439 results match your criteria: "Hypertrophic Cardiomyopathy Center[Affiliation]"

Mavacamten-Associated Temporal Changes in Left Atrial Function in Obstructive HCM: Insights From the VALOR-HCM Trial.

JACC Cardiovasc Imaging

September 2024

Hypertrophic Cardiomyopathy Center, Heart, Vascular and Thoracic Institute, Cleveland Clinic, Cleveland, Ohio, USA; Department of Cardiovascular Medicine, Heart, Vascular and Thoracic Institute, Cleveland Clinic, Cleveland, Ohio, USA; Cleveland Clinic Coordinating Center for Clinical Research, Heart, Vascular and Thoracic Institute, Cleveland Clinic, Cleveland, Ohio, USA.

Background: In severely symptomatic patients with obstructive hypertrophic cardiomyopathy (HCM), the VALOR-HCM (A Study to Evaluate Mavacamten in Adults With Symptomatic Obstructive Hypertrophic Cardiomyopathy Who Are Eligible for Septal Reduction Therapy) trial showed that mavacamten reduced the eligibility for septal reduction therapy with sustained improvement in left ventricular outflow tract gradients. Mavacamten also resulted in favorable cardiac remodeling, including improvement in biomarkers (eg, N-terminal pro-B-type natriuretic peptide and troponin T). However, the impact of mavacamten on left atrial (LA) function is unknown.

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Background: In severely symptomatic patients with obstructive hypertrophic cardiomyopathy, VALOR-HCM (A Study to Evaluate Mavacamten in Adults With Symptomatic Obstructive Hypertrophic Cardiomyopathy Who Are Eligible for Septal Reduction Therapy) demonstrated that mavacamten reduces the need for septal reduction therapy with sustained improvement in left ventricular (LV) outflow tract gradients and symptoms. Global longitudinal strain (GLS), a measure of regional myocardial function, is a more sensitive marker of systolic function. In VALOR-HCM, we assessed serial changes in LV and right ventricular (RV) strain.

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Background: The extent of late gadolinium enhancement (LGE) on cardiovascular magnetic resonance (CMR) in patients with hypertrophic cardiomyopathy (HCM) is associated with an increased risk of sudden cardiac death events. However, the clinical significance of age-specific longitudinal changes in LGE is not well characterized in HCM. We sought to assess whether the risk of LGE progression diverges between young to middle-aged (ages 20-59 years) and older (≥ 60) adults with HCM.

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Transesophageal echocardiography (TEE) plays an important role for real-time procedural guidance during surgical smyectomy (SM) for hypertrophic obstructive cardiomyopathy (HOCM). We aimed to compare (1) interventricular septum (IVS) thickness using 2- (2D) and 3-dimensional (3D) intraoperative TEE and preoperative cardiac magnetic resonance (CMR) and (2) mitral valve (MV) leaflet length using 2D, 3D TEE, automatic quantification of mitral valve (AMVQ) and preoperative CMR. We prospectively studied 50 patients with HOCM (age 59 ± 12 years, 44% men) who underwent SM during 2018 to 2019.

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Novel Cardiac Myosin Inhibitor Therapy for Hypertrophic Cardiomyopathy in Adults: A Contemporary Review.

Am J Cardiovasc Drugs

September 2024

Hypertrophic Cardiomyopathy Center, Section of Cardiovascular Imaging, Robert and Suzanne Tomsich Department of Cardiovascular Medicine, Sydell and Arnold Miller Family Heart, Vascular and Thoracic Institute, Cleveland Clinic, 9500 Euclid Avenue, Desk J1-5, Cleveland, OH, 44195, USA.

Hypertrophic cardiomyopathy (HCM) affects as many as 1 in 200 people in the adult population globally. Patients may present with exertional dyspnea, presyncope or syncope, atrial and ventricular arrhythmias, heart failure, and even sudden cardiac death. Current guideline-based therapy involves medical therapy for treatment of symptoms in milder forms of the disease and surgical or catheter-based septal reduction therapies in obstructive HCM.

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Article Synopsis
  • * Recent findings emphasize that the mitral valve, papillary muscle issues, and an apical-basal muscle bundle also contribute significantly to this obstruction, not just the typical septal hypertrophy.
  • * Four-dimensional computed tomography is recommended for detailed assessment and planning of surgical intervention in HCM, offering better imaging capabilities than traditional methods like echocardiography or cardiac MRI.
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Due to the elevated fatality rate of cardiovascular diseases, myocardial fibrosis emerges as a prominent pathological alteration in the majority of heart ailments and their associated pathologies, thereby augmenting the likelihood of sudden cardiac death. Consequently, the prompt and obligatory identification of myocardial fibrosis assumes paramount importance in averting malignant incidents among patients afflicted with cardiac disorders. Herein, with higher expression osteopontin (OPN) found in cardiac fibrosis tissue, we have developed a dual-modality imaging probe, namely OPN targeted nanoparticles (OPN@PFP-DiR NPs), which loaded perfluoropentane (PFP) for ultrasound (US) and 1,1-dioctadecyl-3,3,3,3-tetramethylindotricarbocyanine iodide (DiR) for near-infrared fluorescence (NIR) of molecular imaging, to investigate the molecular features of cardiac fibrosis using US and NIR imaging.

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The promise of gene therapy in hypertrophic cardiomyopathy: emergence of human experience.

Cardiovasc Res

July 2024

Hypertrophic Cardiomyopathy Center, Heart and Vascular Institute, Cleveland Clinic, 9500 Euclid Avenue, Desk J1-5, Cleveland, OH 44195, USA.

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Feasibility and safety of percutaneous intramyocardial septal cryoablation: A canine model with 6-month follow-up.

