The study concludes that adherence to drug treatment in SLE is a complex and multifactorial phenomenon, and the results corroborate findings from studies conducted in developed countries. The hierarchical modeling proved to be a good alternative for evaluating adherence, since it allowed visualizing the various stages in the analysis.