Materiały źródłowe

• #1 Patient education: Hypertrophic cardiomyopathy (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/hypertrophic-cardiomyopathy-beyond-the-basics

  HYPERTROPHIC CARDIOMYOPATHY TREATMENT […] There is no cure for hypertrophic cardiomyopathy (HCM). However, several medical and invasive treatments are available to relieve or eliminate symptoms, providing the vast majority of people with HCM the opportunity to achieve normal longevity with an excellent quality of life. […] Treatment may be recommended to: […] Reduce or eliminate the burden of limiting symptoms, such as shortness of breath (commonly experienced with inclines such as stairs or hills and less commonly walking on level ground), exertional fatigue, strong heart beats (palpitations), and chest discomfort. […] Protect against future stroke risk. […] Treat abnormal heart rhythms and/or reduce the risk of sudden death. […] The optimal treatment depends upon the individual situation. Most people with HCM have normal longevity and do not develop symptoms or require any treatment.

• #1 Hypertrophic Cardiomyopathy | Tufts Medicine

  https://www.tuftsmedicine.org/services-treatments/cardiovascular-care/hypertrophic-cardiomyopathy

  Many people with HCM successfully control their symptoms for years or even decades with medication alone. Some of the most commonly prescribed medications for HCM include: Antiarrhythmic drugs: Help control irregular heartbeats. Anticoagulants (blood thinners): Reduce blood clot risk, especially with arrhythmia or atrial fibrillation. Beta blockers: Slow the heart rate and reduce symptoms like chest pain or palpitations. Calcium channel blockers: Relax blood vessels and help manage symptoms. Cardiac myosin inhibitors: A new FDA-approved medicine that helps the heart muscle work better in people with HCM. Sodium channel blockers: Control obstructive HCM symptoms. […] When medications aren’t enough to manage your symptoms, we may consider minimally invasive procedures, such as alcohol septal ablation or catheter ablation for recurrent AFib. These procedures may also be recommended for older adults who haven’t responded well to medication.

• #1 Pediatric Hypertrophic Cardiomyopathy Treatment & Management: Approach Considerations, Pharmacologic Therapy, Left Ventricular Myectomy

  https://emedicine.medscape.com/article/890068-treatment

  Medical management of hypertrophic cardiomyopathy (HCM) in children should focus on the following: […] Patients with symptoms or evidence of outflow tract obstruction are generally started on calcium channel blocker or beta-blocker therapy. […] Patients with severe outflow tract obstruction may be candidates for surgical myectomy. […] The 2011 joint American College of Cardiology Foundation (ACCF)/American Heart Association (AHA) recommendations for symptomatic patients with HCM are outlined below. […] For symptomatic HCM (angina or dyspnea) refractory to low doses of beta-blockers: Titrate the dose to a resting heart rate below 60-65 bpm (up to a maximum dose based on the recommended dosing of these agents). […] For symptomatic (angina or dyspnea) HCM (obstructive or nonobstructive) refractory to beta-blockers or in patients intolerant of, or with contraindications to, beta-blockers: Initiate low-verapamil therapy and titrate up to 480 mg/day.

• #1 How Hypertrophic Cardiomyopathy Is Treated

  https://www.verywellhealth.com/hypertrophic-cardiomyopathy-treatment-5222066

  The primary treatment options for hypertrophic cardiomyopathy (HCM) are medications like beta-blockers and calcium channel blockers. […] If symptoms continue despite medical therapy, more invasive therapies like procedures or device installation may be recommended. […] Beta-blockers are the first-line treatment for symptoms of HCM. […] Beta blockers used to treat HCM include: Atenolol, Bisoprolol, Metoprolol. […] The calcium channel blockers verapamil and diltiazem may be used in place of beta-blockers in people who do not tolerate beta-blockers. […] When beta-blockers and calcium channel blockers fail to control symptoms in HCM, disopyramide may be used. […] Mavacamten is a new therapy that has been recently approved by the Food and Drug Administration (FDA) to treat symptomatic HCM.

• #1 Pharmacological Management of Hypertrophic Cardiomyopathy: From Bench to Bedside

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9209358/

  In oHCM patients with gradient exceeding 50 mmHg, persisting even after maximal drug therapy, current guidelines recommend invasive septal reduction therapies with either surgical septal myectomy or catheter-based alcohol septal ablation. […] However, novel compounds have been developed in recent years specifically for HCM, addressing myocardial hypercontractility and altered energetics in a direct manner, through allosteric inhibition of myosin. […] Disopyramide was more effective than other inotropic agents in reducing obstruction and alleviating symptoms. […] The strong negative inotropic effect of disopyramide prompted its use for the treatment of symptomatic HCM associated with LVOTO. […] Cibenzoline is a class IA antiarrhythmic drug used for the treatment of oHCM in Japan and Korea but is currently not in clinical practice in the USA or Europe.

• #1 Medication for Hypertrophic Cardiomyopathy | NYU Langone Health

  https://nyulangone.org/conditions/hypertrophic-cardiomyopathy/treatments/medication-for-hypertrophic-cardiomyopathy

  Medication is the first line of treatment for most people with HCM. The right medication regimen can improve symptoms by reducing the obstruction of the left ventricle, and thereby improving its overall performance and its ability to pump blood to the rest of the body. […] Commonly prescribed medications for HCM include beta blockers, disopyramide, calcium channel blockers, heart rhythm medications, and anticoagulants. […] Beta blockers decrease heart rate, reducing the hearts workload. They prevent the worsening of obstruction that occurs with exercise, helping to decrease HCM symptoms. […] Your doctor may prescribe disopyramide, which stops the heart from beating too forcefully. By relaxing the hearts contractions, disopyramide reduces the obstruction in the heart, improves symptoms, and can also regulate heartbeat.

• #1 Hypertrophic Cardiomyopathy (HCM) | American Heart Association

  https://www.heart.org/en/health-topics/cardiomyopathy/what-is-cardiomyopathy-in-adults/hypertrophic-cardiomyopathy

  There is currently only one disease-specific medication to treat hypertrophic cardiomyopathy. Mavacamten is used to treat the obstructive form of HCM in people who have symptoms. […] For people with HCM, a heart healthy lifestyle which includes staying active, eating a healthy diet, maintaining a normal weight, getting good quality sleep and not smoking is recommended. If you have other medical conditions like high blood pressure or diabetes, it’s important that these are managed to avoid heart complications which can develop if left unchecked. For those with symptoms, the focus is on symptom management using medications and procedures. […] Medications that may be prescribed for HCM include: […] Unlike other HCM medications, the cardiac myosin inhibitor mavacamten is used to improve symptoms and function in people with the obstructive type of HCM who have mild to moderate symptoms with activity.

