Many people have no symptoms and live a normal life with few problems. The decision to place the device in an otherwise healthy individual should be made in the setting of a candid patient-centred discussion of absolute and relative risks of both sudden cardiac death and implantable defibrillator therapy. Hypertrophic cardiomyopathy (HCM) is a condition in which the heart becomes thickened without an obvious cause. Muscles in the heart become abnormally thickened, and the heart has to work harder to push the same amount of blood through the body. Diagnosis can be challenging given phenotypic heterogeneity. A genetic diagnosis can be obtained for patients with hypertrophic cardiomyopathy. Symptoms include dyspnea, chest pain, syncope, and sudden death. If both procedures are available, surgical myectomy is generally recommended for young patients with low surgical-risk profiles, while catheter-based treatment is favoured for elderly patients and those at higher surgical risk (Appendix 4, available at www.cmaj.ca/lookup/suppl/doi:10.1503/cmaj.120138/-/DC1).30, Although early observational reports were promising, the subsequent blinded, randomized crossover trials failed to support the use of dual chamber pacing for treatment of left ventricular outflow tract obstruction. The latter group can be further divided into those who develop outflow tract obstruction and exertional limitations (25% of all affected);26 an additional 25% with provokable outflow tract obstruction;27 those with restrictive physiology and minimal hypertrophy (1%–2%);15 those who have a tendency for ventricular arrhythmias and sudden cardiac death; and the remainder who have hypertrophy without obstruction, but who remain at risk for atrial and ventricular arrhythmias and who may experience exertional limitation because of diastolic dysfunction. But in some people with this condition: The heart doesn't get enough blood and oxygen, which can cause chest pain. Cardiomyopathy can lead to heart failure.The main types of cardiomyopathy include dilated, hypertrophic and restrictive cardiomyopathy. Hypertrophic cardiomyopathy (HCM) is a disease of your heart muscle cells. Note: ACE = angiotensin-converting-enzyme inhibitor, ARB = angiotensin receptor blocker, ICD = implantable cardioverter defibrillator, LVOT = left ventricular outflow tract. Surgical myectomy versus alcohol septal ablation for obstructive hypertrophic cardiomyopathy. Those with a positive genotype, but who do not meet diagnostic criteria, should not be considered to have hypertrophic cardiomyopathy, because clinical issues that are important in phenotypically positive disease (e.g., restriction from competitive sports) are considerably less relevant in this population.16,17 Variable penetrance and expressivity mean that we cannot reliably predict the clinical course for genotype- positive, phenotype-negative individuals based on the clinical histories of members of the same family who have hypertrophic cardiomyopathy. All editorial matter in CMAJ represents the opinions of the authors and not necessarily those of the Canadian Medical Association or its subsidiaries. An electrocardiogram (ECG), echocardiogram and cardiac magnetic resonance imaging (MRI) from a patient with hypertrophic cardiomyopathy are shown in Appendix 1 (available at www.cmaj.ca/lookup/suppl/doi:10.1503/cmaj.120138/-/DC1). Distribution of hypertrophy can be variable. Background — Hypertrophic cardiomyopathy (HCM) is considered a leading cause of sudden cardiac death (SCD) in younger people. This does not occur in all patients. Concerns regarding the potential for creation of an arrhythmogenic focus with septal ablation,66 as well as the increased risk of complete heart block with that procedure, make the appropriate selection of patients for catheter-based treatment complex. In some cases, the condition may develop into dilated cardiomyopathy. For information about this, please see Appendix 5 (available at www.cmaj.ca/lookup/suppl/doi:10.1503/cmaj.120138/-/DC1). Sudden deaths with a prior clinical diagnosis of HCM but no autopsy were considered probable HCM-related SCDs. References . Regular clinical evaluation with specific investigations enables initiation of targeted therapies to reduce morbidity and mortality. Treatment of secondary pulmonary hypertension or placement of a left ventricular assist device is difficult in patients with small ventricular cavities, although small series support its limited use in this setting.93. Hypertrophic cardiomyopathy is a congenital or acquired disorder characterized by marked ventricular hypertrophy with diastolic dysfunction but without increased afterload (eg, due to valvular aortic stenosis, coarctation of the aorta, systemic hypertension). Clinical screening under the age of 12 is not usually pursued unless there is a high-risk family history