Clinical value of the tissue Doppler derived index of myocardial performance (Tei index) in cats with hypertrophic cardiomyopathy.

Surgical treatment of hypertrophic obstructive cardiomyopathy is challenging. It requires balancing adequate myocardial excision against the risk of excessive resection, which can result in an iatrogenic ventricular septal defect. To our knowledge, this is the first reported case of a high-risk septal myectomy in complex anatomy W-shaped hypertrophic obstructive cardiomyopathy with a deep interventricular septum crypt and apical aneurysm. CT angiography-based three-dimensional modeling of the interventricular septum and a virtual myectomy enabled detailed preoperative planning and successful surgery. This planning also informed the choice of a combined transaortic and transapical surgical approach tailored to the patient's anatomy. Keywords: CT Angiography, MR Imaging, Cardiac, Heart, Left Ventricle, Anatomy, Cardiomyopathies, Computer Applications-3D, Computer Applications-Virtual, Segmentation, Modeling, Myocardium, 3D Computer Model, 3D Printing, Cardiac CT Angiography, Cardiac CTA, Cardiac MRI, HOCM, Hypertrophic Obstructive Cardiomyopathy, Septal Myectomy © RSNA, 2026.

Concomitant Mitral Valve Surgery During Surgical Treatment of Hypertrophic Obstructive Cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is characterized by abnormal myocardial mechanics, with or without left ventricular outflow tract obstruction (LVOTO). Myosin inhibitors such as mavacamten have shown clinical efficacy in reducing obstruction, but their effects on left ventricular hemodynamic forces (HDF)-a novel marker of myocardial function-are not yet defined. This study aimed to characterize left ventricular HDF patterns in patients with obstructive (oHCM) and non-obstructive (noHCM) HCM compared to healthy controls, and to evaluate the impact of long-term mavacamten therapy on HDF parameters in oHCM. We retrospectively analyzed 40 HCM patients (20 oHCM, 20 noHCM) and 20 age- and sex-matched healthy controls. HDF parameters were quantified using echocardiographic strain imaging software (Qstrain, Medis Medical Imaging Systems). Parameters included systolic and diastolic force amplitudes and time indices. Six oHCM patients receiving mavacamten were assessed at baseline and after three years of therapy. Compared to controls, HCM patients had significantly reduced systolic time to peak (p<0.001) and diastolic-systolic transition time (p<0.001), indicating premature systolic thrust and impaired relaxation. Diastolic force amplitude was significantly lower in oHCM than noHCM (p<0.001). Mavacamten treatment in oHCM patients significantly prolonged diastolic-systolic transition (p=0.030) and restored longitudinal force dynamics (p=0.031), suggesting improved mechanical coordination and reduced LVOTO. HDF analysis identifies distinct mechanical impairments in HCM regardless of obstruction status. Mavacamten favorably modulates HDF, delaying systolic thrust initiation and normalizing force distribution, offering new mechanistic insights into its therapeutic action in HCM.

Baseline clinical profile of patients with obstructive hypertrophic cardiomyopathy in the Italian Mavacamten early access program.

Fast cardiac magnetic resonance (CMR) protocol for biventricular functional assessment and tissue characterisation.

Staging of Fabry cardiomyopathy in clinical practice: an algorithm proposal.

Integrating AI-ECG and point-of-care cardiac ultrasound for screening structural heart disease: A proof-of-concept study.

Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiac disease and a leading cause of heart failure and sudden cardiac death (SCD) in young adults. Given its complex pathophysiology, phenotypic diversity, and rapidly evolving therapeutic landscape, a structured and multidisciplinary approach to care is essential. This manuscript outlines a six-pillar framework to standardize and optimize evaluation and management of HCM. The proposed model organizes HCM care into six key domains. (i) Establishing the correct diagnosis, which requires differentiation between sarcomeric HCM and phenocopies such as amyloidosis, Fabry, or mitochondrial disease, using multimodal imaging and genetic testing. (ii) Establish presence of symptoms and of left ventricular outflow tract obstruction (LVOTO), which is central to symptom evaluation, prognostication, and treatment. Dynamic assessment with exercise echocardiography when required is essential to guide management, including pharmacotherapy or septal reduction therapy. (iii) Risk stratification for SCD integrates risk scores with adjunctive imaging data to support patient-centred implantable cardioverter defibrillator decisions. (iv) Genetic evaluation and family management enable cascade testing, early detection, and counselling. (v) Management of comorbidities-including atrial fibrillation, hypertension, obesity, and sleep-disordered breathing-is integral to holistic care and symptom control. (vi) Education and lifestyle guidance focus on safe sport participation, avoidance of dehydration and vasodilators, and reproductive counselling within a multidisciplinary setting.

