Mavacamten in Symptomatic Nonobstructive Hypertrophic Cardiomyopathy.

Background: Mavacamten is approved to treat adults with symptomatic obstructive hypertrophic cardiomyopathy (HCM). However, its effects in nonobstructive HCM remain uncertain.

Methods: We conducted a phase 3, international, double-blind, placebo-controlled, clinical trial to determine whether mavacamten improves functional capacity and patient-reported health status among adults with symptomatic nonobstructive HCM.

Effects of Mavacamten on Cardiac Biomarkers in Nonobstructive Hypertrophic Cardiomyopathy: Insights From the ODYSSEY-HCM Trial.

Background: No therapy is approved for patients with symptomatic nonobstructive hypertrophic cardiomyopathy (nHCM). The ODYSSEY-HCM (A Study of Mavacamten in Non-Obstructive Hypertrophic Cardiomyopathy [ODYSSEY-HCM]; NCT05582395) trial, the largest to date in patients with hypertrophic cardiomyopathy (HCM), evaluating the efficacy of mavacamten in symptomatic adults with nHCM, did not demonstrate improvements in its primary endpoints (functional capacity and patient-reported health status).

Objectives: The current exploratory analysis from the ODYSSEY-HCM trial reports the associations between: 1) baseline biomarkers (N-terminal pro-B-type natriuretic peptide [NT-proBNP] and high-sensitivity cardiac troponin I [cTnI]) with clinical, exercise, and echocardiographic characteristics; and 2) comparing changes in these biomarkers from baseline to week 48 between mavacamten and placebo groups.

Left ventricular global longitudinal strain and diagnostic yield of genetic testing in hypertrophic cardiomyopathy in a multicenter registry analysis.

In this multicenter study we investigated whether echocardiography-derived left ventricular global longitudinal strain (LV GLS), an indicator of myocardial fibrosis, independently predicts a positive genotype in hypertrophic cardiomyopathy (HCM). We performed a cross-sectional analysis including HCM patients with genetic testing results and echocardiographic data from two Austrian HCM registries. Echocardiographic parameters were measured in post-processing analysis by a blinded investigator.

Predictors of disease burden in patients with untreated transthyretin amyloid cardiomyopathy and their caregivers: a analysis of an international survey.

Introduction: Transthyretin amyloid cardiomyopathy (ATTR-CM) is a progressive condition with debilitating symptoms. The self-reported burden of ATTR-CM on patients and their caregivers was previously evaluated in an international, multicenter, real-world survey study.

Methods: This analysis used univariate and multivariate models to evaluate survey items as predictors of ATTR-CM burden.

Ambient Temperature and the Frequency of Subsequent Heart Failure Decompensations in an Emergency Department.

The impact of cold temperature on heart failure (HF) decompensations in continental climate zones is unclear. We aimed to evaluate the association between daily temperature and the subsequent frequency of HF decompensations in an emergency department (ED) in Eastern Austria. A systematic retrospective medical chart review of all admissions to the ED of a tertiary care center within 12 months was conducted.

Tafamidis in octogenarians with wild-type transthyretin cardiac amyloidosis: an international cohort study.

Background And Aims: In real-world, wild-type transthyretin cardiomyopathy is increasingly diagnosed in patients ≥ 80 years old (octogenarians), although being underrepresented in randomized clinical trials. Specific data on natural course and outcome under tafamidis treatment in octogenarians are therefore scarce. The impact of tafamidis treatment on mortality in real-world wild-type transthyretin cardiomyopathy octogenarians was studied.

Variant cardiac transthyretin amyloidosis presenting as hypertrophic cardiomyopathy with left ventricular outflow tract obstruction: a case report.

Background: Left ventricular outflow tract obstruction (LVOTO) is common in hypertrophic cardiomyopathy (HCM) but has not been reported in hereditary transthyretin amyloidosis (ATTRv).

Case Summary: Here, we describe a 67-year-old male patient with a hypertrophic phenotype who was initially diagnosed with LVOTO attributed to HCM. Echocardiographic features included a hyperdynamic left ventricular ejection fraction, severe septal hypertrophy, and elongated residual mitral leaflets permitting their systolic anterior motion.

Mavacamten in Symptomatic Nonobstructive Hypertrophic Cardiomyopathy: Design, Rationale, and Baseline Characteristics of ODYSSEY-HCM.

There are no approved therapies for patients with symptomatic nonobstructive hypertrophic cardiomyopathy (nHCM). The authors describe the baseline characteristics of ODYSSEY-HCM (A Study of Mavacamten in Non-Obstructive Hypertrophic Cardiomyopathy), a phase 3, randomized, double-blind, placebo-controlled trial conducted worldwide at 201 sites evaluating mavacamten in symptomatic adult patients with nHCM. The 2 primary endpoints are the changes from baseline to week 48 in: 1) Kansas City Cardiomyopathy Questionnaire 23-item Clinical Summary Score; and 2) peak oxygen consumption (pVO) on cardiopulmonary exercise testing.

Prognostic impact of gait speed, muscle strength and muscle mass in chronic heart failure-A prospective cohort study.

