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Hypertrophic cardiomyopathy (HCM) is a primary myocardial disease of unknown cause that is characterized by a hypertrophied, nondilated, hypercontractile left ventricle. Its etiology and pathogenesis remain undefined but the three principal factors implicated are a genetic predisposition, a hypersensitivity to catecholamines, and an abnormal calcium metabolism. The hypertrophy typically involves the intraventricular septum to varying degrees, but may also involve the apex or free wall and even be concentric. The disease occurs in either an obstructive or a nonobstructive form depending on whether an intraventricular pressure gradient can be demonstrated at rest or on provocation. The gradient and obstruction to outflow is usually seen in patients with asymmetric septal hypertrophy (ASH) and anterior motion of the mitral valve during systole (SAM). Abnormal left ventricular diastolic function characterized by inadequate filling and impaired relaxation has been shown to be very important in both the obstructive and nonobstructive forms of the disease. In addition, inadequate coronary vasodilator reserve as a result of small vessel disease, microvascular spasm, and/or low capillary density per unit myocardial mass has been implicated as an important cause of ischemia in patients without coronary artery disease. HCM is a disease of young adulthood with relatively slow progression; young patients are often asymptomatic, whereas older patients are more limited by dyspnea, angina, dizziness, or syncope. Supraventricular tachyarrhythmias occur in 30% of patients, and high-grade ventricular arrhythmias occur in over 75%. The annual mortality is 3-5%. The common mode of demise is sudden cardiac death. Therefore, the primary objectives of treatment are the amelioration of symptoms, the control of arrhythmias, and the prevention of sudden death. Beta-adrenoreceptor blocking agents decrease myocardial contractility and oxygen demands and increase ventricular volume; therefore, they are most useful in patients with the obstructive form of HCM. Calcium channel antagonists enhance left ventricular relaxation, relieve microvascular spasm, and improve coronary filling and therefore are the agents of choice in patients with diastolic dysfunction. The ability of the calcium channel antagonists to decrease contractility makes them valuable in patients with obstructive HCM. Arterial vasodilators, diuretics, nitrates, and inotropic agents should be avoided because they can increase the intraventricular gradient. Myomyectomy is reserved for those patients with the obstructive form of HCM whose symptoms are refractory to medical therapy.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Hypertrophic cardiomyopathy: current views on etiology, pathophysiology, and management. 331 Jun 37

A thorough cardiovascular evaluation is necessary to detect potentially lethal abnormalities that may predispose children and adolescents to sports-related sudden death. Hypertrophic cardiomyopathy and anomalous origin of the left coronary artery from the right sinus of Valsalva are the two most common cardiac abnormalities associated with sudden death during childhood. Other conditions potentially associated with sudden death are Marfan's syndrome, aortic valve stenosis, primary pulmonary hypertension, and arrhythmias. A detailed medical evaluation is indicated for subjects who have a history of nonvasodepressor syncope, exercise-related syncope or presyncope, angina pectoris, or known congenital or acquired heart disease. In addition, a family history of premature sudden unexpected syncope or death, hypertrophic cardiomyopathy, premature atherosclerotic heart disease, severe arrhythmias, or Marfan's syndrome or premature aortic aneurysms is an indication for a thorough cardiovascular evaluation before participation in sports.
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PMID:Cardiovascular evaluation of the child and adolescent before participation in sports. 406 61