• #1 Pharmacological Management of Hypertrophic Cardiomyopathy: From Bench to Bedside

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9209358/

  Calcium channel blockers (CCBs) are an important resource for clinicians, representing drugs of choice for angina pectoris, supraventricular arrhythmias, and hypertension. […] The presence of interstitial fibrosis is an important feature of HCM pathology. […] Recent data show that sacubitril/valsartan may increase exercise tolerance in heart failure with preserved ejection fraction (HFpEF) following amelioration of diastolic function, suggesting a potential use in symptomatic patients with HCM, especially in non-obstructive patients. […] Mavacamten is a novel specific myosin inhibitor that has recently been identified through a chemical screening for molecules decreasing the ATPase rate of myosin in bovine myofibrils. […] Mavacamten treatment significantly reduced the maximal tension produced by skinned cardiac muscle fibers isolated from adult rats in a dose-dependent manner.

• #1 Hypertrophic Cardiomyopathy Treatment & Management: Approach Considerations, Left Ventricular Myomectomy and Mitral Valve Replacement, Pacemaker Implantation

  https://emedicine.medscape.com/article/152913-treatment

  Evaluation usually can be conducted on an outpatient basis. Inpatient studies and surgical treatment also may be necessary. Medical and surgical therapy are used to reduce ventricular contractility or increase ventricular volume, increase ventricular compliance and outflow tract dimensions, and, in the case of obstructive hypertrophic cardiomyopathy (HCM), reduce the pressure gradient across the LV outflow tract. Paramount to any therapy is reduction in the risk of sudden death by identification of these patients early on and effective medical and/or surgical implantation of an automatic defibrillator. […] Medications include beta blockers, calcium channel blockers, and, rarely, diltiazem, amiodarone, and disopyramide. […] Mavacamten, a first-in-class allosteric inhibitor of cardiac myosin, gained approval from the FDA for adults with symptomatic New York Heart Association class II-III obstructive hypertrophic cardiomyopathy (HCM) to improve exercise capacity and symptoms.

• #1 New Treatment in Pipeline for Patients with Hypertrophic Cardiomyopathy | Lahey Hospital & Medical Center

  https://www.lahey.org/news-stories/all-news-stories/news/2024/05/new-treatment-in-pipeline-for-patients-with-hypertrophic-cardiomyopathy

  Now, a phase 3 trial has shown that a cardiac myosin inhibitor called aficamten enhances exercise capacity in patients with HCM and leads to significant relief in the burden of limiting symptoms, improving the quality of patients’ lives. […] The trial demonstrated that aficamten can reliably and safely lower obstruction to blood flow out of the heart in patients with obstructive HCM using a simple and stepwise dosing regimen and was associated with substantial improvements in clinically relevant endpoints such as exercise capacity and symptoms, said Maron. […] Over the 24-week treatment period, aficamten improved exercise capacity and was also associated with significant improvements in all secondary end points. Efficacy was evident by week 12, with significant improvements in outflow gradients, health status, and symptoms. […] It was impressive to see that the beneficial effects of aficamten occurred rapidly and consistently over the treatment period and that the doses could be adjusted effectively and safely using only site read echocardiography measures, said Maron.

• #1 New hypertrophic cardiomyopathy clinical guideline recommends novel drug therapy and regular exercise – Mayo Clinic

  https://www.mayoclinic.org/medical-professionals/cardiovascular-diseases/news/new-hypertrophic-cardiomyopathy-clinical-guideline-recommends-novel-drug-therapy-and-regular-exercise/mac-20569318

  New hypertrophic cardiomyopathy clinical guideline recommends novel drug therapy and regular exercise. […] Key changes in the 2024 HCM clinical guideline: A new drug therapy and less restricted exercise. The guideline encourages collaborative decision-making with patients and recommends seeking HCM specialists at an experienced multidisciplinary center. […] Cardiac myosin inhibitors are now recommended as therapy for patients with obstructive HCM who have symptoms due to left ventricular outflow tract obstruction despite the use of beta blockers (first-line therapy) or nondihydropyridine calcium channel blockers (used if beta blockers are ineffective). […] „The addition of mavacamten is an option alongside surgical myectomy, percutaneous septal ablation, or disopyramide, for patients with symptomatic obstructive HCM who have not responded to first-line medications.”

• #1 Patient education: Hypertrophic cardiomyopathy (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/hypertrophic-cardiomyopathy-beyond-the-basics

  Avoidance of dehydration — People with HCM should try to maintain a steady fluid balance and avoid situations in which fluid is lost from the body and not adequately replaced (eg, having diarrhea and not drinking adequate fluids). […] Activity restriction — Most people with HCM can participate in mild to moderate physical activity, but are advised to avoid engaging in most competitive sports, with the possible exception of some low-intensity sports (eg, golf, billiards). Certain recreational sports are also not advised. These recommendations generally apply to all people with HCM. Anyone with HCM should talk to their health care provider about the safety of exercise. […] Medications […] Shortness of breath and chest pain — One or more medications may be used to treat limiting symptoms. Options include a beta blocker, verapamil, disopyramide, and the myosin inhibitor mavacamten. These medications work to decrease the heart’s need for oxygen, improve the heart’s ability to fill with blood, improve blood flow to the heart, and decrease the obstruction of blood flow out of the heart. In general, the first-line medical therapy is either a beta blocker or verapamil and second line is disopyramide or a myosin inhibitor. All of these drugs have certain strengths and limitations, which should be thoroughly discussed with your HCM provider to best determine which drug options may be best.

• #1 Hypertrophic cardiomyopathy – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hypertrophic-cardiomyopathy/diagnosis-treatment/drc-20350204

  The goals of hypertrophic cardiomyopathy treatment are to ease symptoms and prevent sudden cardiac death in people at high risk. Treatment depends on how severe the symptoms are. […] Medicines can help reduce how strongly the heart muscle squeezes and slow the heart rate. That way, the heart can pump blood better. Medicines to treat hypertrophic cardiomyopathy and its symptoms might include: […] A septal myectomy is an open-heart surgery. A surgeon removes part of the thickened, overgrown septum between the lower heart chambers called the ventricles, as shown in the heart on the right. […] An apical myectomy is an open-heart surgery to treat hypertrophic cardiomyopathy. A surgeon removes thickened heart muscle from near the tip of the heart. […] Several surgeries or procedures are available to treat cardiomyopathy or its symptoms. They include:

• #1 Surgical treatment for hypertrophic cardiomyopathy: a historical perspective – Hang- Annals of Cardiothoracic Surgery

  https://www.annalscts.com/article/view/14525/html

  Surgical treatment of HCM has become the gold standard of therapy with mortality 1% and symptom relief in over 90% of patients. […] In most cases, the mitral valve regurgitation observed in HCM is solely due to SAM of the leaflets and apparatus. […] A safe and effective approach to intraoperative management of associated MR is to first perform adequate extended septal myectomy, discontinue cardiopulmonary bypass, and then re-evaluate the mitral valve apparatus with intraoperative transesophageal echocardiography to assess the need, if any exists, for an additional concomitant mitral valve procedure. […] In patients with both subaortic and midventricular obstruction, or complex long-segment septal hypertrophy, residual midventricular obstruction may persist postoperatively if inadequate resection was done through a transaortic approach alone.

