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

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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Background: The phase 2 PIONEER-HCM (Phase 2 Open-label Pilot Study Evaluating Mavacamten in Subjects With Symptomatic Hypertrophic Cardiomyopathy and Left Ventricular Outflow Tract Obstruction) study showed that mavacamten improved left ventricular outflow tract gradients, exercise capacity, and symptoms in patients with obstructive hypertrophic cardiomyopathy (HCM), but the results of longer-term treatment are less well described. We report interim results from the PIONEER-OLE (PIONEER Open-Label Extension) study, the longest-term study of mavacamten in patients with symptomatic obstructive HCM.

Methods And Results: Patients who previously completed PIONEER-HCM (n=20) were eligible to enroll in PIONEER-OLE.

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A case report of percutaneous intramyocardial septal radiofrequency ablation in an adult with re-obstruction after Morrow procedure.

Eur Heart J Case Rep

April 2024

Xijing Hypertrophic Cardiomyopathy Center, Department of Ultrasound, Xijing Hospital, Airforce Military Medical University, Xi'an, Shannxi 710032, China.

Background: Some patients with hypertrophic cardiomyopathy (HCM) re-occur with drug-refractory symptoms but are not eligible for re-operation after the Morrow procedure. Traditional treatment options are limited. We present the first case of the use of ultrasound-guided percutaneous intramyocardial septal radiofrequency ablation (PIMSRA) for the treatment of a patient with HCM combined with congenital anatomically corrected malposition of the great arteries (MGA) after Morrow procedure.

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Background: Estimated plasma volume status (ePVS) estimated by the Duarte formula is associated with clinical outcomes in patients with heart failure. It remains unclear the predictive value of the ePVS to the postoperative hypotension (POH) in percutaneous intramyocardial septal radiofrequency ablation (PIMSRA) treating hypertrophic obstructive cardiomyopathy (HOCM).

Methods: Data of HOCM patients who underwent PIMSRA were retrospectively collected.

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Article Synopsis
  • This phase 2 trial assessed the safety and effectiveness of a medication called aficamten in patients suffering from nonobstructive hypertrophic cardiomyopathy (nHCM).
  • 41 patients participated, and after 10 weeks of treatment, over half showed improvement in heart failure symptoms, with many reaching a better functional class.
  • While some patients experienced a slight decrease in heart function (LVEF), the overall results indicated that aficamten is generally safe and effective in improving symptoms and relevant heart health markers.
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"On/off"-switchable crosslinked PTX-nanoformulation with improved precise delivery for NSCLC brain metastases and restrained adverse reaction over nab-PTX.

Biomaterials

June 2024

Department of Oncology, The Second Affiliated Hospital, Medical School of Xi'an Jiaotong University, Xi'an, Shaanxi, 710061, China; School of Basic Medical Sciences, Key Laboratory of Environment and Genes Related to Diseases of Ministry of Education, Xi'an Jiaotong University, Xi'an, Shaanxi, 710061, China. Electronic address:

Non-small cell lung cancer (NSCLC) brain metastases present a significant treatment challenge due to limited drug delivery efficiency and severe adverse reactions. In this study, we address these challenges by designing a "on/off" switchable crosslinked paclitaxel (PTX) nanocarrier, BPM-PD, with novel ultra-pH-sensitive linkages (pH 6.8 to 6.

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Percutaneous intramyocardial septal radiofrequency ablation after 5-year follow-up.

Heart

May 2024

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

Objective: The objective is to evaluate the 5-year follow-up results of percutaneous intramyocardial septal radiofrequency ablation (PIMSRA) for hypertrophic obstructive cardiomyopathy (HOCM), including clinical status, electrocardiographic and echocardiographic characteristics.

Methods: 27 patients (age: 44.3±15.

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Beating-Heart Septal Myectomy: An Innovative Approach for Hypertrophic Obstructive Cardiomyopathy.

JACC Asia

February 2024

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

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Medical Treatment Strategies for Hypertrophic Cardiomyopathy.

Am J Cardiol

February 2024

The Hypertrophic Cardiomyopathy Center, Department of Cardiovascular Medicine, Cleveland Clinic Foundation, Cleveland, Ohio. Electronic address:

Hypertrophic cardiomyopathy (HCM) is a heterogeneous genetic heart disease inherited in an autosomal dominant pattern with an estimated prevalence of 0.6% in the general population. Clinical manifestations of HCM vary considerably, with symptoms ranging from none or mild exercise intolerance to severe lifestyle-limiting symptoms, advanced heart failure, and sudden cardiac death.

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Role of Imaging in the Diagnosis, Evaluation, and Management of Hypertrophic Cardiomyopathy.

Am J Cardiol

February 2024

UCSF Hypertrophic Cardiomyopathy Center of Excellence, Division of Cardiology, University of California San Francisco, San Francisco, California. Electronic address:

Hypertrophic cardiomyopathy (HCM) is increasingly recognized and may benefit from the recent approval of new, targeted medical therapy. Successful management of HCM is dependent on early and accurate diagnosis. The lack of a definitive diagnostic test, the wide variation in phenotype and the commonness of phenocopy conditions, and the presence of normal or hyperdynamic left ventricular function in most patients makes HCM a condition that is highly dependent on imaging for all aspects of management including, diagnosis, classification, predicting risk of complications, detecting complications, identifying risk for ventricular arrhythmias, evaluating choice of therapy and monitoring therapy, intraprocedural guidance, and screening family members.

