Active clinical trials for "Cardiomyopathy, Hypertrophic"

Hypertrophic Obstructive cardiomyopathy (HOCM) is the most common genetic cardiomyopathy, heterogeneous in phenotype and clinical course. The genotype-phenotype relationship and associated molecular mechanisms are still incompletely understood. In the HOCM milieu, increased energy cost of force production, impairing performance and mitochondrial function, may be associated to patients' genotype and/or phenotype

Left ventricular obstruction is an invalidating complication of hypertrophic cardiomyopathies (HCM), and endothelial dysfunction has also been observed in these pathologies. However, the relation between obstruction and endothelial and venous dysfunctions has not been previously studied. The main objective is to investigate the relations between endothelial and venous dysfunctions and symptomatic left ventricular outflow-tract obstruction in HCM patients.

Cardiac amyloidosis are related to the accumulation of fibrillar proteins in the extracellular leading to disruption of the cardiac tissue architecture. Amyloidosis in transthyretin (TTR) are the most common hereditary amyloidosis but remain poorly studied at heart. This is serious and deadly. The prevalence of TTR amyloidosis is probably underestimated in hypertrophic cardiomyopathy (HCM) often of unknown etiology because of the lack of systematic implementation of myocardial biopsy because of their side effects.

This study will examine the relationship between certain measures of heart function and exercise capacity in patients with hypertrophic cardiomyopathy (HCM). Patients who participated in NHLBI studies 01-H-0006 ("Double Blind Placebo-Controlled Study of Pirfenidone - A Novel Anti-Fibrotic Drug - in Symptomatic Patients with Hypertrophic Cardiomyopathy Associated with Left Ventricular Diastolic Dysfunction") and 96-H-0144 ("Double Blind Placebo-Controlled Study of Long-Term Effects of Angiotensin-Converting Enzyme Inhibition (Enalapril) and Angiotensin II Receptor Blockade (Losartan) on Genetically-Induced Left Ventricular Diastolic Dysfunction") are eligible for this study. Data from echocardiograms and measures of left ventricular pressure obtained from patients in those studies will be analyzed in the current study to assess their influence on exercise capacity. No additional tests, treatments or other procedures are required. Information from this study may help in the development of improved drug treatments for HCM.

The human heart is divided into four chambers. One of the four chambers, the left ventricle, is the chamber mainly responsible for pumping blood out of the heart into circulation. Hypertrophic cardiomyopathy (HCM) is a genetically inherited disease causing an abnormal thickening of the heart muscle, especially the muscle making up the left ventricle. When the left ventricle becomes abnormally large it is called left ventricular hypertrophy (LVH). This condition can cause symptoms of chest pain, shortness of breath, fatigue, and heart beat palpitations. This study is designed to compare the ability of two drugs (enalapril and losartan) to improve symptoms and heart function of patients diagnosed with hypertrophic cardiomyopathy (HCM). Researchers have decided to compare these drugs because each one has been used to treat patients with other diseases causing thickening of the heart muscle. In these other conditions, enalapril and losartan have improved symptoms, decreased the thickness of heart muscle, improved blood flow and supply to the heart muscle, and improved the pumping action of the heart muscle. In this study researchers will compare the effectiveness of enalapril and losartan when given separately and together to patients with hypertrophic cardiomyopathy (HCM).

In some patients, septal hypertrophy extends more distally, from the subaortic portion of the septum to the midventricular portion. In these patients, classic transaortic surgical myectomy may not be effective in removing the midventricular obstruction, resulting in a suboptimal surgical outcome. These patients may present recurrence of symptoms and not have an improvement in the prognosis related to the treatment of hypertrophic cardiomyopathy, in some cases determining the need for reoperation. Since 2015, our Institute has used a surgical technique that allows us to improve transaortic exposure of the interventricular septum, using a probe with application of cryoenergy the hypertrophic portion of the septum is hooked and in this way the myectomy can be extended more distally, performing a more complete removal of the myocardium. The aim of this study is to compare the results obtained with classical myectomy compared to myectomy performed with the aid of cryoenergy. The primary endpoint is the comparison in terms of mortality between patients undergoing classical myectomy versus those undergoing cryoenergy-assisted myectomy. Secondary endpoints are: extent of myectomy, persistence of residual left ventricular outflow tract obstruction, persistence of mitral regurgitation related to systolic anterior motion of the mitral leaflets, occurrence of ventricular defect, and need for PM implantation.

