Exercise Training in Hypertrophic Cardiomyopathy: (SAFE-HCM)
Hypertrophic CardiomyopathyTo explore the feasibility, safety, health and psychological benefits of a 12-week high intensity exercise programme in a young group of individuals with hypertrophic cardiomyopathy (HCM). This will pave the way for a large-scale randomised study of safety of exercise in HCM, the results of which will strengthen the evidence base for exercise recommendations.
Microvascular Dysfunction in Nonischemic Cardiomyopathy: Insights From CMR Assessment of Coronary...
Hypertrophic CardiomyopathyNon-ischemic Dilated Cardiomyopathy1 moreThe aim of this study is to assess microvascular function as determined by a cardiovascular magnetic resonance measurement of whole-heart (global) perfusion reserve. The goal is to determine the prevalence of MVD in two common forms of non-ischemic cardiomyopathy, hypertrophic cardiomyopathy (HCM) and idiopathic dilated cardiomyopathy (IDCM). The hypothesis that an optimized technique will provide robust detection of MVD and that a multifaceted approach will provide new insights into the pathophysiology of MVD, including the influence of myocardial scarring upon the presence and severity of MVD.
Echocardiography-guided Transthoracic Radio Frequency/Laser Ablation for Ventricular Septum of Hypertrophic...
Hypertrophic Obstructive CardiomyopathyHypertrophic Obstructive Cardiomyopathy (HOCM) patients have significant clinical symptoms, including progressively increasing fatigue, angina, exertional dyspnea, and syncope. Conservative medications are used to treat the vast majority of patients. Invasive therapy, which includes surgical myectomy, septal ethanol ablation and dual-chamber pacing is introduced to patients with refractory symptoms or drug resistance. Considering the sternotomy and relatively high patients' tolerance required in myectomy, the potentially risky misplacement of ethanol and the anatomic variability of the vascularised hypertrophic septum, and the potential risk of conduction block after these two treatments, the development of new minimally invasive approach is warranted. Previous researches have illustrated the effectiveness and feasibility of transcatheter radio frequency ablation for HOCM patients. By far, there has been no report on transthoracic laser-induced interstitial thermotherapy (LITT) for human treatment. Since 2004, our department has adopted High Intensity Focused Ultrasound, radio frequency and laser in solid tumors treatment, including liver tumors and fibroid. Also our center has conducted several animal experiments to verify the feasibility of radio frequency/laser in septal myocardium ablation. The purpose of this study is to lead echocardiography-guided transthoracic radio frequency/laser ablation for HOCM ventricular septum, make minimally invasive treatment plans for HOCM patients, and verify the safety and validity of intervention treatment in long term.
The Clinical Research of the Safety and Effectiveness Evaluation for Using Echocardiography-guided...
Hypertrophic Obstructive CardiomyopathyObjective to investigate the safety and effectiveness of Echocardiography-guided radiofrequency ablation in patients with Hypertrophic obstructive Cardiomyopathy (HOCM). Percutaneous intramyocardial septal radiofrequency ablation (Liwen Procedure) is a safe and effective treatment approach for Hypertrophic obstructive Cardiomyopathy and results in sustained improvement in exercise capacity, persistent in reducing Left Ventricle Outflow Tract (LVOT) gradient, and sustained improvement in cardiac function. In patients with disabling symptoms caused by Hypertrophic obstructive Cardiomyopathy (HOCM),Echocardiography-guided radiofrequency ablation could be a less invasive treatment option. Percutaneous intramyocardial septal radiofrequency ablation (Liwen Procedure) is a new method for the diagnosis or treatment of heart disease by using a special diagnosis and treatment device to the heart target area under the guidance of image technology. The method breaks through the worldwide problem of the minimally invasive diagnosis and treatment of the myocardium on the beating heart, so as to avoid the X-ray radiation and contrast agent damage . As a new pathway of cardiac disease intervention, Liwen Procedure can be used in congenital heart disease, myocardial biopsies, drug injection, cell implantation and instrument implantation in addition to Hypertrophic Cardiomyopathy and cardiac tumors. It has important clinical value and broad application prospect. In this study, Liwen RF radiofrequency ablation system was used to treat HOCM , and evaluate its safety and effectiveness , in order to provide a new medical device for Liwen Procedure of HOCM.