We undertook a prospective study of the symptoms of hypertrophic cardiomyopathy with the aim of profiling symptomatic morbidity in detail, determining the prevalence of anxiety and depression, and describing the prevalence and associations of syncope and postprandial symptom exacerbation. A questionnaire was administered to consecutive outpatients; 70 with hypertrophic cardiomyopathy, 43 with coronary artery disease, 32 with idiopathic dilated cardiomyopathy, and to 40 normal subjects. Hypertrophic cardiomyopathy patients underwent exercise testing, echocardiography, and Holter monitoring. Hypertrophic cardiomyopathy patients had a high frequency of cardiac symptoms and, on average, had a level of symptomatic morbidity equivalent to that of chronic stable angina and dilated cardiomyopathy. There was no evidence for an excess of anxiety (14%) or depression (6%) in patients with hypertrophic cardiomyopathy. Syncope and presyncope, especially provoked by exertion or posture change, were characteristic and common symptoms in hypertrophic cardiomyopathy. A history of syncope was associated with an abnormal blood pressure response to exercise in over 50% of cases that may be the mechanism of syncope in some. Postprandial exacerbation of symptoms occurred in over one-third of hypertrophic cardiomyopathy patients, half of coronary disease patients, and infrequently in dilated cardiomyopathy. Hypertrophic cardiomyopathy patients with postprandial symptoms had a greater frequency of angina, were more symptomatic, and had a reduced exercise capacity, suggesting that postprandial symptoms are a marker for more severe disease.
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PMID:Symptoms of hypertrophic cardiomyopathy, with special emphasis on syncope and postprandial exacerbation of symptoms. 872 95

Hypertrophic cardiomyopathy is a heterogeneous, progressive disease with a variable age of debut. Hypertrophic cardiomyopathy is characterized by myocardial hypertrophy with a bizarre fibre disarray. Angina pectoris, dyspnoea and syncope are the most frequent symptoms. Hypertrophic cardiomyopathy is an important cause of sudden death, especially in children and young adults. The aetiology is genetic in more than 60% of the cases, with an autosomal dominant mode of inheritance. More than 50 different mutations involving six genes have so far been associated with the development of hypertrophic cardiomyopathy. These mutations are located to genes coding for several of the proteins in the cardiac sarcomere. The protein changes seem to compromise contractility as well as sarcomere assembly, thereby secondarily causing compensatory hypertrophy. The management of hypertrophic cardiomyopathy has been markedly improved within the last few years. This emphasizes the importance of determining prognostic markers in each patient. A specific genetic diagnosis may prove to be of major importance.
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PMID:[Hypertrophic cardiomyopathy]. 976 19

Hypertrophic cardiomyopathy is a primary disease of myocardium resulting in myocardial hypertrophy without any inciting pressure or volume overload. The typical triad of symptoms includes exertional angina, syncope, and shortness of breath. Sudden cardiac death, the most dreadful complication of this disorder, can be the first manifestation of the disease and is more common in young patients. Elderly patients, on the other hand, may have a relatively benign course with normal or near-normal life span. The electrocardiogram (ECG) and echocardiography are the two most useful measures to diagnose hypertrophic cardiomyopathy. The electrocardiographic features of hypertrophic cardiomyopathy are numerous, including ST segment elevation that may simulate other ST segment elevation syndromes, including acute myocardial infarction, variant angina pectoria, acute pericarditis, bundle branch blocks, ventricular paced rhythm, dyskinetic ventricular segment, ventricular aneurysm, left ventricular hypertrophy, Wolff-Parkinson-White syndrome, and early repolarization syndrome. This report describes a case of an asymptomatic patient who presented with ST segment elevation of acute injury type and, therefore, was admitted to rule out silent myocardial infarction. Myocardial infarction was ruled out by cardiac enzyme levels, but ST segment elevation remained persistent in all of the subsequent ECGs. Echocardiography was performed, which clearly showed hypertrophic cardiomyopathy with left ventricular outflow tract obstruction and a high intracavity pressure gradient. Subsequently, retrieval of old ECGs showed a similar type of ST segment elevation in the patient's previous ECGs.
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PMID:Persistent ST segment elevation: a new ECG finding in hypertrophic cardiomyopathy. 1033 93

Hypertrophic cardiomyopathy is a familial cardiac disorder with heterogeneous expression and a diversity of morphological, functional and clinical features. Some individuals with hypertrophic cardiomyopathy may be asymptomatic while others are disabled by symptoms of angina and breathlessness. This article summarizes the genetics, pathophysiology and present management of this important condition.
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PMID:Current management of hypertrophic cardiomyopathy. 1123 21