Hypertrophic cardiomyopathy (HCM) is a common genetic cardiovascular disease. Outflow-tract gradient of 30 mmHg or more under resting conditions is an independent determinant of symptoms of progressive heart failure and death. The investigators hypothesize that the electrical approach by dual chamber pacing could improve symptoms and reduce outflow-tract obstruction in a specific sub-group of selected patients with a mechanical hyper-synchronicity. The aim of the study is to identify and describe this phenomenon in HCM with (O-HCM) and without (NO-HCM) outflow-tract obstruction thanks to innovative multi-imaging approach.

Study population: 1) mutation carriers without the hypertrophic phenotype (pre-clinical Hypertrophic Cardiomyopathy (HCM)) and in 2) patients with clinically overt HCM (clinical HCM). Hypothesis: Cardiac troponin release after exercise can be demonstrated in both clinical and pre-clinical HCM patients.

This study will examine how heart stiffness and a weak atrium affect exercise capacity and symptoms in patients with hypertrophic cardiomyopathy (HCM). The atrium is the booster pumping chamber of the heart that helps the ventricle (main pumping chamber), to fill properly. HCM is an inherited disease in which the ventricle becomes thickened and, in some patients, stiff. The stiffness makes it difficult for the ventricle to fill and empty, causing breathing difficulty, fatigue, and reduced exercise capacity. Scar formation and a weakened atrium can cause the heart to stiffen. Information gained from this study may guide doctors in prescribing medicines to reduce scarring or improve atrial function. Patients 21 years of age and older with hypertrophic cardiomyopathy may be eligible for this study. Candidates will be screened with a medical history and physical examination, electrocardiogram (EKG), blood tests, Holter monitor, and echocardiogram. A Holter monitor is a device about the size of a Walkman that is connected to three wires that are attached to the chest. It is worn for 24 hours to provide continuous monitoring of heart rhythm. An echocardiogram uses a small probe that emits sound waves to produce images of the heart. The probe is moved across the chest and the reflection of the sound waves from the chambers of the heart produce images showing the heart's thickness and function. Participants will undergo the following tests and procedures over 3 days: Physical examination and echocardiogram. Intravenous cannula insertion: A plastic tube is inserted into an arm vein for collecting blood samples to measure substances that the heart and circulatory system release at rest and during exercise. Impedance cardiography: A small current of electricity is passed across the chest and electrodes similar to those used for an EKG test are placed to measure blood flow in the area of the current. Pulmonary artery catheterization: A catheter (plastic tube) is inserted into a vein either in the arm, under the collarbone, or in the neck and advanced to the right atrium and ventricle. The catheter remains in place during the echocardiogram tilt and bicycle exercise tests (see below). Echocardiogram tilt test: The patient lies flat on a table. After a few minutes, the table is tilted so that the patient's head is just above his or her feet for a short while, then is positioned flat again, and then tilted so the feet are just above the head. Echocardiographic measurements and blood samples are taken at intervals to examine heart function during changes in posture. Echocardiogram bicycle stress test: The patient exercises for as long as possible on a bicycle-like machine while lying on his or her back. Echocardiographic measurements and blood samples are taken at intervals during the test. Treadmill stress test: The patient runs for as long as possible on a treadmill that increases in difficulty. The patient wears a facemask or mouthpiece through which small amounts of gases are added in order to measure the ability of the heart and lung to increase their effectiveness with exercise. Digoxin loading: Only patients who demonstrate limited exercise capacity and for whom digoxin is not a risk will undergo this procedure. A medicine that makes the heart contract more strongly, digoxin is used to treat certain heart abnormalities. Patients are given doses of either digoxin or placebo (a look-alike injection with no active ingredient) at 4-hour intervals over a 24-hour period and then repeat the tilt test and the bicycle and treadmill exercise tests

This study is being done to determine whether or not new blood test(s) can determine the severity of heart conditions. Aortic stenosis, hypertrophic cardiomyopathy, mitral regurgitation, aortic regurgitation, artificial heart valve regurgitation or stenosis, and tricuspid valve regurgitation associated with pacemaker leads are the cardiac disorders under study. The blood tests involve analysis for von Willebrand Factor antigen and activity, von Willebrand Factor multimers, and brain natriuretic peptide (BNP) levels. The results of the blood tests will be compared to the information from the clinically-indicated echocardiogram and one blood test compared to another.