Comparison of Squat-to-Stand Maneuver With Amyl Nitrite, Valsalva, and Exercise Stress Echocardiography...
Hypertrophic CardiomyopathyHypertrophic Obstructive Cardiomyopathy2 moreThe purpose of this study is to assess the effectiveness of squat-to-stand maneuver in eliciting left ventricular outflow gradients in patients with Hypertrophic Cardiomyopathy (HCM) compared to Valsalva, amyl nitrite inhalation, and exercise stress echocardiogram (ESE).
Trimetazidine Therapy in Hypertrophic Cardiomyopathy
Hypertrophic CardiomyopathyHypertrophic cardiomyopathy (HCM) is a common inherited heart condition that causes breathlessness, chest pain and fatigue. There are few treatments available. The investigators have recently shown that a drug called perhexiline reduced symptoms and improved exercise capacity in patients with HCM. This change appears to be driven by alterations in myocardial energy metabolism. The aim of this trial is to test a similar drug, trimetazidine, in a group of symptomatic patients with non-obstructive HCM. HYPOTHESIS: trimetazidine will improve symptoms, peak oxygen consumption, cardiac function and arrhythmia burden in medically refractory symptomatic patients with non-obstructive HCM.
Role of Perhexiline in Hypertrophic Cardiomyopathy
Hypertrophic CardiomyopathyHypertrophic Cardiomyopathy (HCM) is the most common inherited heart muscle condition affecting up to 1 in 200 of the general population. It results from mutations in genes encoding components of the contractile apparatus in the heart muscle cell (myocyte). These mutations result in increased energy cost of force production for the myocyte which then cumulatively causes a myocardial energy deficit. This myocardial energy deficit is then thought to lead to cardiac hypertrophy ('left ventricular hypertrophy' or LVH) in HCM. LVH leads to impairments in heart muscle function, heart muscle oxygenation and microvascular blood flow and is the chief driver of patient symptoms in HCM. These symptoms consist of chest pain, shortness of breath, dizziness, fainting episodes or palpitations. Occasionally, the disease may cause sudden cardiac death (SCD). HCM is the most common cause of SCD in young people including competitive athletes. In addition, HCM has been found to result in significant global deterioration in health-related quality of life. Treatment of HCM has focused on relief of symptoms by drugs such as ß-blockers which slow the heart rate and improve heart function. However, symptom relief is often incomplete and there is no evidence on the benefit of ß-blockers or related medications to reverse LVH. Perhexiline, a potent carnitine palmitoyl transferase-1 (CPT-1) inhibitor shifts myocardial metabolism to more efficient glucose utilisation and rectifies impaired myocardial energetics. It is currently used to treat angina in patients with coronary artery disease. There is some preliminary evidence that Perhexiline may aid in the improvement of symptoms in patients with HCM. However, the effect of any form of therapy on potential regression of LVH in HCM remains unexplored. In this randomised double-blind placebo-controlled trial, the investigators will use state of the art cardiac imaging, principally advanced echocardiography and Cardiovascular Magnetic Resonance (CMR) to study the effects of perhexiline on LVH, cardiac function, and oxygenation in symptomatic patients with HCM. The investigators hypothesize that perhexiline will favourably reduce LVH and improve myocardial oxygenation by improving myocardial energetics, and that these putative morphological and functional changes can be accurately measured utilizing echocardiography and CMR. If this pilot study supports the hypothesis, then it will pave the way for a major randomised controlled trial to definitely determine the role of Perhexiline in HCM.