or the individual will be pursuing intensive competitive sports. When cardiomyopathy develops as a way to compensate for some other underlying disease, like hypertension or valve diseases, it’s called secondary cardiomyopathy; when it develops all by itself, it’s called primary cardiomyopathy. Most SCDs occurred during rest (64.8%) or light activity (18.5%). The same diagnostic advances are poised to contribute meaningfully to risk stratification (e.g., likelihood of sudden cardiac death). Perhaps most important, patient advocacy groups have created an environment in which patients are educated and empowered to work with their physicians as partners in their care.73 Research goals and the availability of progressive specialty programs in inherited cardiomyopathy have both been positively affected by patient advocacy. Operator and institutional experience are important factors, as are patient preference and individual predictors of therapeutic success. Clinical screening of identified affected individuals is recommended in 12- to 18-month intervals. Muscular subaortic stenosis: hemodynamic and clinical improvement after disopyramide, Disopyramide in hypertrophic cardiomyopathy II. Less widely appreciated sequelae of hypertrophic cardiomyopathy include atrial arrhythmia and consequent embolic phenomena, as well as progression to heart failure and, in some cases, requirement for cardiac transplant.3 Increasingly, the use of imaging and mutation analysis have made very early and preclinical genetic diagnosis possible. It is the most common genetic heart disease, as well as the most frequent cause of sudden cardiac death in young people. No prespecified age for release from follow-up has been established, and continuation of follow-up should be based on family history and patient-specific factors (Box 2).13. Hypertrophic cardiomyopathy (HCM) is a genetic disorder that is characterized by left ventricular hypertrophy unexplained by secondary causes and a nondilated left ventricle with preserved or increased ejection fraction. Development of new therapies to address these goals based on knowledge of the genetic basis of hypertrophic cardiomyopathy has been disappointing. Hypertrophic cardiomyopathy (HCM) is a genetic disorder of cardiac myocytes that is characterized by cardiac hypertrophy, unexplained by the loading conditions; a nondilated left ventricle; and a normal or increased ejection fraction. Find nearby businesses, restaurants and hotels. Hypertrophic cardiomyopathy (HCM) is the most common heritable cardiomyopathy, historically believed to affect ∼1 of 500 people (Online Ref. The implantation of a pacemaker, a procedure that has a 30% success rate Hypertrophic cardiomyopathy (say "hy-per-TROH-fik kar-dee-oh-my-AWP-uh-thee") happens when the heart muscle grows too thick, so the heart gets bigger and its chambers get smaller. For example, cardiac MRI may identify noncontiguous regions of hypertrophy that are difficult to appreciate on echocardiography, and may be useful in identifying infiltrative processes, as well as scar tissue.25, Treatment depends on disease expression, which can differ greatly among individuals, even within a single family. Disease penetrance is incomplete and expression is variable, making the familial nature of this disease occasionally challenging to appreciate.4 Although some reports indicate that hypertrophy may develop later in life in a subset of patients (in particular, those with MYBC3 mutations),5 general experience is that late development or progression of hypertrophy is uncommon, with most cases of hypertrophy developing during adolescence and early adulthood. Clinical screening of first-degree relatives and other family members should be encouraged if a DNA diagnosis cannot be established or is not feasible, including: Annual clinical screening is recommended in these individuals from 12 to 18 years of age. Depending on the severity and location of hypertrophy, dynamic obstruction of the left ventricular outflow tract can occur, and it may be quite limiting in some cases. However, pacing may be beneficial for selected patients (i.e., those with end-stage disease or for whom myectomy or alcohol septal ablation cannot be performed).49,77,78, Structural abnormalities of the mitral valve and valve apparatus are not uncommon in patients with hypertrophic cardiomyopathy.79 In the presence of substantial mitral regurgitation, surgery is the preferred approach. Anticoagulation is recommended for all patients with hypertrophic cardiomyopathy and atrial fibrillation.13,81 Because rate-versus-rhythm control strategies have not been studied in this population, existing management strategies for atrial fibrillation based on large prospective studies involving patients with heart failure from more usual causes cannot be applied. However, the skew of