Aims: Heart failure (HF) impairs skeletal muscle mass and function, which contributes to reduced physical performance. We investigated the prognostic impact of gait speed (GS), handgrip strength (HG) and appendicular skeletal muscle index (ASMI) on cardiovascular outcomes in a prospective HF cohort.

Methods: This single-centre prospective cohort study included adults with stable chronic HF with a previous diagnosis of overtly reduced left ventricular ejection fraction (LVEF) <40% and LVEF < 50% at enrolment.

Air Pollution and Myocardial Infarction-A New Smoker's Paradox?

Ambient air pollution is a significant public health concern, known to affect cardiovascular health adversely. Research has identified both long-term and short-term cardiovascular risks associated with various air pollutants, including those linked to acute coronary syndromes. However, the observed effects are rather small, with most data sourced from highly polluted regions.

Myeloid sarcoma of the heart-A clinicopathological correlation.

A 63-year-old woman with a history of acute myeloid leukemia followed by stem cell transplantation presented with acute heart failure. Transthoracic echocardiography revealed a preserved left ventricular ejection fraction with severe ventricular hypertrophy and signs of elevated filling pressures indicating infiltrative cardiomyopathy. She died from cardiac arrest due to cardiogenic shock.

Impact of SGLT2-Inhibitor Therapy on Survival in Patients with Transthyretin Amyloid Cardiomyopathy: Analysis of a Prospective Registry Study.

Patients with transthyretin amyloid cardiomyopathy (ATTR-CM) represent a high-risk heart failure population with continued unmet therapeutic needs. Sodium-glucose co-transporter 2 inhibitors (SGLT2i) improve cardiovascular outcomes in patients with heart failure across the whole spectrum of ejection fraction, and first evidence regarding their safety and effectiveness in patients with ATTR-CM is arising. This study investigates the association between SGLT2i therapy and clinical outcomes in these patients.

Impact of empagliflozin on cardiac structure and function assessed by echocardiography after myocardial infarction: a post-hoc sub-analysis of the emmy trial.

Background: Empagliflozin administered after acute myocardial infarction proofed to improve cardiometabolic parameters and biomarkers, but the impact on cardiac function is still largely unknown. The aim of this post-hoc echocardiographic sub-analysis of the EMMY trial was to provide in-depth echocardiographic analysis on the effects of empagliflozin versus placebo on standard and novel echocardiographic structural and functional parameters after acute myocardial infarction.

Methods: In this post-hoc analysis of the EMMY trial a subset of 313 patients (157 empagliflozin vs.

MetSCORE: a molecular metric to evaluate the risk of metabolic syndrome based on serum NMR metabolomics.

Background: Metabolic syndrome (MetS) is a cluster of medical conditions and risk factors correlating with insulin resistance that increase the risk of developing cardiometabolic health problems. The specific criteria for diagnosing MetS vary among different medical organizations but are typically based on the evaluation of abdominal obesity, high blood pressure, hyperglycemia, and dyslipidemia. A unique, quantitative and independent estimation of the risk of MetS based only on quantitative biomarkers is highly desirable for the comparison between patients and to study the individual progression of the disease in a quantitative manner.

The Prognostic Value of Right Ventricular Function in Patients with Chronic Heart Failure-A Prospective Study.

In patients with stable chronic heart failure with a reduced ejection fraction (HFrEF), left ventricular ejection fraction (LVEF) provides limited prognostic value, especially in patients with moderately to severely reduced LVEF. Echocardiographic parameters of right ventricular function may be associated with adverse clinical events in these patients. Therefore, we analyzed 164 patients with HFrEF in a prospective single-center cohort study to evaluate whether the parameters of right ventricular function are associated with worsening heart failure (WHF) hospitalizations, cardiovascular and all-cause deaths and combined endpoints.

Non-invasive estimation of left ventricular systolic peak pressure: a prerequisite to calculate myocardial work in hypertrophic obstructive cardiomyopathy.

Aims: Myocardial work (MyW) is an echocardiographically derived parameter to estimate myocardial performance. The calculation of MyW utilizes pressure strain loops from global longitudinal strain and brachial blood pressure (BP) as a surrogate of left ventricular systolic pressure (LVSP). Since LVSP cannot be equated with BP in hypertrophic obstructive cardiomyopathy (HOCM), we explored whether LVSP can be derived non-invasively by combining Doppler gradients and BP.

Burden of untreated transthyretin amyloid cardiomyopathy on patients and their caregivers by disease severity: results from a multicenter, non-interventional, real-world study.

Background: The humanistic burden of transthyretin amyloid cardiomyopathy (ATTR-CM) is poorly defined.

Methods: An international study to comprehensively characterize the burden of ATTR-CM on patients naïve to disease-modifying therapy and their unpaid primary caregivers using study-specific and established surveys (patients: Kansas City Cardiomyopathy Questionnaire Overall Summary [KCCQ-OS], 12-Item Short Form Health Survey [SF-12], Hospital Anxiety and Depression Scale [HADS], Patient-Reported Outcomes Measurement Information System [PROMIS] Fatigue and Dyspnea; caregivers: SF-12, HADS, PROMIS Fatigue, Zarit Burden Interview [ZBI]). All data were summarized descriptively.