• #1 Hypertrophic Cardiomyopathy (HCM) | American Heart Association

  https://www.heart.org/en/health-topics/cardiomyopathy/what-is-cardiomyopathy-in-adults/hypertrophic-cardiomyopathy

  A range of surgical and nonsurgical procedures can be used to treat HCM: […] Septal myectomy, also called septal reduction therapy, is open-heart surgery. It’s considered for people with obstructive HCM who, despite taking HCM medications, continue to have severe symptoms. […] Alcohol septal ablation (nonsurgical procedure) – Also called nonsurgical septal reduction therapy, alcohol septal ablation is a procedure where ethanol (a type of alcohol) is injected through a tube into the small artery that supplies blood to the area of heart muscle thickened by HCM. […] Depending on specific patient risk factors, there are devices that can be implanted in the body to help the heart work better, including: […] In HCM patients with advanced, end-stage disease, a heart transplant may be considered.

• #1 Hypertrophic Cardiomyopathy: Diagnosis & Treatment | NewYork-Presbyterian

  https://www.nyp.org/heart/hypertrophic-cardiomyopathy/treatment

  Medications to treat hypertrophic cardiomyopathy should be taken as prescribed. These medications are designed to improve symptoms and heart function by increasing blood flow and the hearts ability to pump blood. Medications are used to lower blood pressure, slow the heart rate, remove excess fluid retained in the body, and prevent blood clots. Medications include: Beta blockers, Calcium channel blockers, Diuretics, Myosin inhibitors. […] Hypertrophic cardiomyopathy can be treated with non-surgical procedures. These include: Alcohol septal ablation. This procedure involves ethanol alcohol being injected through a small tube into the artery that supplies blood to the thickened heart muscle damaged by hypertrophic cardiomyopathy. The alcohol destroys these cells, allowing the heart muscle to shrink to normal size, thus allowing blood to flow through the area. Heart surgery complications and risks increase with age; therefore, alcohol septal ablation is the preferred procedure for older patients.

• #1 HCM – Treatment Options – Atlantic Health System

  https://ahs.atlantichealth.org/conditions-treatments/heart-care/treatment-services/hypertrophic-cardiomyopathy/treatment-options.html

  To prevent sudden cardiac death in hypertrophic cardiomyopathy, an implantable defibrillator may be necessary. Defibrillators have eliminated the vast majority of sudden death events in HCM. […] Open heart surgery – may be recommended if medications do not improve your symptoms. It involves removing part of the thickened, overgrown wall (septum) between the heart chambers. Septal myectomy helps improve blood flow out of the heart and reduces backward flow of blood through the mitral valve (mitral regurgitation). […] Septal ablation – this procedure reduces the thickened heart muscle with injection of alcohol. The alcohol is injected through a long, thin tube (catheter) into the artery supplying blood to the area of thickness. Possible complications include disruption of the heart’s electrical system (heart block), which requires implantation of a pacemaker.

• #1 Hypertrophic Cardiomyopathy Treatment & Management: Approach Considerations, Left Ventricular Myomectomy and Mitral Valve Replacement, Pacemaker Implantation

  https://emedicine.medscape.com/article/152913-treatment

  Approval of mavacamten was based on results from the multicenter, phase 3 EXPLORER-HCM trial (n = 251). […] Avoid inotropic drugs if possible; also avoid nitrates and sympathomimetic amines, except in those patients with concomitant coronary artery disease. […] Pacemaker implantation has been a proposed treatment for patients with hypertrophic cardiomyopathy (HCM). […] Transvenous catheter ablation of the septal region has been performed using selective arterial ethanol infusion to destroy myocardial tissue. […] Alcohol septal ablation offers some advantages over surgical myectomy in that (1) it does not require surgical incision and/or general anesthesia, (2) the recovery time is shorter, and (3) its results lead to less discomfort and greater patient satisfaction than are reported with surgical myectomy.

• #1 Hypertrophic Cardiomyopathy Treatment & Management: Approach Considerations, Left Ventricular Myomectomy and Mitral Valve Replacement, Pacemaker Implantation

  https://emedicine.medscape.com/article/152913-treatment

  Sudden cardiac death occurs in approximately 1% of patients with hypertrophic cardiomyopathy (HCM) each year, and pharmacotherapy has not shown protection against sudden cardiac death. […] Heart transplantation is recommended in specific situations for patients with hypertrophic cardiomyopathy (HCM). […] Atrial fibrillation (AF) is common in hypertrophic cardiomyopathy (HCM). […] In general, women with HCM can safely undergo pregnancy and labor with minimal risks. […] Left ventricular (LV) myomectomy is used for patients with severe symptoms refractory to therapy and an outflow gradient of more than 50 mmHg, either with provocation or with rest. […] Mitral valve replacement is reserved for patients with severe mitral regurgitation due to systolic anterior movement of the mitral valve, particularly when mitral regurgitation (large regurgitant fraction) is associated with the development of congestive heart failure or severe pulmonary hypertension.

• #1 Hypertrophic Cardiomyopathy Program | Valley Health System

  https://www.valleyhealth.com/services/hypertrophic-cardiomyopathy-program

  If you are diagnosed with hypertrophic cardiomyopathy (HCM), you want the best care possible. […] The Valley Hospitals Hypertrophic Cardiomyopathy Program proudly offers: A treatment plan based on the latest guidelines, tailored to meet your needs. […] Every patient in the Hypertrophic Cardiomyopathy Program receives an individualized treatment plan tailored to meet their needs. Medical management, implantable devices, lifestyle changes, or interventions may be recommended to manage and control this condition. […] Hypertrophic cardiomyopathy with heart failure symptoms, both obstructive and non-obstructive, is first managed using medication: Beta-blockers help decrease HCM symptoms by blocking the hormone adrenaline. […] Patients with HCM who, after evaluation, are considered at high risk for sudden cardiac death will be recommended for an implantable defibrillator-cardioverter (ICD).

• #1 Treatment of Hypertrophic Cardiomyopathy: What Every Cardiologist Needs to Know

  https://www.acc.org/Latest-in-Cardiology/Articles/2020/02/19/18/19/Treatment-of-Hypertrophic-Cardiomyopathy

  Septal reduction therapy (SRT) is indicated when medical therapy fails to control NYHA Class III symptoms or following LVOTO-associated syncope or near syncope refractory to medical therapy. There are two forms of SRT: surgical myomectomy and alcohol septal ablation. Surgical myectomy provides definitive therapy for symptomatic LVOTO and is associated with low post-operative mortality and morbidity when carried out by experienced operators in expert centers, although no significant long-term mortality benefit has been demonstrated. […] Patients with HCM are at increased risk for SCD, and risk stratification for implantable cardioverter defibrillator (ICD) implantation is critical in this population. Primary prevention with an ICD is reasonable for patients with severe hypertrophy (30mm), family history of sudden death in a first degree relative, recent unexplained syncope, or „burnt-out” HCM (LVEF 50%). Secondary prevention with ICD placement in those surviving SCD or with sustained ventricular tachycardia (VT) is universally recommended.