prevalence of phenotypes is mild, making the use of this term misguided.7–9 Hypertrophic cardiomyopathy has been most extensively studied in white populations with associated genetic subtypes best appreciated in this population. This thickening typically occurs in the lower left chamber of the heart, called the left ventricle. It is occasionally restricted to other myocardial regions, such as the apex, the midportion, and the posterior wall of the left ventricle… 2019; 140(21):1706-16. While there are reports of arrhythmia following alcohol septal ablation,66 the data do not suggest significantly increased arrhythmia burden when the procedure is correctly performed.76 Although there is longer-term experience with myectomy than with septal ablation, published data on both procedures suffer from incomplete follow-up. HCM is a condition where areas of heart muscle become thickened and stiff. HCM is a disease state characterised by unexplained, marked and asymmetric left ventricular (LV) hypertrophy associated with non dilated ventricular chambers in the absence of another cardiac or systemic disease capable of producing the magnitude of hypertrophy evident in a given patient (2). The identification of risk factors is the starting point for determining the utility of an implantable defibrillator as primary prophylaxis in any patient. The evidence base for the management of out-Tow obstruction is variable (Appendix 3, available at www.cmaj.ca/lookup/suppl/doi:10.1503/cmaj.120138/-/DC1); however, in all cases, treatment should be restricted to patients who exhibit the associated symptoms. https://www.alltrails.com/trail/canada/alberta/crescent-falls Left ventricular hypertrophy in the absence of another cardiac or systemic disease that could cause hypertrophy: Wall thickness of 1.5 cm or greater in adults or the equivalent relative to body surface area in children with a nondilated hyperdynamic left ventricle. Our mission: To reduce the burden of cardiovascular disease. Copyright 2021, Joule Inc. or its licensors. Although the genes associated with hypertrophic cardiomyopathy are well described, the pathways that lead from gene mutation to hypertrophy, restrictive physiology, and atrial and ventricular arrhythmias remain incompletely understood. The main feature of hypertrophic cardiomyopathy is an excessive thickening of the heart muscle (hypertrophy literally means to thicken). Some people who have hypertrophic cardiomyopathy are at high risk for sudden death. Retrospective cohort data support the use of disopyramide to reduce left ventricular outflow tract gradients and symptoms, with good effect and reasonable safety profile in combination with β-blockers in patients with refractory symptoms (Appendix 3). Learn more. Septal alcohol ablation (catheter-based approach) is reserved for patients who are not g… Even though the effectiveness of this approach is unclear, on balance the medical community feels that that this is a reasonable recommendation. They pump blood to your lungs and the rest of your body. Such pronounced findings are not always present, however. Despite its rarity, it receives considerable attention in part because of an associated risk of sudden death, even in apparently healthy individuals who are not known to have heart disease. We also included seminal articles that have made important contributions to our knowledge of hypertrophic cardiomyopathy, even if such articles were not identified in our search. In the subset of people with hypertrophic cardiomyopathy and predominantly restrictive features, atrial arrhythmias may be tolerated poorly and are associated with a significantly increased risk of stroke.53 Management of these arrhythmias and prevention of thromboembolism are achievable therapeutic targets. Prospective randomized controlled trials are largely absent from the literature on hypertrophic cardiomyopathy. Maintenance of sinus rhythm using cardioversion and antiarrhythmic agents, and ablation in select cases, may be indicated. The natural history of hypertrophic cardiomyopathy includes those who remain asymptomatic and those who develop symptoms. Voltage criteria showed T-wave inversion with left ventricular hypertrophy, echocardiography and MRI showed major hypertrophy, and late-gadolinium enhancement showed evidence of scar tissue. While the media often highlight these tragic deaths, sudden death is rare. Weather. Once identified, hypertrophic cardiomyopathy is generally a benign or manageable condition, provided appropriate clinical evaluation is carried out. Disopyramide should be considered for patients with obstructive hypertrophic cardiomyopathy before more invasive interventions.62 It should not, however, be administered with other antiarrhythmic drugs (e.g., sotalol, amiodarone). Download a PDF version. Clues to the possible diagnosis of hypertrophic