Renal Denervation in Hypertrophic Cardiomyopathy
Hypertrophic CardiomyopathyHypertrophic cardiomyopathy (HCM) is the most common inherited monogenic heart disease. There is an abnormal increase in myocardial mass in this disorder that leads to a state of cardiac sympathetic hypertonia, which is involved in disease progression, development of arrhythmias and heart failure. Cardiac sympathetic hyperactivity may constitute a new therapeutic target in HCM patients who persist symptomatic despite conventional treatment. The hypothesis of this project is that renal denervation (a minimally invasive percutaneous interventional therapy with proven efficacy in resistant arterial hypertension) reduces cardiac sympathetic activity in HCM. The SNYPER pilot study is a non-randomized clinical trial with medical devices (proof of concept), in which a renal denervation procedure will be performed in 20 patients with genetically confirmed sarcomeric HCM, severe left ventricular hypertrophy and persistent symptoms. The impact of denervation in reducing the 123I-meta iodo benzyl guanidine (MIBG) washout rate quantified by isotopic tracing (planar imaging and SPECT) at 6 months is established as a primary efficacy objective, and the proportion of renal denervation-related complications as a safety objective. The most relevant secondary endpoints are the outcomes of renal denervation on left ventricular mass (echocardiogram), diastolic function, maximum oxygen consumption (ergospirometer), ventricular arrhythmia burden (Holter), blood pressure (ABPM), N-terminal (NT) Pro Brain Natriuretic Peptide (BNP) and quality of life (KCCQ questionnaire). The results of this study may open the development of a new, technically simple and easily accessible therapeutic line for the treatment of HCM.
Transapical Beating-Heart Septal Myectomy in Patients With Symptomatic Nonobstructive Hypertrophic...
Nonobstructive Hypertrophic CardiomyopathyThe primary purpose of this study is to evaluate the feasibility, the safety and the efficacy of the transapical beating-heart septal myectomy for the treatment of nonobstructive hypertrophic cardiomyopathy. This is a prospective, single-arm, single-center study.
The Purpose of This Study is to Compare the Efficacy and Safety of Aficamten (CK-3773274) Compared...
Obstructive Hypertrophic Cardiomyopathy (oHCM)The purpose of this study is to compare the efficacy and safety of aficamten (CK-3773274) compared with metoprolol succinate in adults with symptomatic hypertrophic cardiomyopathy and left ventricular outflow tract obstruction
Transapical Beating-Heart Septal Myectomy in Patient With Hypertrophic Obstructive Cardiomyopathy:...
Hypertrophic Obstructive CardiomyopathyThe primary purpose of this study is to evaluate the feasibility, the safety and the efficacy of the transapical beating-heart septal myectomy for the treatment of hypertrophic obstructive cardiomyopathy. This is a prospective, single-arm, multi-center study.
The Effect of TASH in Patients With HOCM
Hypertrophic Obstructive CardiomyopathyTo examine patients with hypertrophic obstructive cardiomyopathy (HOCM) before and after septal alcohol ablation, to investigate the effect of the treatment in regards to changes in myocardial function, perfusion, invasive hemodynamics and exercise tolerance.
Study of Safety and Tolerability of TN-201 in Adults With Symptomatic MYBPC3 Mutation-associated...
Hypertrophic CardiomyopathyThis is a first-in-human, non-randomized, open-label study designed to evaluate the safety, tolerability, and pharmacodynamics (PD) of TN-201 in adult patients with symptomatic MYBPC3 mutation-associated nonobstructive hypertrophic cardiomyopathy (nHCM).
Reverse Remodeling After PTSMA in Severe But Asymptomatic LVOT Obstruction
HOCMHypertrophic Obstructive Cardiomyopathy3 morePatients with HOCM and severe LVOT obstruction can remain asymptomatic while significant cellular and structural changes of the heart (adverse remodeling) may occur preceding heart failure and rhythm disorders. Hence, preventing adverse remodeling through LVOT desobstruction may have significant impact on cardiac function and geometry in this particular population, as it is in symptomatic patients. The investigators will assess functional and structural characteristics of the myocardium in asymptomatic vs. symptomatic patients with severe LVOT obstruction before and after PTSMA, using advanced imaging studies with LGE-CMR and echocardiography.
The Efficacy and Mechanism of Trientine in Patients With Hypertrophic Cardiomyopathy
Hypertrophic CardiomyopathyThis research study has been designed to test whether a drug called trientine dihydrochloride (also called Cufence) reduces heart muscle thickening, improves exercise capacity, improves heart function and reduces abnormal heart rhythms in patients with hypertrophic cardiomyopathy (HCM). The study is also assessing how trientine works in HCM. Participants will be prescribed either trientine or placebo, for a period of 12 months.
Permanent Pacing for Drug-refractory Symptomatic Patients With Obstructive Hypertrophic Cardiomyopathy....
Obstructive Hypertrophic CardiomyopathyThe present trial is undertaken as a pilot study on selected patients, to evaluate the ability of acute hemodynamic testing to optimize pacing therapy and to predict the response of Obstructive Hypertrophic Cardiomyopathy (OHCM) to pacing, in terms of reduction of Left Ventricular Outflow Tract (LVOT) pressure gradient and functional capacity improvement. The Primary Objective is to demonstrate a long-term reduction in LVOT obstruction at rest and/or after Valsalva/Exercise > 30% in patients responding to acute stimulation test
Surgical Septal Myectomy vs Percutaneous Transluminal Alcohol Septal Ablation in Patients With Hypertrophic...
Hypertrophic CardiomyopathyThe aim of this randomized trial is to compare the improvement in exercise capacity among patients with highly symptomatic hypertrophic obstructive cardiomyopathy despite optimal medical treatment who undergo alcohol septal ablation (ASA) or surgical septal myectomy (SSM).