Selecting patients with nonischemic dilated cardiomyopathy for ICDs

No abstract available

When can heart failure treatment be stopped safely? – Authors' reply

No abstract available

Personalizing sudden death risk stratification in dilated cardiomyopathy: past, present and future

Results from the DANISH Study (Danish Study to Assess the Efficacy of ICDs in Patients with Nonischemic Systolic Heat Failure on Mortality) suggest that, for many patients with dilated cardiomyopathy (DCM), implantable cardioverter defibrillators (ICD) do not increase longevity. Accurate identification of patients who are more likely to die of an arrhythmia and less likely to die from other causes is required to ensure improvement in outcomes and wise use of resources. Until now, left ventricular ejection fraction (LVEF) has been used as a key criterion for selecting patients with DCM for an ICD for primary prevention purposes. However, registry data suggest that many patients with DCM and an out-of-hospital cardiac arrest do not have a markedly reduced LVEF. Additionally, many patients with reduced LVEF die from non-sudden causes of death. Methods to predict a higher or lower risk of sudden death include the detection of myocardial fibrosis (a substrate for ventricular arrhythmia), microvolt T-wave alternans (MTWA; a marker of electrophysiological vulnerability) and genetic testing. Mid-wall fibrosis is identified by late gadolinium enhancement cardiovascular magnetic resonance imaging in around 30% of patients and provides incremental value in addition to LVEF for the prediction of SCD events. MTWA represents another promising predictor, supported by large meta-analyses that have highlighted the negative predictive value of this test. However, neither of these strategies has been routinely adopted for risk stratification in clinical practice. More convincing data from randomized trials are required to inform the management of patients with these features. Understanding of the genetics of DCM and how specific mutations affect arrhythmic risk is also rapidly increasing. The finding of a mutation in LMNA, the cause of around 6% of idiopathic DCM, commonly underpins more aggressive management due to the malignant nature of the associated phenotype. With the expansion of genetic sequencing, the identification of further high-risk mutations appears likely, leading to better informed clinical decision-making as well as providing insight into disease mechanisms. Over the next 5-10 years we expect these techniques to be integrated into the existing algorithm to form a more sensitive, specific and cost-effective approach to the selection of DCM patients for ICD implantation

Evolutions in care, unmet needs, and research priorities in heart failure

The current treatment landscape for heart failure is predominantly stratified using ejection fraction. Established drug combinations and devices such as cardiac resynchronisation therapy are available for heart failure with reduced ejection fraction (HFrEF), but medical options for heart failure with preserved ejection fraction (HFpEF) have, until recently, been lacking.A major advance in recent years has been the discovery of effective therapies for HFpEF, including sodium-glucose co-transporter 2 (SGLT2) inhibitors and perhaps also the mineralocorticoid receptor antagonist, spironolactone. For patients with atrial fibrillation and heart failure, the benefit of rhythm control with either radiofrequency ablation or medical therapy is uncertain. Targeted therapies for the small proportion of patients with transthyretin cardiac amyloidosis are available, while antifibrotics seem promising for a larger proportion of patients.For patients with HFrEF, additional treatment options have emerged in the past 10 years. The angiotensin receptor–neprilysin inhibitor (ARNI) combination sacubitril–valsartan and SGLT-2 inhibitors reduce mortality and improve life expectancy in symptomatic patients with HFrEF and at least mildly elevated plasma concentrations of N-terminal pro-brain natriuretic peptide (NT-proBNP). The oral soluble guanylate cyclase stimulator vericiguat and cardiac myosin activator omecamtiv mecarbil are not yet licensed in the UK but may provide further treatment options, perhaps in more select groups of patients.Whether all patients with a prior diagnosis of HFrEF who are now in heart failure remission should continue all therapies at maximum tolerated dose indefinitely remains a dilemma. Individualised de-escalation of therapy remains controversial due to the risk of relapse but is occasionally trialled, particularly in patients with a triggering factor such as pregnancy. The ultimate aim is a personalised treatment plan—based on disease phenotype and trajectory—that minimises the risk of relapse and maximises the individual’s quality of life