• #1 Hypertrophic cardiomyopathy (HCM) – BHF

  https://www.bhf.org.uk/informationsupport/conditions/hypertrophic-cardiomyopathy

  Theres currently no cure for HCM, but treatments are available to help control your symptoms and prevent other health issues. Your treatment will depend on how your heart is affected and what symptoms you have. […] You may need: […] Medicines like diuretics, beta blockers and cardiac myosin inhibitors. Medicines such as these can reduce the amount of work your heart does, control your blood pressure, manage abnormal heart rhythms and other symptoms. […] An ICD or CRT if you’re at risk of having a life threatening abnormal heart rhythm. […] An LVAD to help your heart pump blood. […] A pacemaker to control your heart rate. […] An ablation (catheter ablation) to treat some kinds of arrhythmias. […] A septal myectomy (a type of heart surgery) to remove part of your thickened muscular wall.

• #1 Hypertrophic Cardiomyopathy: a Review | USC Journal

  https://www.uscjournal.com/articles/hypertrophic-cardiomyopathy-form-heart-failure-preserved-ejection-fraction-diagnosis-drugs?language_content_entity=en

  The 2024 AHA/ACC HCM guidelines recommend the use of CMI, disopyramide and septal reduction therapy (SRT) in patients with persistent symptoms despite first-line therapy. […] CMIs are a novel class of drugs that act by blocking myosin ATPase, which reduces the availability of myosin heads for engagement in cross-bridge formation with actin filaments. […] Mavacamten is the first-in-class CMI approved for treatment of obstructive HCM. […] The management of symptomatic non-obstructive HCM with preserved EF is challenging. -blockers and non-dihydropyridine calcium channel blockers are recommended for patients with exertional angina or dyspnea. […] Advanced therapies, including cardiac transplantation, should be considered for individuals with HCM who develop severe, persistent heart failure symptoms due to severe restrictive LV dysfunction or severe LV systolic dysfunction.

• #1 New hypertrophic cardiomyopathy clinical guideline recommends novel drug therapy and regular exercise – Mayo Clinic

  https://www.mayoclinic.org/medical-professionals/cardiovascular-diseases/news/new-hypertrophic-cardiomyopathy-clinical-guideline-recommends-novel-drug-therapy-and-regular-exercise/mac-20569318

  „This class of medications can be a big benefit for patients with symptomatic obstructive HCM, but there are limitations.” […] „It’s great to have a new tool in the tool belt for managing HCM. Myosin inhibition therapy will help patients, but it’s not right for everyone.” […] „While low-moderate intensity exercise was introduced in the 2020 guideline, with the 2024 guideline, more vigorous exercise is reasonable for many patients with HCM.” […] „Disqualification from competitive sports is no longer an automatic in patients with HCM but may be considered for individuals after comprehensive evaluation and discussion about the potential risks of extreme training.” […] „Myosin inhibitor therapy is a novel treatment.”

• #1 The Diagnosis and Treatment of Hypertrophic Cardiomyopathy

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3078548/

  It is recommended to adapt physical activity characterized by endurance in the aerobic range (abstaining from high-performance sports, athletics or competitive sports); a general prohibition on sport does not appear to be justified. […] Due to the risk of sudden cardiac death, identification of high-risk HCM patients is a particular challenge with regard to the need to implant an ICD (implantable cardioverter defibrillator). […] An individual decision must be made for patients with one first-degree risk marker. […] Septal myectomy using the Morrow procedure has been defined as the therapy standard from many years for patients with HOCM who cannot be adequately treated using pharmacotherapy. […] The range of therapeutic options for symptomatic HOCM patients was expanded with the introduction of percutaneous septal ablation (known as PTSMA, TASH, ASA or ESA). […] After myectomy or septal ablation we recommend continuing pharmacotherapy (beta blockers, calcium antagonists) to prevent progression of the underlying disease.

• #1 Hypertrophic Cardiomyopathy Program | Valley Health System

  https://www.valleyhealth.com/services/hypertrophic-cardiomyopathy-program

  If you have HCM, your physician may recommend lifestyle modifications such as diet and exercise, monitoring your blood pressure, managing stress levels, quitting smoking, or limiting your alcohol intake. […] If you have obstructive HCM, and medication proved to be ineffective, you may be recommended for a surgical intervention. […] If heart function deteriorates and medication, devices, and/or surgery fail to improve the function or the symptoms, a heart transplant may be required.

• #1 Diagnosing and Treating HCM | OHSU

  https://www.ohsu.edu/knight-cardiovascular-institute/hypertrophic-cardiomyopathy-treatment-options

  We recommend genetic counseling if you or a family member are diagnosed with hypertrophic cardiomyopathy. A simple blood or saliva test can determine the risk of developing HCM. For anyone at risk, we recommend monitoring the hearteven if there are no symptoms. […] Early detection and diagnosis can prevent, delay or lessen some HCM complications.

• #1 Hypertrophic Cardiomyopathy | Stanford Health Care

  https://stanfordhealthcare.org/medical-conditions/blood-heart-circulation/hypertrophic-cardiomyopathy.html

  With the right care, most people can have a full life and avoid the life-threatening complications that can arise when hypertrophic cardiomyopathy is not properly identified and managed. […] Our team of cardiologists, electrophysiologists, genetic counselors, advanced practice providers, nurses, and other specialists work together to thoroughly evaluate your heart and your medical and family histories. […] We tailor your treatment recommendations to your hearts specific needs. Some people may need only lifestyle adjustments and annual clinic visits with heart testing. Other people may require medications or additional interventions to manage their condition. […] Our genetic counselors have special training in the genetics of hypertrophic cardiomyopathy, as well as the best ways to help you assess your familys risk. Test results can guide treatment, identify affected family members, and help with family planning.

• #1 Hypertrophic Cardiomyopathy | Tufts Medicine

  https://www.tuftsmedicine.org/services-treatments/cardiovascular-care/hypertrophic-cardiomyopathy

  Hypertrophic cardiomyopathy is a heart condition where the muscle walls of the heart become thicker than normal. This can make it harder for the heart to relax and pump blood and may cause symptoms like chest pain, shortness of breath or irregular heartbeats. […] At Tufts Medicine, our expert team treats all types of HCM with compassion and expertise. HCM is manageable with the right care. We work with you to create a personalized care plan that fits your needs and lifestyle. […] With the right care, HCM is manageable. At Tufts Medicine, our expert team provides personalized treatment to help you live your best life. […] HCM is treatable, and most people won’t need surgery or major procedures. If you ever need advanced care, we’ll be here to answer your questions and support you every step of the way. Whether it’s medication, minimally invasive procedures or more advanced treatments, we’ll work closely with you to find the best plan for your heart health. Our goal is to help you live a full and active life, with the right care and support tailored to your needs.

