Dilated cardiomyopathy (DCM) is a heterogenous group of disorders characterised by left ventricular dilatation and dysfunction, in the absence of factors affecting loading conditions such as hypertension or valvular disease, or significant coronary artery disease. The prevalence of idiopathic DCM is estimated between 1:250 and 1:500 individuals. Determining the aetiology of DCM can be challenging, particularly when evaluating an individual and index case with no classical history or investigations pointing towards an obvious acquired cause, or no clinical clues in the family history to suggest a genetic cause. We present a family affected by DCM associated with Filamin C variant, causing sudden cardiac death at a young age and heart failure due to severe left ventricular impairment and myocardial scarring. We review the diagnosis and treatment of DCM, its genetic associations and potential acquired causes. Thorough assessment is mandatory to risk stratify and identify patients who may benefit from primary prevention implantable cardioverter defibrillator therapy according to international guidelines. Genetic testing has some limitations, and is positive in only 20%–35% of DCM, but should be considered in specific cases to identify families who may benefit from cascade screening after appropriate counselling. The management of often complex familial cardiomyopathy requires specialist input for every case, and the appropriate infrastructure to coordinate investigations.

The management of heart failure with a reduced ejection fraction is a true success story of modern medicine. Evidence from randomised clinical trials provides the basis for an extensive catalogue of disease-modifying drug treatments that improve both symptoms and survival. These treatments have undergone rigorous scrutiny by licensing and guideline development bodies to make them eligible for clinical use. With an increasing number of drug therapies however, it has become a complex management challenge to ensure patients receive these treatments in a timely fashion and at recommended doses. The tragedy is that, for a condition with many life-prolonging drug therapies, there remains a potentially avoidable mortality risk associated with delayed treatment. Heart failure therapeutic agents have conventionally been administered to patients in the chronological order they were tested in clinical trials, in line with the aggregate benefit observed when added to existing background treatment. We review the evidence for simultaneous expedited initiation of these disease-modifying drug therapies and how these strategies may focus the heart failure clinician on a time-defined smart goal of drug titration, while catering for patient individuality. We highlight the need for adequate staffing levels, especially heart failure nurse specialists and pharmacists, in a structure to provide the capacity to deliver this care. Finally, we propose a heart failure clinic titration schedule and novel practical treatment score which, if applied at each heart failure patient contact, could tackle treatment inertia by a constant assessment of attainment of optimal medical therapy.

Introduction: The soluble ST2 (sST2) biomarker is upregulated in response to myocardial strain, remodeling and fibrosis, and may play a role in risk stratification of patients with decompensated Heart Failure with a Reduced Ejection Fraction (HFrEF). The widely accepted prognostic sST2 cut-off for chronic HF is 35ng/ml. What this value would be for acute HF is debated. Objectives: To determine the prognostic value of sST2 in patients admitted with acute decompensated HFrEF. Methods: Between January 2020 and June 2021, consecutive patients hospitalized with HFrEF had sST2 measured at admission (within 24 hours) and at recompensation, as part of the INST2ANT-HF study (UK REC no:19/EE/0269). To determine an appropriate prognostic cut-off, we evaluated the median sST2 in our group as a potential threshold. The primary outcome was mortality at one year. Cumulative incidences, and the relationship between several clinical variables were tested in a Cox multivariate model for the primary outcome and comparisons between groups for events was performed using the χ2 test. Results: In the first 34 consecutive patients (Male 64%, AF 38%) with complete follow up (FU) data, median (IQR) FU was 405 (262-438)days. Median (IQR) age, 77 (60-85)years, mean (SD) EF, 27.9(±12.1). Median (IQR) eGFR, 62 (50-83)ml/min/1.73m 2 . Median (IQR) admission sST2 was 68 (43-91)ng/ml and admission NTproBNP was 7307 (5019-12809)ng/L. The primary endpoint occurred more frequently in patients with admission sST2>68ng/ml (p<0.01) (Fig.1). Conclusion: Our data suggests that in an unselected population of patients admitted with acute decompensated HFrEF, an admission sST2 value >68ng/ml has important prognostic value. Patients with high sST2 values on presentation require close follow up and a lower threshold for consideration of advanced HF therapies. A higher sST2 prognostic cut-off compared to CHF patients may hence be required for risk stratification.