Cryobiology

September 2024

Xijing Hypertrophic Cardiomyopathy Center, Department of Ultrasound, Xijing Hospital, Fourth Military Medical University, Xi'an, Shaanxi, China. Electronic address:

Echocardiography-guided percutaneous intramyocardial septal radiofrequency ablation (PIMSRA, Liwen procedure) is a novel treatment option for hypertrophic obstructive cardiomyopathy (HOCM). The safety and feasibility of using this procedure for cryoablation are unknown. We aimed to investigate the feasibility and safety of echocardiography-guided percutaneous intramyocardial septal cryoablation (PIMSCA) for septal thickness reduction in a canine model.

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Introduction: Hypertrophic cardiomyopathy (HCM) is a heterogeneous genetic heart disease with an estimated prevalence in the general population of 0.2% to 0.6%.

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Myocardial contractility characteristics of hypertrophic cardiomyopathy patients with and without sarcomere mutation.

Heart Vessels

November 2024

Department of Ultrasound, Xijing Hypertrophic Cardiomyopathy Center, Xijing Hospital, Fourth Military Medical University, 127 Changle West Road, Xi'an, 710032, Shaanxi, China.

Hypertrophic cardiomyopathy (HCM) patients with sarcomere mutations have an increased risk of heart failure and left ventricular (LV) systolic dysfunction. We hypothesize that sarcomere mutation carriers have abnormal myocardial contractility before LV dysfunction. Therefore, we aimed to associate myocardial contractility with identified sarcomere mutations and predict genotyped HCM patients with sarcomere mutation by three-dimensional speckle tracking imaging (3D-STI).

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Quantitative analysis of four PAHs in oily sludge by surface-enhanced Raman spectroscopy (SERS) combined with partial least squares regression (PLS) based on a novel nano-silver-silicon coupling substrate.

Spectrochim Acta A Mol Biomol Spectrosc

October 2024

Key Laboratory of Synthetic and Natural Functional Molecular of the Ministry of Education, College of Chemistry & Material Science, Northwest University, Xi'an 710127, China. Electronic address:

Polycyclic aromatic hydrocarbons (PAHs) present in oily sludge generated by the petroleum and petrochemical industries have emerged as a prominent concern within the realm of environmental conservation. The precise determination of PAHs holds immense significance in both petroleum geochemistry and environmental protection. In this study, a combination of surface-enhanced Raman spectroscopy (SERS) and solid-liquid extraction was employed for the screening of PAHs in oily sludge.

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Article Synopsis
  • Percutaneous left atrial appendage closure (LAAC) is being evaluated as an alternative to anticoagulation (AC) for preventing strokes in patients with atrial fibrillation (AF) who have hypertrophic cardiomyopathy (HCM) and are at high bleeding risk.
  • A study compared the stroke risk between HCM-AF patients treated with LAAC and those treated with AC, utilizing data from the TriNetX Global Research Network through 2024.
  • Results showed that HCM patients receiving LAAC had higher rates of ischemic strokes and systemic embolism compared to those on AC, indicating that LAAC may not be effective as a primary treatment strategy for reducing stroke risk in this population.
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Machine Learning to Predict Future Disease-Specific Outcomes: The Brave New Frontier.

JACC Asia

May 2024

Hypertrophic Cardiomyopathy Center, Heart Vascular Thoracic Institute, Cleveland Clinic, Cleveland, Ohio, USA.

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Article Synopsis
  • The study examined a large group of patients (1468) diagnosed with hypertrophic cardiomyopathy (HCM) to understand the relationship between genetic status (genotype positive [G+] or negative [G-]) and clinical outcomes over an average follow-up of 7.8 years.
  • It found no significant differences in mortality rates or adverse heart events between G+ and G- patients, indicating that genetic factors did not influence the clinical course of the disease.
  • The researchers concluded that genotype status should not guide clinical management or predictions regarding outcomes in HCM patients, with age being the only significant factor affecting mortality and heart failure progression.
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The data on myocardial perfusion of the percutaneous intramyocardial septal radiofrequency ablation (PIMSRA) for obstructive hypertrophic cardiomyopathy (HOCM) are still lacking, although PIMSRA have been proved to be of great safety and efficacy. The aim of this study was to quantitatively analyze the changes in myocardial perfusion after PIMSRA using myocardial contrast echocardiography (MCE). 27 HOCM patients treated with PIMSRA were retrospectively analyzed, and their echocardiographic parameters and perfusion parameters of MCE were collected before and 12 months after PIMSRA.

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The burden and management competency of cardiomyopathies in China: a nationwide survey study.

Lancet Reg Health West Pac

May 2024

State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.

Background: The public health burden of cardiomyopathies and competency in their management by health agencies in China are not well understood.

Methods: This study adopted a multi-stage sampling method for hospital selection. In the first stage, nationwide tertiary hospital recruitment was performed.

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Article Synopsis
  • Calcium channel gene variations may be linked to hypertrophic cardiomyopathy (HCM), but their overall impact in the general population is still unclear.
  • A study involving 371 unrelated HCM patients revealed that those with double gene variations had a more severe form of HCM, characterized by younger age, thicker heart walls, and more significant electrical disturbances.
  • Patients with double gene variations also experienced worse outcomes during follow-up, and it was determined that these variations may cumulatively worsen the condition and its prognosis.
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Article Synopsis
  • Nonobstructive hypertrophic cardiomyopathy (nHCM) currently lacks approved treatments, but a new drug, ninerafaxstat, aims to improve heart energy and alleviate symptoms.
  • A clinical trial with 67 patients showed that ninerafaxstat led to better ventilatory efficiency after 12 weeks compared to a placebo, despite similar peak exercise levels.
  • Overall, ninerafaxstat was safe, well tolerated, and particularly beneficial for patients with more severe symptoms, indicating potential for improving their health and exercise capacity.
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