• #1 Hypertrophic Cardiomyopathy: Diagnosis and Therapeutic Options

  https://www.ajmc.com/view/hypertrophic-cardiomyopathy-diagnosis-and-therapeutic-options

  Presently, there are no disease-modifying nor preventive therapies for HCM. Treatments, such as valsartan, gene editing (CRISPR/Cas9), gene replacement therapy, and allele-specific silencing remain under investigation. […] Mavacamten is a first-in-class, small molecule selective inhibitor of cardiac myosin ATPase that reduces actin-myosin cross-bridge formation. […] Mavacamten was well tolerated, with a comparable safety profile to placebo. […] In summary, while HCM is the most common monogenetic cardiovascular disorder, it remains widely underdiagnosed, with a large number of pathologic variants. It has a highly variable clinical presentation, with some patients being asymptomatic and others having significant limitation of functional status. Diagnosis requires a thorough evaluation, including risk stratification for SCD. Treatment depends on symptom status, the presence of LVOTO, and coexistence of other cardiac conditions including AF, ventricular arrhythmias, and HF. While there are no approved disease-modifying therapies for HCM, it remains an area of ongoing investigation.

• #1 Hypertrophic Cardiomyopathy (HCM) Center | OHSU

  https://www.ohsu.edu/knight-cardiovascular-institute/hypertrophic-cardiomyopathy

  Hypertrophic cardiomyopathy (HCM) is the most common genetic heart condition. It occurs when a muscle in your heart thickens and makes it harder for your heart to pump blood. […] New medicines, like cardiac myosin inhibitors, are improving the way we treat HCM. […] Our doctors work with HCM specialists around the world to develop new treatments for hypertrophic cardiomyopathy. […] A new type of medicine called cardiac myosin inhibitors treats the root cause of HCM. These medicines help your heart beat less forcefully. That can reduce your symptoms and make it easier for you to exercise. […] OHSU was part of a study of aficamten, one type of cardiac myosin inhibitor. Aficamten helped people in the study use much more oxygen while exercising. This medicine could make walking and other everyday tasks easier for HCM patients. Aficamten is currently under review for approval by the U.S. Food and Drug Administration.

• #1 New perspectives in the pharmacological treatment of hypertrophic cardiomyopathy | Revista Portuguesa de Cardiologia (English edition)

  https://www.revportcardiol.org/en-new-perspectives-in-pharmacological-treatment-articulo-S2174204920300878

  Ranolazine was thus thought to have the potential to modify the disease’s natural history or to provide symptomatic relief. […] Perhexiline may therefore be useful for treating HCM. […] Despite these promising initial results, a recent clinical trial in 35 patients with obstructive HCM was discontinued prematurely as perhexiline showed no demonstrable efficacy in symptom improvement and oxygen consumption in HCM patients. […] Overall, despite their theoretical beneficial effect on the pathophysiology of HCM, metabolic modulators have not demonstrated efficacy in improving symptoms for patients with the disease. […] Gene therapy has the potential to help prevent disease development in HCM. […] These approaches are targeted at key events early in the development of HCM. Unlike the drugs currently recommended for the treatment of HCM, which seek to alleviate symptoms or prevent complications, these new strategies may mitigate or delay the development of the disease phenotype or even prevent disease onset.

• #1

  https://www.healio.com/news/cardiology/20231013/one-giant-leap-for-hcm-gene-therapy-may-reverse-hypertrophic-cardiomyopathy

  A first-in-human phase 1 trial is testing a novel gene therapy for hypertrophic cardiomyopathy. The one-time infusion, if successful, could be a cure for the debilitating disease. […] There were no disease-specific treatments for HCM until 2022, when the FDA approved mavacamten (Camzyos, Bristol Myers Squibb). Mavacamten has been shown to improve symptom burden and quality of life, but it is not a cure and more options are needed. […] One such new option could be gene therapy. […] This specific type of gene therapy is designed to deliver a working MYBPC3 gene copy to the heart muscle by using an adenovirus vector with a single IV infusion. It is cardiac-specific. It goes into the heart muscle; DNA turns into RNA and RNA turns into the missing protein. For lack of a better phrase, it tops off the missing amount of protein. If this works, then this one-time injection can potentially cure the disease. […] If this gene therapy is successful, what could it mean for the field? […] More importantly if this concept works, then, fast forward a decade or more. A patient with HCM can receive a single IV infusion and be monitored for a few weeks and we would potentially see positive results.

• #1 Treatment

  https://www.cardiosmart.org/topics/hypertrophic-cardiomyopathy/treatment

  Knowing that you have hypertrophic cardiomyopathy (HCM) is important so that your treatment can start as quickly as possible. […] Your treatment plan will depend on several factors including: […] Treatment may include a combination of medications, procedures, lifestyle changes, and ongoing follow-up visits and tests. […] The overall goal of treatment will be to: […] Treating other conditions that can affect your heart is also important high blood pressure, high cholesterol, diabetes, smoking, sleep apnea, carrying too much weight. These all can make HCM worse. […] Shared decision-making can help you work with your care team to find the right option for you. […] Together you will personalize your care decisions.

• #1 Hypertrophic Cardiomyopathy Causes, Symptoms, Treatments

  https://www.upmc.com/services/heart-vascular/conditions/hypertrophic-cardiomyopathy

  UPMC provides personalized care for HCM and is recognized as a Center of Excellence by the Hypertrophic Cardiomyopathy Association. […] Your care team at UPMC will design an HCM treatment plan that’s right for you. […] Goals of HCM care are to: Educate you and your families about how to live with HCM. Improve your symptoms. Reduce potential complications. […] Providers treat inherited HCM in several ways: […] When you have HCM, you should: Avoid strenuous exercise. Eat healthy foods low in saturated fats. Get routine mild to moderate exercise. […] Providers use drugs that help control blood flow and regulate heartbeats to manage HCM symptoms. These include: Antiarrhythmics. Beta-blockers. Calcium channel blockers. Water pills. […] Providers sometimes suggest surgery when HCM causes decreased blood flow. These procedures include: Alcohol septal ablation. Using a catheter, or thin tube, providers inject alcohol into a small artery that carries blood to the septum. Over time, the alcohol reduces the size of the muscle to allow blood to flow more freely. Septal Myectomy. Myectomy is an open-heart surgery in which the surgeon trims the thickened muscle to allow blood to flow freely.