cardiomyopathy include the presence of left ventricular hypertrophy on electrocardiography or echocardiogram in the absence of abnormal loading conditions. All rights reserved. Intense exertion may bring on ventricular arrhythmias and SCD. The parts of the heart most commonly affected are the interventricular septum and the ventricles. Cases with typical features but no myofiber disarray were considered possible HCM. From the Division of Cardiovascular Medicine (Jacoby), Yale University School of Medicine, New Haven, Conn.; Division of Cardiology (DePasquale), David Geffen School of Medicine, University of California, Los Angeles, Calif.; and the Institute of Cardiovascular Science, University College of London, London, UK. Diagnosis and management of valve disease in patients with hypertrophic cardiomyopathy Aortic valve disease; Mitral valve disease; Endocarditis prophylaxis; Living with cardiomyopathy: advice to patients. Hypertrophic Cardiomyopathy. To estimate the number of HCM-related aborted cardiac arrests and lives potentially saved by implantable cardioverter-defibrillators, all de novo implantations for secondary prevention and all implantations and appropriate shocks for primary prevention in patients with HCM 10 to 45 years of age, respectively, were identified with the use of a registry containing data on implantable cardioverter-defibrillator implantations from all implanting sites throughout Ontario. Reduction in the intensity of follow-up during adulthood is reasonable. When the ventricles are thick or stiff, your heart cannot fill with enough blood. However, when the regurgitant jet is closely related to systolic anterior motion of the mitral valve (posteriorly directed in association with normal valve structure and major left ventricular outflow tract gradient), either method of septal reduction should alleviate the mitral regurgitation.80. Initial treatment is with medication. In obstructive hypertrophic cardiomyopathy, the thickened muscles push into a chamber of the heart, blocking blood flow and sometimes causing the mitral valve of the heart to leak. Risk stratification algorithm for prevention of sudden cardiac death. Full Screen Open Map Latest News. SCDs are infrequently related to exercise. Brackets indicate third-line therapy with, at best, borderline evidence to support their use. Invasive therapies for obstructive symptoms should be chosen based on clinical characteristics, physician and institutional expertise, and patient preferences. There are many options for treating hypertrophic cardiomyopathy. The incidence of HCM-related SCD and its relationship to exercise have not been well studied in large comprehensive studies outside of tertiary care settings. Hypertrophic cardiomyopathy (HCM), also called hypertrophic obstructive cardiomyopathy (HOCM) occurs in one out of 500 people. We performed a PubMed search using the term “hypertrophic cardiomyopathy.” We reviewed all articles published between Jan. 1, 2005, and May 1, 2012, for relevance. We’re committed to keeping clients and staff safe during COVID-19 with NEW admittance and check-out processes. Men and women have the condition at the same frequency. With current testing, pathogenic mutations will be identified in 60%–70% of patients in 1 of 9 genes encoding the components of the cardiac sarcomere.3 Although identification of a pathogenic sarcomere mutation is helpful, an inability to identify a pathogenic sarcomere gene mutation in a patient who meets the clinical criteria for diagnosis does not negate the diagnosis. Explore! The availability of genetic testing with a reasonable signal-to-noise ratio enables the identification of genotype-positive, phenotype-negative individuals. *Risk factors include cardiac arrest,84,86 spontaneous sustained ventricular tachycardia,84,86 family history of premature sudden cardiac death,30 unexplained syncope,91 left ventricular thickness of 3 or more cm,36 abnormal blood pressure response to exercise85 and nonsustained ventricular tachycardia (≥ 3 beats, at least 120 beats/min).87 Possible risk factors include LVOT obstruction (≥ 50 mm Hg at rest),86 contrast cardiac magnetic resonance imaging with extensive delayed enhancement,90 and high-risk mutation. DOI: https://doi.org/10.1161/CIRCULATIONAHA.119.040271. Management of hypertrophic cardiomyopathy. The first step in the management of hypertrophic cardiomyopathy and atrial fibrillation should include attempts at rhythm control. Clinica… The thickened heart muscle can make it harder for the heart to pump blood.Hypertrophic cardiomyopathy often goes undiagnosed because many people with the disease have few, if any, symptoms and can lead normal lives with no significant problems. Clinical screening every 5 years is recommended in individuals older than 18 years. Standard medical heart failure therapy may be