Echocardiography-guided percutaneous intramyocardial septal radiofrequency ablation procedure for the treatment of Fabry disease: a case report.

Background: This is a case report of a patient with Fabry disease (FD). We successfully treated a patient with ventricular septal hypertrophy and left ventricular outflow tract (LVOT) obstruction caused by FD. We report our exclusive new surgery for patients with LVOT obstruction, percutaneous intramyocardial septal radiofrequency ablation (PIMSRA) procedure™ (percutaneous intramyocardial septal radiofrequency ablation).

Single Cell Transcriptomic Profiling of -Associated Hypertrophic Cardiomyopathy Across Species Reveals Conservation of Biological Process But Not Gene Expression.

Background: Hypertrophic cardiomyopathy (HCM) is a common heritable heart disease where the most frequently associated mutations occur in the myosin-binding protein C () sarcomere-associated gene. HCM is also a common veterinary clinical problem in certain cat breeds such as Maine Coons and Ragdolls, also most associated with mutations in . Mouse models of HCM in which mutations are introduced recapitulate some, but not all, features of human HCM.

Echocardiographic Changes Following Surgical Myectomy in Severely Symptomatic Obstructive Hypertrophic Cardiomyopathy: Insights From the SPIRIT-HCM Study.

Background: In obstructive hypertrophic cardiomyopathy, myectomy improves symptoms, quality of life, and left ventricular (LV) outflow tract gradients. We prospectively evaluated the temporal changes in various echo parameters after myectomy.

Methods And Results: In 173 adults with obstructive hypertrophic cardiomyopathy (53±10 years, 63% men) who underwent myectomy between March 2017 and June 2020, clinical and blinded echo assessment (before and at 12±6 months follow-up) was performed prospectively (SPIRIT-HCM [Quality of Life and Functional Capacity Following Septal Myectomy in Obstructive Patients With Hypertrophic Cardiomyopathy]).

Endpoint Selection in Randomized Clinical Trials for Hypertrophic Cardiomyopathy.

Randomized clinical trials (RCTs) for hypertrophic cardiomyopathy (HCM) have long been challenging caused by the condition's rarity, low event rates, and diverse clinical presentations. However, recent advances in targeted therapies have sparked increased interest in HCM research. Despite this, designing effective RCTs remains complex, particularly in identifying clinically meaningful endpoints.

Mavacamten in Patients With Hypertrophic Cardiomyopathy Referred for Septal Reduction: Week 128 Results from VALOR-HCM.

Background: In severely symptomatic patients with obstructive hypertrophic cardiomyopathy (HCM), VALOR-HCM trial (Study to Evaluate Mavacamten in Adults With Symptomatic Obstructive HCM Who Are Eligible for Septal Reduction Therapy [URL: https://clinicaltrials.gov; Unique identifier: NCT04349072]) reported that mavacamten reduced the short-term need for septal reduction therapy (SRT). The current report examined the longer-term effect of mavacamten through end of treatment at week 128.

Association of Histologic Findings With Long-Term Outcomes in Symptomatic Obstructive Hypertrophic Cardiomyopathy Patients Undergoing Surgical Myectomy.

Background: In hypertrophic cardiomyopathy, histologic findings like myocyte hypertrophy and disarray, interstitial fibrosis (IF), and small intramural coronary artery dysplasia (SICAD) result in left ventricular hypertrophy, diastolic dysfunction, arrhythmogenicity, and microvascular ischemia.

Objectives: The authors sought to evaluate the association between histology and outcomes in obstructive hypertrophic cardiomyopathy (oHCM) patients undergoing surgical myectomy (SM).

Methods: The study included 1,722 symptomatic oHCM patients (mean age: 56 ± 14 years; 948 [55%] men) who underwent SM at a tertiary center between 2005 and 2018.

QRS prolongation is associated with associated with adverse cardiac remodeling in hypertrophic cardiomyopathy.

Background: Signal-averaged electrocardiogram (SAECG) records myocardial depolarization, and can detect inhomogeneous/slow conduction in fibrotic myocardium, which promotes reentrant ventricular arrhythmias (VAs). Hypertrophic cardiomyopathy (HCM) is associated with a high prevalence of cardiac fibrosis and VAs, but abnormal SAECG has low predictive power for VAs. We hypothesized that HCM-specific structural/electrical remodeling underlies this result.

Diagnosis and management of hypertrophic cardiomyopathy: European vs. American guidelines.

Hypertrophic cardiomyopathy (HCM) is the most common genetic heart disease, affecting 1:200 to 1:500 individuals worldwide. Guidelines on the diagnosis and management of HCM have been recently published by the European Society of Cardiology (ESC) and American societies. The ESC guidelines cover a broad range of cardiomyopathies, including HCM, with 119 recommendations, whereas the American guidelines focus exclusively on HCM with 141 specific recommendations.

Long-Term Outcomes of Patients With Apical Hypertrophic Cardiomyopathy Utilizing a New Risk Score.

Background: Apical hypertrophic cardiomyopathy (aHCM) is a distinct variant characterized by predominant hypertrophy of the left ventricle apex.

Objectives: This study sought to describe aHCM patients' characteristics and develop a risk score for aHCM patients.

Methods: A total of 462 patients (age 58 ± 15 years, 68% male) diagnosed with aHCM were included.

Follow-Up Study of Percutaneous Intramyocardial Septal Radiofrequency Ablation in the Treatment of Hypertrophic Obstructive Cardiomyopathy With HFpEF.

Background: The study aimed to evaluate the changes of left ventricular diastolic function and the improvement of clinical symptoms in hypertrophic cardiomyopathy (HCM) patients with heart failure with preserved ejection fraction (HFpEF) after percutaneous intramyocardial septal radiofrequency ablation (PIMSRA).

Methods: This study enrolled 31 adult HCM patients with HFpEF who underwent PIMSRA treatment. Electrocardiogram, imaging, and blood biochemical examinations were performed on these patients during a 6-month follow-up.

Allele-specific dysregulation of lipid and energy metabolism in early-stage hypertrophic cardiomyopathy.

Introduction: Hypertrophic cardiomyopathy (HCM) results from pathogenic variants in sarcomeric protein genes that increase myocyte energy demand and lead to cardiac hypertrophy. However, it is unknown whether a common metabolic trait underlies cardiac phenotype at the early disease stage. To address this question and define cardiac biochemical pathology in early-stage HCM, we studied two HCM mouse models that express pathogenic variants in cardiac troponin T () or myosin heavy chain () genes, and have marked differences in cardiac imaging phenotype, mitochondrial function at early disease stage.

Extended Ambulatory ECG Monitoring Enhances Identification of Higher-Risk Ventricular Tachyarrhythmias in Patients With Hypertrophic Cardiomyopathy.

Background: In hypertrophic cardiomyopathy (HCM), 48-hour ambulatory monitoring has been standard practice to detect nonsustained ventricular tachycardia (NSVT), a sudden death risk marker. Extended-wear ambulatory electrocardiographic (ECG) devices have more recently used for monitoring patients with HCM.

Objective: We aimed to evaluate NSVT burden identified with continuous ambulatory monitoring for up to 2 weeks compared with initial 48 hours.