• #2 The Diagnosis and Treatment of Hypertrophic Cardiomyopathy

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3078548/

  Hypertrophic cardiomyopathy (HCM) is the most common hereditary disease of the heart. […] In this article, we summarize the current state of the diagnosis and treatment of HCM on the basis of a selective review of recent publications with relevance to clinical practice. […] The pharmacotherapy of symptomatic HNCM consists of the treatment of heart failure with a normal ejection fraction (HFNEF). In HOCM, the patients symptoms and the obstructive gradient are the guide to treatment with beta-blockers or verapamil. For patients with drug-resistant disease, surgical myectomy and percutaneous septal ablation are now standard treatments. […] A near-normal life expectancy and a highly satisfactory quality of life are now realistic treatment goals for patients with HCM. […] Therapeutic recommendations are mostly based on observational studies or case series.

• #2 Hypertrophic Cardiomyopathy: Causes, Symptoms & Treatments

  https://my.clevelandclinic.org/health/diseases/17116-hypertrophic-cardiomyopathy

  Hypertrophic cardiomyopathy (HCM) is a disease that causes your heart muscle to enlarge (hypertrophy). Most people who have it can have a normal life, but it can be serious for some people. If this is the case for you, there are several treatment options. […] The goal of your treatment is to minimize or prevent symptoms and reduce the risk of complications, such as heart failure and sudden cardiac death. […] Treatment can include: Risk identification and regular follow-up. Lifestyle changes. Medications. Procedures. […] Healthcare providers often prescribe medications to treat your symptoms and prevent further complications. Medications such as beta blockers and calcium channel blockers relax the heart muscle, allowing it to fill better and pump more effectively. Other medications can help control your heart rate or decrease the occurrence of arrhythmias.

• #2 Hypertrophic Cardiomyopathy: Diagnosis and Therapeutic Options

  https://www.ajmc.com/view/hypertrophic-cardiomyopathy-diagnosis-and-therapeutic-options

  Non-vasodilating -blockers (eg, atenolol, propranolol) are usually utilized first line for symptom relief and should be titrated as tolerated (typically to a resting heart rate of 60 bpm). […] While combination therapy with -blockers and CCBs for HCM is generally not recommended, this approach may be considered when used to manage concomitant hypertension. […] If patients with HCM fail to respond to -blockers and non-dihydropyridine CCBs, advanced therapies such as disopyramide (a class 1a antiarrhythmic) and septal reduction therapy (SRT) may be considered. […] SRT is usually reserved for patients who fail to achieve symptom relief from guideline-directed medical therapy (GDMT) and continue to experience impaired QOL. […] Preference is given to surgical myectomy in patients with symptomatic obstructive HCM who have associated cardiac disease that requires surgical treatment.

• #2 Medication for Hypertrophic Cardiomyopathy | NYU Langone Health

  https://nyulangone.org/conditions/hypertrophic-cardiomyopathy/treatments/medication-for-hypertrophic-cardiomyopathy

  Medication is the first line of treatment for most people with HCM. The right medication regimen can improve symptoms by reducing the obstruction of the left ventricle, and thereby improving its overall performance and its ability to pump blood to the rest of the body. […] Commonly prescribed medications for HCM include beta blockers, disopyramide, calcium channel blockers, heart rhythm medications, and anticoagulants. […] Beta blockers decrease heart rate, reducing the hearts workload. They prevent the worsening of obstruction that occurs with exercise, helping to decrease HCM symptoms. […] Your doctor may prescribe disopyramide, which stops the heart from beating too forcefully. By relaxing the hearts contractions, disopyramide reduces the obstruction in the heart, improves symptoms, and can also regulate heartbeat.

• #2 New perspectives in the pharmacological treatment of hypertrophic cardiomyopathy | Revista Portuguesa de Cardiologia (English edition)

  https://www.revportcardiol.org/en-new-perspectives-in-pharmacological-treatment-articulo-S2174204920300878

  In obstructive HCM or non-obstructive HCM with ejection fraction 50%, beta-blockers, calcium channel blockers, disopyramide (obstructive HCM) and diuretics are the therapy of choice, while patients with non-obstructive HCM and reduced ejection fraction should be treated with beta-blockers and angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARBs), and possibly diuretics and mineralocorticoid receptor antagonists (MRAs). […] Although HCM was first described more than 50 years ago, evidence-based therapies are scarce and there have been few clinical trials, with small numbers of patients, evaluating the efficacy of pharmacological treatments for this disease. […] Diltiazem is a non-dihydropyridine L-type calcium channel blocker indicated as a second-line therapy for symptomatic relief in HCM, since it is less effective than beta-blockers.

• #2 Patient education: Hypertrophic cardiomyopathy (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/hypertrophic-cardiomyopathy-beyond-the-basics

  Avoidance of dehydration — People with HCM should try to maintain a steady fluid balance and avoid situations in which fluid is lost from the body and not adequately replaced (eg, having diarrhea and not drinking adequate fluids). […] Activity restriction — Most people with HCM can participate in mild to moderate physical activity, but are advised to avoid engaging in most competitive sports, with the possible exception of some low-intensity sports (eg, golf, billiards). Certain recreational sports are also not advised. These recommendations generally apply to all people with HCM. Anyone with HCM should talk to their health care provider about the safety of exercise. […] Medications […] Shortness of breath and chest pain — One or more medications may be used to treat limiting symptoms. Options include a beta blocker, verapamil, disopyramide, and the myosin inhibitor mavacamten. These medications work to decrease the heart’s need for oxygen, improve the heart’s ability to fill with blood, improve blood flow to the heart, and decrease the obstruction of blood flow out of the heart. In general, the first-line medical therapy is either a beta blocker or verapamil and second line is disopyramide or a myosin inhibitor. All of these drugs have certain strengths and limitations, which should be thoroughly discussed with your HCM provider to best determine which drug options may be best.

• #2 New medication may treat underlying causes of hypertrophic cardiomyopathy | American Heart Association

  https://newsroom.heart.org/news/new-medication-may-treat-underlying-causes-of-hypertrophic-cardiomyopathy

  Mavacamten, a new investigational cardiac medication, may improve heart function for people with thickened heart muscle leading to obstructed blood flow through the heart, a condition known as obstructive hypertrophic cardiomyopathy. […] The treatment appears to improve heart structure, reduce cardiac filling pressures (decrease measures of stiffness) and restore normal mitral valve motion. […] Currently, treatment for obstructive hypertrophic cardiomyopathy focuses on symptom relief, and this medication has been shown in a recently published Phase 3, placebo-controlled study to relieve symptoms; this additional analysis of the data demonstrates that mavacamten also addresses the underlying causes of the condition. […] This study is an additional analysis of data from the EXPLORER-HCM trial. Researchers found that mavacamten also reduces the size of the enlarged left atrium of the heart, decreases elevated filling pressures (reduces measures of stiffness) and restores normal mitral valve motion.