used in this population; however, cautious use of afterload reduction and diuretics are necessary in patients with restrictive physiology. Conclusions — The incidence of HCM-related SCD in the general population 10 to 45 years of age is substantially lower than previously reported, with most cases occurring in previously undiagnosed individuals. Evidence in this area comes from highly varied sources, including relatively large populations in retrospective cohort and population studies, as well as family-focused observational analyses (Box 1). Prevention of hypertrophy, regression of hypertrophy and non–device-driven reduction in the risk of sudden cardiac death are the ultimate goals of treatment of this condition. Treatment is dependent on the clinician’s ability to identify and treat the underlying physiology (Figure 1).13,29–68. People with hypertrophic cardiomyopathy are at higher risk for sudden death than people without the condition. The thickening makes it harder for the heart to contract and pump blood out to the body. In this review, we address these needs and identify areas of ongoing controversy. Thank you for your interest in spreading the word on CMAJ. Cardiomyopathy (kahr-dee-o-my-OP-uh-thee) is a disease of the heart muscle that makes it harder for your heart to pump blood to the rest of your body. Patients who cannot tolerate or whose condition is refractory to medical therapy are candidates for surgical or catheter-based treatment of outflow obstruction.74,75 In experienced centres, both procedures are associated with low rates of complications and successful relief of obstruction and associated symptoms.76 There is debate over which procedure is best. Although the presence of such diverse phenotypes in the general population can make the identification of classic hypertrophic cardiomyopathy difficult, genetic testing can help to differentiate this disease from other subtypes of pathologic ventricular hypertrophy. Autosomal dominant disease is predominant, with most sporadic and alternate inheritance patterns (X-linked, mitochondrial) representing phenocopies. Hypertrophic cardiomyopathy has come to public recognition in large part because of sudden cardiac death in a subset of young, otherwise healthy individuals with the condition. As such, the decision about the placement of an implantable defibrillator can be one of the most difficult in the care of a patient with hypertrophic cardiomyopathy (Figure 2).30,36,37,84–91 It should be noted that there are no prospective data on reduction in mortality with the use of implantable cardioverter defibrillators in this population; however, retrospective cohort data using age- and risk factor–matched controls are strongly compelling for their use in high-risk patients.36,37,48,52. Hypertrophic cardiomyopathy should be considered if a patient has unexplained symptoms, a family history of premature cardiac disease, or electrocardiographic abnormalities. Your physician will take your full medical history, evaluate your symptoms and order an echocardiogram to show if your heart muscle is abnormally thick, if your heart valves are … Will there ever be a randomized trial? Hypertrophic cardiomyopathy (HCM) is a genetic disorder characterized by left ventricular hypertrophy (LVH) without an identifiable cause. These studies guide expert opinion and, in many cases, are the result of careful clinical observation of specialty care offered at select high-volume centres. Non-invasive assessment after oral administration, Hypertrophic obstructive cardiomyopathy: comparison of outcomes after myectomy or alcohol ablation adjusted by propensity score, A decade of percutaneous septal ablation in hypertrophic cardiomyopathy, Long-term results of left ventricular myotomy and myectomy for obstructive hypertrophic cardiomyopathy, Use of calcium-channel blocking drugs in hypertrophic cardiomyopathy, Management of symptomatic hypertrophic obstructive cardiomyopathy — long-term results after surgical therapy, Multicenter study of the efficacy and safety of disopyramide in obstructive hypertrophic cardiomyopathy, Non-surgical myocardial reduction for hypertrophic obstructive cardiomyopathy, Outcome of alcohol septal ablation for obstructive hypertrophic cardiomyopathy, Hypertophic subaortic stenosis clinical and hemodynamic effects of long-term propranolol therapy, Long-term outcome of alcohol septal ablation in patients with obstructive hypertrophic cardiomyopathy: a word of caution, Comparison of surgical septal myectomy and alcohol septal ablation with cardiac magnetic resonance imaging in patients with hypertrophic obstructive cardiomyopathy, Langzeitverlauf nach perkutaner septumablation bei hypertropher obstruktiver kardiomyopathie, Prevalence of exercise-induced left ventricular outflow tract obstruction in symptomatic patients with