Non-Invasive Radiation Ablation in Patients With Hypertrophic CardioMyopathy: NIRA-HOCM
Hypertrophic CardiomyopathyHypertrophic cardiomyopathy (HCM) is a common disease of the heart which causes thickening of the heart muscle. HCM primarily affects the muscle of the main pumping chamber of the heart (the left ventricle) and particularly the septum (this is the muscular wall which separates the right and left side of the heart). In a subgroup of patients, the thickened heart muscle at the septum prevents blood from leaving the heart during contraction (this is called obstruction). This form of the disease is called hypertrophic obstructive cardiomyopathy (HOCM). HOCM is a common cause of shortness of breath, chest pain and dizzy spells. These symptoms are treated with tablets and if symptoms are uncontrolled, patients are often offered invasive treatment to get rid of some of the thick heart muscle and reduce obstruction. This is achieved either by: open heart surgery (myectomy) where a surgeon cuts out the thick muscle injection of alcohol to the thick heart muscle via a tube in the wrist or groin (alcohol septal ablation). The alcohol thins the heart muscle at the point of obstruction, mimicking the effects of myectomy. Unfortunately, some patients are not suitable for both these procedures. This study will test whether radiotherapy, usually used for the treatment of tumours, can be used to destroy the thick heart muscle at the point of obstruction safely and effectively. Study patients will be monitored following the procedure and the investigators plan to measure the levels of heart muscle thinning, reduction of obstruction and improvement in symptoms and importantly document any side effects. Radiotherapy works by precisely targeting high energy X-rays (ionising radiation) at a specific area of the body with the aim of destroying abnormal tissue. CyberKnife is one of the latest radiotherapy delivery systems, which will deliver highly focussed and accurate radiotherapy.
HCM-AF Ablation With ACUTUS
Atrial FibrillationHypertrophic CardiomyopathyBACKGROUND About 1/4 of patients with hypertrophic cardiomyopathy (HCM) seem to develop atrial fibrillation (AF) over their life-span. Typically, symptoms of heart failure and especially shortness of breath get much worse once AF is present. Catheter ablation of AF in HCM has been proposed by several centres, but outcomes are much worse than in non-HCM AF. Accurate mapping of the arrhythmia is crucial with regard to improving the procedural outcome. Interestingly, intracardiac mapping during AF has demonstrated very long average cycle length during ongoing AF in HCM which should make identification of the critical re-entry/rotors much easier using dipole cardiac mapping (Acutus mapping system, Acutus Medical, CA, USA). POPULATION and PURPOSE This is a pilot trial recruiting a total of 20 patients with HCM and AF (paroxysmal or persistent with <12 months duration time in persistent AF) eligible for catheter ablation, without other significant structural heart disease Primary endpoints Safety: Absence of acute adverse events due to the use of ACUTUS mapping system during AF ablation Evidence of chronic adverse events due to the use of ACUTUS mapping system guided catheter ablation during the 12 months F/U period Safety endpoint of the entire mapping and ablation strategy Efficacy: Assessment on efficacy of ACUTUS mapping system guided AF ablation in HCM patients using a double-arm study design RF time to termination of AF to SR Secondary endpoints RF time to termination of AF to atrial tachycardia (AT) Freedom from AF/flutter/tachycardia (> 30 sec) at the end of the 12 months follow up (F/U) period Time to first recurrence of AF/flutter/tachycardia (> 30 sec) Freedom of AF/flutter/tachycardia on previously failed anti-arrhythmic medication Ablation procedure First 10 patients (group 1): ablation will be carried out after acquisition of a left atrium (LA) and right atrium (RA) dipole map at baseline, pre and post administration of Adenosine IV. Then pulmonary vein isolation (PVI) as a first step and subsequent remap and ablation of all patterns of interest in the LA until restoration of sinus rhythm (SR) or decision to proceed with direct current cardioversion (DCCV, 360J). Second 10 patients (group 2): after the acquisition of a dipole map of LA and RA at baseline (pre and post Adenosine IV administration), ablation of all identified areas of interest (API) will be performed, followed by remap and finally PVI +/- DCCV. For all patients: final step will be the deployment of a RA isthmus line and demonstration of bidirectional block. FOLLOW UP Patients will be followed up at 3, 6, and 12 months.
Efficacy of Psychotherapy for Improving Quality of Life in Patients With Hypertrophic Cardiomyopathy...
Hypertrophic CardiomyopathyDepression2 moreA Study to evaluate the efficacy of psychotherapy for easing the cardiac symptoms and improving and quality of life in patients with hypertrophic cardiomyopathy accompanied with depression