• #2 Hypertrophic Cardiomyopathy Treatment & Management: Approach Considerations, Left Ventricular Myomectomy and Mitral Valve Replacement, Pacemaker Implantation

  https://emedicine.medscape.com/article/152913-treatment

  Approval of mavacamten was based on results from the multicenter, phase 3 EXPLORER-HCM trial (n = 251). […] Avoid inotropic drugs if possible; also avoid nitrates and sympathomimetic amines, except in those patients with concomitant coronary artery disease. […] Pacemaker implantation has been a proposed treatment for patients with hypertrophic cardiomyopathy (HCM). […] Transvenous catheter ablation of the septal region has been performed using selective arterial ethanol infusion to destroy myocardial tissue. […] Alcohol septal ablation offers some advantages over surgical myectomy in that (1) it does not require surgical incision and/or general anesthesia, (2) the recovery time is shorter, and (3) its results lead to less discomfort and greater patient satisfaction than are reported with surgical myectomy.

• #2 Patient education: Hypertrophic cardiomyopathy (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/hypertrophic-cardiomyopathy-beyond-the-basics

  If these treatments do not adequately relieve symptoms or a person prefers not to escalate drug therapy, an invasive procedure to reduce the obstruction of blood out of the heart may be recommended. […] Arrhythmias — People with HCM are at risk for irregular heart rhythms (arrhythmias). Although most arrhythmias do not cause symptoms, some can have serious consequences or even lead to sudden cardiac death. Therefore, testing for arrhythmias is important. […] Procedures to relieve left ventricular outflow tract obstruction — People with HCM who are severely symptomatic and who do not improve with medications, or who choose not to pursue escalating drug therapy, may be advised to consider invasive septal reduction therapies such as alcohol septal ablation or heart surgery such as myectomy.

• #2 Surgical treatment for hypertrophic cardiomyopathy: a historical perspective – Hang- Annals of Cardiothoracic Surgery

  https://www.annalscts.com/article/view/14525/html

  During the latter half of the 20th century, our understanding of hypertrophic cardiomyopathy (HCM) has evolved substantially, perhaps most noticeably illustrated by the many variations in its nomenclature, ranging from functional aortic stenosis, to idiopathic hypertrophic subaortic stenosis, to hypertrophic obstructive cardiomyopathy, to HCM. […] Today, the gold standard for obstructive HCM refractory to medical therapy is surgical septal myectomy. […] Surgical septal myectomy is the gold standard for those patients with HCM who are symptomatic with a resting LVOT gradient of 30 mmHg and refractory to maximal medical therapy. […] A recent study from Mayo Clinic compared outcomes after myectomy between those symptomatic patients with latent LVOT obstruction and those with high resting LVOT gradients.

• #2 Hypertrophic cardiomyopathy and treatment options – BroadcastMed

  https://www.broadcastmed.com/surgery/5214/videos/hypertrophic-cardiomyopathy-and-treatment-options

  Surgical myectomy has been a very successful operation for many of our patients. […] With surgical myectomy, the surgeon removes a portion of the hypertrophied septum, which is narrowing the path of blood, out of the heart. […] An operation is available for some patients that have non-obstructive hypertrophic cardiomyopathy. […] While we see great results from the surgical myectomy as performed now, it is still something that should only be performed at true centers of excellence. […] The mortality for the procedure is less than 1%, especially for patients who are otherwise healthy. […] Septal myectomy cures the symptoms of hypertrophic cardiomyopathy when it relieves the obstruction.

• #2 Patient education: Hypertrophic cardiomyopathy (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/hypertrophic-cardiomyopathy-beyond-the-basics

  Septal myectomy — Septal myectomy is the surgical removal of excess muscle from the thickened ventricular septum, the muscular wall between the left and right ventricles. The surgery is performed by opening the chest, placing the person on cardiopulmonary bypass (a heart lung machine), and removing muscle from the ventricular septum, which will then prevent the mitral valve from making contact with the septum, allowing the blood to flow out of the heart unimpeded. By removing the obstruction, the pressures in the heart will be restored to normal and limiting symptoms due to HCM significantly improved or abolished. […] Nonsurgical septal reduction therapy — Alcohol ablation of the septum, also known as nonsurgical septal reduction therapy (NSRT), or transcoronary ablation of septal hypertrophy (TASH) involves injecting ethanol (alcohol) into the blood vessel supplying the upper part of the thickened septum.

• #2 Hypertrophic Cardiomyopathy: Diagnosis and Therapeutic Options

  https://www.ajmc.com/view/hypertrophic-cardiomyopathy-diagnosis-and-therapeutic-options

  Likewise, preference is given to alcohol septal ablation in patients with symptomatic obstructive HCM and contraindication to surgery, advanced age, or limiting comorbidities. […] Treatment of chest pain and dyspnea in those with nonobstructive HCM most commonly involves -blockers and non-dihydropyridine CCBs. […] An oral anticoagulant is recommended in all patients with HCM and AF, as thromboembolism occurs in up to 30%. […] Restoration of sinus rhythm should be considered in those with AF, particularly in the presence of poorly tolerated symptoms. […] Because ICD shocks are associated with worse outcomes and impaired QOL in HCM, prevention of VT is an important goal. […] Current guideline recommendations endorse use of GDMT in patients with HCM and a left ventricular ejection fraction under 50%.

• #2 Patient education: Hypertrophic cardiomyopathy (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/hypertrophic-cardiomyopathy-beyond-the-basics

  Myectomy versus ethanol ablation — Following either of these procedures, heart muscle does not grow back, which is the reason that these procedures can successfully relieve obstruction to blood flow over the person’s lifetime. Although the outcomes with myectomy and ablation are similar, each procedure has its own advantages and disadvantages. People should discuss the risks and benefits of each procedure with their health care providers. […] Pregnancy — Women with HCM are generally able to become pregnant with normal vaginal birth in the majority of cases without any increased risk of adverse events. Before becoming pregnant, the woman should speak with her health care provider to discuss any potential risks, any medication changes that may be needed, and care during pregnancy. Women with HCM who get pregnant are often followed by a high-risk OB/GYN physician. […] Management of surgical procedures — Because people with HCM have alterations in the heart function, special care may be needed before, during and after surgical procedures to reduce the risk of complications such as heart failure or low blood pressure (hypotension).

• #2 Hypertrophic Cardiomyopathy | UVA Health

  https://uvahealth.com/services/heart/cardiomyopathy-treatment/hypertrophic

  HCM Treatment at UVA Health […] HCM can cause abnormal heart rhythms, which can be fatal. That’s why you want top-rated care. At UVA Health, you’ll find a heart care team with expertise in HCM like no other. […] Hypertrophic Cardiomyopathy Treatment Options […] We offer heart screenings, genetic testing, and a range of treatment options to help you and your family manage your condition. […] If you do have symptoms, medicines can help. That might include: […] Blood pressure medicine […] Anticlotting medicine (blood thinners) […] Heart rhythm medicine […] If medication isnt enough, surgery might be the next step. […] ICD […] An implantable cardioverter defibrillator (ICD) helps control your heart rhythm. This device can decrease your risk for cardiac arrest.