Evolutions in care, unmet needs, and research priorities in heart failure

The current treatment landscape for heart failure is predominantly stratified using ejection fraction. Established drug combinations and devices such as cardiac resynchronisation therapy are available for heart failure with reduced ejection fraction (HFrEF), but medical options for heart failure with preserved ejection fraction (HFpEF) have, until recently, been lacking.A major advance in recent years has been the discovery of effective therapies for HFpEF, including sodium-glucose co-transporter 2 (SGLT2) inhibitors and perhaps also the mineralocorticoid receptor antagonist, spironolactone. For patients with atrial fibrillation and heart failure, the benefit of rhythm control with either radiofrequency ablation or medical therapy is uncertain. Targeted therapies for the small proportion of patients with transthyretin cardiac amyloidosis are available, while antifibrotics seem promising for a larger proportion of patients.For patients with HFrEF, additional treatment options have emerged in the past 10 years. The angiotensin receptor–neprilysin inhibitor (ARNI) combination sacubitril–valsartan and SGLT-2 inhibitors reduce mortality and improve life expectancy in symptomatic patients with HFrEF and at least mildly elevated plasma concentrations of N-terminal pro-brain natriuretic peptide (NT-proBNP). The oral soluble guanylate cyclase stimulator vericiguat and cardiac myosin activator omecamtiv mecarbil are not yet licensed in the UK but may provide further treatment options, perhaps in more select groups of patients.Whether all patients with a prior diagnosis of HFrEF who are now in heart failure remission should continue all therapies at maximum tolerated dose indefinitely remains a dilemma. Individualised de-escalation of therapy remains controversial due to the risk of relapse but is occasionally trialled, particularly in patients with a triggering factor such as pregnancy. The ultimate aim is a personalised treatment plan—based on disease phenotype and trajectory—that minimises the risk of relapse and maximises the individual’s quality of life

Outcome in Dilated Cardiomyopathy Related to the Extent, Location, and Pattern of Late Gadolinium Enhancement.

OBJECTIVES: This study sought to investigate the association between the extent, location, and pattern of late gadolinium enhancement (LGE) and outcome in a large dilated cardiomyopathy (DCM) cohort. BACKGROUND: The relationship between LGE and prognosis in DCM is incompletely understood. METHODS: The authors examined the association between LGE and all-cause mortality and a sudden cardiac death (SCD) composite based on the extent, location, and pattern of LGE in DCM. RESULTS: Of 874 patients (588 men, median age 52 years) followed for a median of 4.9 years, 300 (34.3%) had nonischemic LGE. Estimated adjusted hazard ratios for patients with an LGE extent of 0 to 2.55%, 2.55% to 5.10%, and >5.10%, respectively, were 1.59 (95% confidence interval [CI]: 0.99 to 2.55), 1.56 (95% CI: 0.96 to 2.54), and 2.31 (95% CI: 1.50 to 3.55) for all-cause mortality, and 2.79 (95% CI: 1.42 to 5.49), 3.86 (95% CI: 2.09 to 7.13), and 4.87 (95% CI: 2.78 to 8.53) for the SCD endpoint. There was a marked nonlinear relationship between LGE extent and outcome such that even small amounts of LGE predicted a substantial increase in risk. The presence of septal LGE was associated with increased mortality, but SCD was most associated with the combined presence of septal and free-wall LGE. Predictive models using LGE presence and location were superior to models based on LGE extent or pattern. CONCLUSIONS: In DCM, the presence of septal LGE is associated with a large increase in the risk of death and SCD events, even when the extent is small. SCD risk is greatest with concomitant septal and free-wall LGE. The incremental value of LGE extent beyond small amounts and LGE pattern is limited

Sex‐ and age‐based differences in the natural history and outcome of dilated cardiomyopathy

Aim: To evaluate the relationship between sex, age and outcome in dilated cardiomyopathy (DCM). Methods and results: We used proportional hazard modelling to examine the association between sex, age and all‐cause mortality in consecutive patients with DCM. Overall, 881 patients (290 women, median age 52 years) were followed for a median of 4.9 years. Women were more likely to present with heart failure (64.0% vs. 54.5%; P = 0.007) and had more severe symptoms (P 60 years of age was driven by non‐sudden death. Conclusion: Women with DCM have better survival compared to men, which may partly be due to less severe left ventricular dysfunction and a smaller scar burden. There is increased mortality driven by non‐sudden death in patients >60 years of age that is less marked in women. Outcomes with contemporary treatment were favourable, with a low incidence of sudden death