Heyde syndrome is an uncommon clinical condition characterized by the triad of aortic stenosis, gastrointestinal angiodysplasia, and acquired von Willebrand syndrome. Its occurrence in the context of hypertrophic obstructive cardiomyopathy is rare. Common in individuals over 65, diagnosis is challenging due to the prevalence of these conditions. Hypertrophic obstructive cardiomyopathy increases shear stress, leading to von Willebrand Factor degradation and a higher risk of gastrointestinal bleeding. A 69-year-old male presented to the outpatient clinic with melena, abdominal cramping, and fatigue, along with a history of anemia requiring blood transfusions. Despite a negative upper gastrointestinal endoscopy, further tests revealed angiodysplasias with recent bleeding. He was also diagnosed with hypertrophic cardiomyopathy with left ventricular outflow tract obstruction. After being treated with octreotide and nadolol, his condition improved markedly, with hemoglobin levels rising and no further episodes of bleeding. Recognizing hypertrophic obstructive cardiomyopathy as a new variant of Heyde syndrome broadens our understanding of this complex disorder. It emphasizes the need to consider hypertrophic obstructive cardiomyopathy in patients with unexplained gastrointestinal bleeding and cardiac murmurs, prompting a more tailored approach to diagnosis and treatment.

Performance and Safety of the Extravascular Implantable Cardioverter-Defibrillator in Patients With Hypertrophic Cardiomyopathy.

ABSTRACTAimsImplantable cardioverter‐defibrillator (ICD) therapy in children, particularly for primary prevention, remains under investigation, with limited data from less affluent European countries. With increasing use of fully subcutaneous (S‐ICD) and extravascular (EV‐ICD) systems, we analyzed epidemiology, indications, complications, and outcomes in pediatric ICD recipients at our tertiary center.MethodsThis retrospective, population‐based, nationwide, single‐center study consecutively analyzed medical records from 2012 to 2025. Patients aged 0–18 years who underwent ICD implantation were included. Kaplan–Meier analyses were performed for shock‐free and mortality endpoints.ResultsForty‐nine patients were included, corresponding to an estimated national incidence of approximately one ICD implantation per 22 000 live births. ICDs were predominantly implanted for secondary prevention (80%). Long QT syndrome (32%) and hypertrophic cardiomyopathy (22%) were the most frequent diagnoses. During 207 patient‐years of follow‐up (median follow‐up 4 years), appropriate ICD shocks occurred in 27% of patients exclusively in the secondary prevention group. No appropriate therapies occurred in the primary prevention group despite higher overall mortality. Inappropriate shocks occurred in 22% of patients. Device‐related major complications affected 17% of implanted systems. Median transvenous ICD battery longevity was 6.5 years. Overall mortality was 6%, all related to underlying structural heart disease, with no ICD‐related deaths. S‐ICDs demonstrated a favorable acute and mid‐term safety profile in patients without pacing requirements.ConclusionsPediatric ICD therapy is limited by substantial device‐related morbidity and imperfect risk stratification for primary prevention. The emerging S‐ICD and EV‐ICD systems represent promising, less invasive alternatives for selected pediatric patients.

Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiovascular disease. Recent metabolomics studies have revealed pathogenic mechanisms and provided new perspectives for diagnosis. This study aimed to analyze plasma metabolic alterations and construct a preliminary diagnostic model for HCM based on untargeted metabolomics and machine learning (ML) algorithms, in order to explore potential pathogenic pathways and improve diagnostic accuracy during screening. A total of 76 HCM patients and 35 normal participants were consecutively recruited from August, 2023 to December, 2023. Data were split into discovery and validation sets at a ratio of 7:3 and the feature combinations were selected using support vector machine (SVM) and random forest (RF). Stepwise multivariate linear regression analysis was performed to identify key metabolites associated with left ventricular wall thickness. Metabolic pathway analysis was performed using KEGG. Totally 1481 metabolites were identified with 640 differential metabolites and 240 significant differential metabolites. Multivariate statistical analysis showed that metabolism results could effectively differentiate the two cohorts (OPLS-DA positive ion mode R2Y = 0.744, Q2 = 0.456; negative ion mode R2Y = 0.611, Q2 = 0.441). SVM and RF screened the same combination of features including 7-keto-8-aminopelargonic acid (KAPA), γ-linolenoyl ethanolamid, nitrilotriacetic acid, D-quinovose and N-acetyl-l-aspartic acid (NAA), which could effectively and accurately differentiate HCM patients from normal participants (in discovery and validation sets, the SVM model AUROC was 0.996 and 0.985 with accuracies of 96.1% and 97.1%, respectively; the RF model AUROC was 1.000 with accuracies of 94.8% and 100.0%, respectively). In metabolic pathway analysis, central carbon metabolism in cancer and protein digestion and absorption were significantly upregulated in HCM patients, which were connected by alanine, aspartate and glutamate metabolism. Stepwise multivariate linear regression analysis revealed that NAA was correlated with left ventricular mass index and RV5+SV1 (P < 0.05), which may be the central target of the connecting pathway. Plasma metabolite diagnostic model including KAPA, γ-linolenoyl ethanolamid, nitrilotriacetic acid, D-quinovose and NAA can effectively and accurately screen HCM patients. Metabolomics combined with ML algorithm showed that alanine, aspartate and glutamate metabolism may be the pathogenic pathway leading to the occurrence of HCM with NAA as the central target.