• #2 Cardiomyopathy Treatment

  https://www.dukehealth.org/treatments/heart/cardiomyopathy

  A battery-powered ICD offers protection against unpredictable arrhythmias. The device is surgically implanted under your skin. Thin wires placed within or near your heart muscle continuously monitor your heart rhythm and can pace or shock the heart back into rhythm if a life-threatening arrhythmia develops. This device is recommended for people with certain types of cardiomyopathy and who have certain risk factors, such as extremely thick heart muscle or severely reduced heart strength. […] If cardiomyopathy has severely weakened your heart, leading to heart failure, you may be a candidate for a VAD. This mechanical heart pump can support your heart and optimize your blood flow while you’re waiting for a heart transplant. It can also be a long-term solution if you have heart failure and are not a candidate for a heart transplant.

• #2 Hypertrophic cardiomyopathy – Wikipedia

  https://en.wikipedia.org/wiki/Hypertrophic_cardiomyopathy

  For people with HCM who exhibit one or more of the major risk factors for sudden cardiac death, an implantable cardioverter-defibrillator (ICD) or a combination pacemaker/ICD all-in-one unit may be recommended as an appropriate precaution. […] In cases that are unresponsive to all other forms of treatment, cardiac transplantation is one option. It is also the only treatment available for end-stage heart failure.

• #2 Hypertrophic Cardiomyopathy: Diagnosis & Treatment | NewYork-Presbyterian

  https://www.nyp.org/heart/hypertrophic-cardiomyopathy/treatment

  Septal myectomy. This open-heart surgery is an option for people whose severe obstructive hypertrophic cardiomyopathy cannot be controlled with medication. A heart surgeon will remove the thickened portion of the septum that extends into the left ventricle. The elimination of this damaged portion allows blood to flow freely to the heart and the rest of the body. […] Cardiac implantable electronic devices (CIEDs). These devices are surgically implanted in the body to improve heart function: Implantable cardioverter defibrillator (ICD). This device sends an electric shock to the heart when an irregular heartbeat is detected, reducing the risk of death from sudden cardiac death. Pacemaker. This device is implanted under the skin of the chest or abdomen. It is often used to correct a slow heartbeat. Electrical pulses are used to regulate the heartbeat. Ventricular assist device (VAD). This procedure is considered when less invasive procedures were not successful. The device helps blood through the heart and is used either short or long term for people waiting for a heart transplant. […] Heart transplant. This procedure is used for people with end-stage hypertrophic cardiomyopathy; the damaged heart is replaced with a new, healthy donor heart.

• #2 Diagnosis and Management of Hypertrophic Cardiomyopathy: Updated Guidelines From the ACC/AHA | AAFP

  https://www.aafp.org/pubs/afp/issues/2022/0200/p207.html

  HCM is evaluated and monitored with regular ECG, echocardiography, and prolonged cardiac monitoring. […] Implantable cardiac defibrillators can reduce sudden cardiac death in patients with risk factors. […] Moderate intensity exercise is safe for patients with stable HCM. […] All patients with HCM should be managed in conjunction with a center specializing in HCM; such centers have better outcomes. […] In symptomatic patients, beta blockade is used to reduce the resting heart rate and LVOTO to treat symptoms. […] Nondihydropyridine calcium channel blockers are an alternative for those with significant adverse effects or insufficient control on beta blockers. […] Patients who continue to have symptoms despite beta blockers or calcium channel blockers are candidates for disopyramide (Norpace), an antidysrhythmic agent, or septal reduction therapy.

• #2 Hypertrophic Cardiomyopathy (HCM) Center | OHSU

  https://www.ohsu.edu/knight-cardiovascular-institute/hypertrophic-cardiomyopathy

  Hypertrophic cardiomyopathy (HCM) is the most common genetic heart condition. It occurs when a muscle in your heart thickens and makes it harder for your heart to pump blood. […] New medicines, like cardiac myosin inhibitors, are improving the way we treat HCM. […] Our doctors work with HCM specialists around the world to develop new treatments for hypertrophic cardiomyopathy. […] A new type of medicine called cardiac myosin inhibitors treats the root cause of HCM. These medicines help your heart beat less forcefully. That can reduce your symptoms and make it easier for you to exercise. […] OHSU was part of a study of aficamten, one type of cardiac myosin inhibitor. Aficamten helped people in the study use much more oxygen while exercising. This medicine could make walking and other everyday tasks easier for HCM patients. Aficamten is currently under review for approval by the U.S. Food and Drug Administration.

• #2 Hypertrophic Cardiomyopathy (HCM) Center | OHSU

  https://www.ohsu.edu/knight-cardiovascular-institute/hypertrophic-cardiomyopathy

  Research is an important part of the work we do at OHSU. Clinical trials of new medicines help doctors find new ways to treat disease and improve your health. […] You may qualify to take part in a clinical trial for people with HCM. If you do, your doctor will talk to you about what you can expect.

• #2 How Hypertrophic Cardiomyopathy Is Treated

  https://www.verywellhealth.com/hypertrophic-cardiomyopathy-treatment-5222066

  Septal reduction therapy is a type of invasive therapy that aims to decrease the bulk of the thickened muscle to improve the obstruction of blood flow out of the heart. […] Septal reduction therapy may be done with alcohol septal ablation or septal myectomy. […] Alcohol septal ablation is an invasive therapy performed by cardiologists at specialized centers. […] Septal myectomy is a type of heart surgery performed in specialized centers. […] Because of this, cardiologists may recommend the placement of an implantable cardioverter defibrillator (ICD), a device that monitors the heart rhythm and can deliver an electric shock to stop dangerous arrhythmias. […] First-line therapy for HCM includes beta-blockers or calcium channel blockers. […] In addition, disopyramide may be used when first-line medical treatments fail, and a new medication called mavacamten can be considered for certain cases. […] When medical therapy fails and symptoms are still present and severe, invasive therapy with either alcohol septal ablation or surgery may be recommended.

• #3 Medication for Hypertrophic Cardiomyopathy | NYU Langone Health

  https://nyulangone.org/conditions/hypertrophic-cardiomyopathy/treatments/medication-for-hypertrophic-cardiomyopathy

  Calcium channel blockers work by slowing the absorption of calcium by the heart and blood vessel walls. They slow heart rate and may improve symptoms. […] Antiarrhythmic medications help the heart beat normally by blocking irregular electrical activity and rhythms caused by the thickening of the hearts walls. The most common antiarrhythmics used for people with HCM are amiodarone, sotolol, and disopyramide, which is also used to reduce obstruction. […] Your doctor may prescribe anticoagulants, or blood thinners, to help prevent blood clots that result from atrial fibrillation. […] In most cases, the benefits of preventing stroke outweigh the bleeding risk. […] In these cases, cholesterol-lowering medications such as statins may be prescribed after detailed discussion.

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