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FARS2 Deficiency Causes Cardiomyopathy by Disrupting Mitochondrial Homeostasis and the Mitochondrial Quality Control System.

Circulation

April 2024

Department of Biochemistry and Molecular Biology, Shaanxi Provincial Key Laboratory of Clinical Genetics (B.L., X.C., T.C., J.Z., Y.L., Y.Y., W.H., M.Z., Y.W.), Air Force Medical University, Xi'an, China.

Background: Hypertrophic cardiomyopathy (HCM) is a common heritable heart disease. Although HCM has been reported to be associated with many variants of genes involved in sarcomeric protein biomechanics, pathogenic genes have not been identified in patients with partial HCM. FARS2 (the mitochondrial phenylalanyl-tRNA synthetase), a type of mitochondrial aminoacyl-tRNA synthetase, plays a role in the mitochondrial translation machinery.

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Real-world experience with mavacamten in obstructive hypertrophic cardiomyopathy: Observations from a tertiary care center.

Prog Cardiovasc Dis

October 2024

From the Hypertrophic Cardiomyopathy Center, Heart and Vascular Institute, Cleveland Clinic, Weston, Cleveland, OH, USA.

Article Synopsis
  • Mavacamten is approved for treating symptomatic obstructive hypertrophic cardiomyopathy (oHCM) and aims to improve left ventricular outflow tract gradients and reduce the need for septal reduction therapy (SRT).
  • A study of 150 patients showed significant improvements in New York Heart Association (NYHA) class and reduced LVOT gradients after 12 weeks of mavacamten treatment, with 46% of patients showing at least one class improvement.
  • Although there was a slight decline in left ventricular ejection fraction (LVEF), no patients required SRT or experienced severe heart failure complications during the study period.
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Introduction: In hypertrophic cardiomyopathy (HCM), atrial fibrillation (AF) has historically been regarded to have a deleterious impact on clinical course, strongly associated with progressive heart failure (HF) symptoms. However, there is a paucity of information regarding the impact of AF on HCM employing validated quality of life (QoL) surveys. Therefore, we evaluated the impact of AF on QoL utilizing patient reported outcome measures (PROMs).

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Echocardiography-guided percutaneous intramyocardial alginate hydrogel implants for heart failure: canine models with 6-month outcomes.

Front Cardiovasc Med

January 2024

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

Background: Echocardiography-guided percutaneous intramyocardial alginate-hydrogel implantation (PIMAHI) is a novel treatment approach for heart failure (HF). We validated PIMAHI safety and efficacy in canine HF models.

Methods: Fourteen canines with HF [produced by coronary artery ligation, left ventricular ejection fraction (LVEF) < 35%] were randomised to PIMAHI treatment ( = 8) or controls ( = 6).

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Exercise After Acute Myocarditis: When and How to Return to Sports.

Card Electrophysiol Clin

March 2024

Chanin T. Mast Hypertrophic Cardiomyopathy Center and Sports Cardiology, Atlantic Health, Morristown Medical Center, 111 Madison Avenue, Morristown, NJ, USA. Electronic address:

Myocarditis is an inflammatory disease of the myocardium secondary to infectious and noninfectious insults. The most feared consequence of myocarditis is sudden cardiac death owing to electrical instability and arrhythmia. Typical presenting symptoms include chest pain, dyspnea, palpitations and/or heart failure.

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Article Synopsis
  • Patients with hypertrophic cardiomyopathy (HCM) face a risk of sudden death, especially if they have major risk markers, leading to the consideration of implantable cardioverter-defibrillators and cardiac magnetic resonance (CMR) imaging for risk assessment.
  • A study aimed to develop deep-learning models based on electrocardiogram (ECG) data to identify high-risk imaging features in HCM patients, using a cohort from Tufts Medical Center in Boston.
  • The ECG deep-learning models showed reliable identification of high-risk features and could potentially reduce the need for CMR testing by using a screening strategy that combines echocardiography with selective CMR recommendations.
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The Revolution of Cardiac Myosin Inhibitors in Patients With Hypertrophic Cardiomyopathy.

Can J Cardiol

May 2024

The Hypertrophic Cardiomyopathy Center, Knight Cardiovascular Institute, Oregon Health & Science University, Portland, Oregon, USA. Electronic address:

Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiomyopathy worldwide and causes significant morbidity and mortality. For decades, medical treatment options have been limited and untargeted, with frequent need for invasive interventions not readily accessible to many HCM patients. More recently, our understanding of the genetic basis and pathophysiologic mechanism of HCM has grown significantly, leading to the discovery of a new class of medications, cardiac myosin inhibitors (CMIs), that shift myosin into the super-relaxed state to counteract the hypercontractility in HCM.

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Heart failure (HF) is characterized by a progressive clinical course marked by frequent exacerbations and repeated hospitalizations, leading to considerably high morbidity and mortality rates. Patients with HF present with a constellation of bothersome symptoms, which range from physical to psychological and mental manifestations. With the transition to more advanced HF stages, symptoms become increasingly more debilitating, interfere with activities of daily living and disrupt multiple domains of life, including physical functioning, psychological status, emotional state, cognitive function, intimate relationships, lifestyle status, usual role activities, social contact and support.

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