Incremental value of implantable loop recorders in arrhythmia detection and management in cardiomyopathies: Prospective study.

Pharmacological effects and molecular targets of L-theanine in cardiovascular diseases and comorbidities.

European Research Council starting grant: ultrafast ultrasound imaging for genotype-phenotype interplay in hypertrophic cardiomyopathy.

Aficamten is a next-in-class, oral selective cardiac myosin inhibitor that ameliorates hypercontractility in hypertrophic cardiomyopathy (HCM). This study assessed the safety and efficacy of extended aficamten treatment in symptomatic obstructive HCM (oHCM). Patients completing a parent aficamten study were eligible to enrol in FOREST-HCM (NCT04848506), an open-label study evaluating long-term aficamten treatment. Patients with oHCM (N = 296; mean age ±SD 61 ± 12.3 years, 44.3% female) enrolled between May 2021 and August 2024. Cumulative exposure was 352 patient-years; median follow-up 51.6 (IQR 41.5, 70.8) weeks. At Weeks 12 and 96, aficamten reduced Valsalva left ventricular outflow tract gradient by 56 ± 43 and 62 ± 33 mmHg from baseline (both P < 0.0001), with minimal reduction in left ventricular ejection fraction (LVEF) (-3% ± 6% and -5% ± 5%); 69% and 93% of participants had at least one NYHA class improvement; Kansas City Cardiomyopathy Questionnaire-Clinical Summary Score improved by 15 ± 16 and 16 ± 16 points. Treatment-emergent serious adverse events (TESAEs) occurred in 36 (12.2%) patients; no deaths, heart failure, or events considered related to aficamten were reported. One (0.3%) patient terminated therapy due to a TESAE (ischemic colitis). LVEF<50% occurred in 10 (3.4%) patients [exposure-adjusted incidence rate (EAIR): 2.9 per 100 patient-years] with 2 having non-serious mild/moderate dyspnoea. No treatment interruptions for LVEF<50%, and no events of LVEF<40% occurred. New-onset atrial fibrillation occurred in seven (2.4%) patients (EAIR 2.0 per 100 patient-years). Extended aficamten treatment in patients with symptomatic oHCM yielded early and sustained hemodynamic and clinical responses with low incidences of new-onset atrial fibrillation and LVEF<50%.

The clinical relevance of myocardial bridge (MB) remains uncertain and inconsistently reported across studies. This study aimed to assess the relationship between MB and long-term mortality and morbidity, while accounting for the influence of varying patient populations and diagnostic methods used to detect MB. A comprehensive literature search was conducted to identify studies published up to December 2024 that examined the association between MB and adverse outcomes, including all-cause death (ACD), cardiovascular death (CVD), and major adverse cardiac events (MACE). A total of 22 studies involving 37,940 participants were included in the analysis. MB was not significantly associated with an increased risk of ACD or CVD. However, MB showed a significant association with MACE, with a pooled hazard ratio (HR) of 1.958 (95% CI: 1.490-2.572; P < 0.001) across 19 studies. Subgroup analysis indicated that MB identified through conventional coronary angiography was significantly linked to a higher risk of MACE (pooled HR: 1.992; 95% CI: 1.432-2.772; P < 0.001), whereas MB detected using multi-detector computed tomography did not show a statistically significant association with MACE. Among high-risk groups, such as patients with hypertrophic cardiomyopathy and those with high-risk coronary artery disease, MB was associated with a notably increased risk of MACE, with pooled HRs of 2.174 (95% CI: 1.067-4.426; P = 0.032) and 3.435 (95% CI: 1.732-6.810; P < 0.001), respectively. MB was associated with an increased relative risk of MACE in pooled analyses but showed no significant association with isolated ACD or CVD.

Biomarkers as Clinical Endpoints in Hypertrophic Cardiomyopathy Clinical Trials.

Predictive Value of Existing Prediction Models for Short-term Outcomes after Percutaneous Intramyocardial Septal Radiofrequency Ablation.