non-obstructive hypertrophic cardiomyopathy, Outcome of mildly symptomatic or asymptomatic obstructive hypertrophic cardiomyopathy, Support for routine use of metabolic stress testing in hypertrophic cardiomyopathy, Prognostic utility of metabolic exercise testing in minimally symptomatic patients with obstructive hypertrophic cardiomyopathy, Hypertrophic obstructive cardiomyopathy-alcohol septal ablation vs. myectomy: a meta-analysis, Outcome of patients with hypertrophic obstructive cardiomyopathy after percutaneous transluminal septal myocardial ablation and septal myectomy surgery, Long-term follow-up after percutaneous septal ablation in hypertrophic obstructive cardiomyopathy, Placebo effect of pacemaker implantation in obstructive hypertrophic cardiomyopathy, Effect of biventricular pacing on symptoms and cardiac remodelling in patients with end-stage hypertrophic cardiomyopathy, Mitral valve abnormalities identified by cardiovascular magnetic resonance represent a primary phenotypic expression of hypertrophic cardiomyopathy, Mitral regurgitation in hypertrophic obstructive cardiomyopathy: relationship to obstruction and relief with myectomy, 2011 ACCF/AHA/HRS focused updates incorporated into the ACC/AHA/ESC 2006 Guidelines for the management of patients with atrial fibrillation, Role of family history of sudden death in risk stratification and prevention of sudden death with implantable defibrillators in hypertrophic cardiomyopathy, Prognosis in hypertrophic cardiomyopathy: role of age and clinical, electrocardiographic and hemodynamic features, Prognostic significance of 24 hour ambulatory electrocardiographic monitoring in patients with hypertrophic cardiomyopathy: a prospective study, Prospective prognostic assessment of blood pressure response during exercise in patients with hypertrophic cardiomyopathy, Survival after cardiac arrest or sustained ventricular tachycardia in patients with hypertrophic cardiomyopathy, Non-sustained ventricular tachycardia in hypertrophic cardiomyopathy, Left ventricular outflow tract obstruction and sudden death risk in patients with hypertrophic cardiomyopathy, Implantable cardioverter-defibrillators and prevention of sudden cardiac death in hypertrophic cardiomyopathy, Occurrence and frequency of arrhythmias in hypertrophic cardiomyopathy in relation to delayed enhancement on cardiovascular magnetic resonance, Syncope and risk of sudden death in hypertrophic cardiomyopathy, Clinical course of hypertrophic cardiomyopathy in a regional United States cohort, Left ventricular assist device therapy in patients with restrictive and hypertrophic cardiomyopathy, Phenotypic diversity in hypertrophic cardiomyopathy, Hypertrophic cardiomyopathy: distribution of disease genes, spectrum of mutations, and implications for a molecular diagnosis strategy, The efficacy and safety of sunscreen use for the prevention of skin cancer, Prone positioning for patients with hypoxic respiratory failure related to COVID-19, Anticipating and managing coagulopathy and thrombotic manifestations of severe COVID-19, www.cmaj.ca/lookup/suppl/doi:10.1503/cmaj.120138/-/DC1. Methods — Using the Office of the Chief Coroner of Ontario database encompassing all deaths attended by the coroner, we identified all HCM-related SCDs in individuals 10 to 45 years of age between 2005 and 2016 (70 million person-years). While a combination of ECG and echocardiography is more readily available and serves as adequate testing for the diagnosis of hypertrophic cardiomyopathy in most cases, cardiac MRI can provide additional information that can be quite useful. Data supporting the use of either a single risk-factor trigger or a multiple risk-factor trigger exist.94,95 Both approaches are subject to risk tolerance, which itself is variable across individuals and cultures. If the decision is made to delay placement of the device, reassessment of risk factors is necessary as disease expression may change over time. Given the frequency of hypertrophic cardiomyopathy and the development of additional diagnostic and prognostic strategies, practitioners require a reasonable evidence-based approach to diagnose, assess and treat this disease. This study sought to estimate the incidence of HCM-related SCD and its association with exercise in a large unselected population. The estimated annual incidence rate for HCM-related SCD plus aborted cardiac arrest and HCM-related life-threatening arrhythmia (SCD, aborted cardiac arrest, and appropriate implantable cardioverter-defibrillator shocks) was 0.84 per 1000 HCM person-years (95% CI, 0.70–1.0). This leads to stiffening of the walls of the heart and abnormal aortic and mitral heart valve function, both of … An introduction to hypertrophic cardiomyopathy (HCM). What areas of management are controversial. 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