Hypertrophic cardiomyopathy (HCM) is a relatively frequent, genetically determined primary cardiomyopathy, characterized by most often asymmetric hypertrophy of the ventricular septum with or without systolic obstruction of the left ventricular outflow tract. HCM is a genetically heterogeneous disease, with 12 different disease-causing genes beeing indentified to date. Histologically the disease is characterized by hypertophy and disarray of myofibrils as well as by an increase in myocardial fibrosis. Clinically, these changes may lead to palpitations, dyspnoe on exertion, and/or angina pectoris. However, they also lead to an increased propensity to the development of severe ventricular tachyarrhythmias and sudden cardiac death. The incidence of sudden death is significantly increased in HCM, particularly in affected young subjects. Risk stratification in HCM should include a complete clinical-cardiological evaluation that should also consider new diagnostic features, e. g. MR imaging. Major risk factors for sudden cardiac death include a survived cardiac arrest (ventricular fibrillation), non-sustained and sustained ventricular tachycardia, a history of premature familial sudden death, unexplained syncope, an abnormal blood pressure response on exercise, and left ventricular thickness greater than or equal to 3 cm. Ideally, risk stratification should also include genetic testing, since some gene mutations seem to be associated with a higher risk for sudden cardiac death than others. However, genetic testing in HCM in not yet available on a routine basis. The implantation of a cardioverter/defibrillator is first-line therapy in patients with documented ventricular tachycardia/fibrillation or patients who have survived sudden cardiac death. These devices also play an important role in the primary prevention of sudden cardiac death in HCM. Algorithms and scores are available to estimate the risk of sudden death, however, the decision to implant a cardioverter/defibrillator remains an individual decision in every single patient.
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PMID:[Sudden cardiac death in familial hypertrophic cardiomyopathy. Identification of high-risk patients]. 1585 98

Hypertrophic cardiomyopathy (HCM) is a congenital cardiac disease with an estimated prevalence of 1:500 in the population. Individuals with HCM can present with clinical manifestations that include left ventricular outflow obstruction, cardiac dysrhythmias, diastolic heart failure, cardiac angina, and sudden cardiac death. Current treatments include pharmacologic intervention to reduce heart rate and ventricular contractility as well as surgery or septal alcohol ablation to reduce myocardial septal size. Implantable cardiac defibrillators are considered a treatment option in individuals with HCM who are at an increased risk for sudden cardiac death. The identification of persons at risk for complications related to HCM is important for reducing mortality and morbidity in this population. In addition, diagnosis of HCM in an individual allows the healthcare provider caring for these patients to screen, educate, and institute timely preventative measures in other members of the family. The purpose of this review is to provide clinicians caring for cardiac patients with a guide for recognition, diagnosis, prevention, and treatment of HCM.
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PMID:Hypertrophic cardiomyopathy: a review of etiology and treatment. 1722

Hypertrophic cardiomyopathy (HCM) is a disease characterized by primary hypertrophy of the left (and sometimes right) ventricle. The clinical manifestations of the disease are dyspnea, angina, and a continuum encompassing lightheadedness, presyncope, syncope, and sudden death. Although HCM is often caused by an identifiable mutation in a gene coding for a sarcomeric protein and inherited in an autosomal-dominant pattern, many patients do not have any relatives in whom the disease is manifest. The prevalence of HCM is estimated to be 0.2%, with nearly 600,000 Americans affected. This limited exposure of clinicians to HCM understandably accounts for the uncertainty that prevails regarding this disease and its management.
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PMID:Hypertrophic cardiomyopathy: etiology, diagnosis, and treatment. 1856 7

Hypertrophic cardiomyopathy (HCM) is a common genetic cardiovascular disease that affects the left ventricle. HCM can appear at any age, with the majority of the patients remaining clinically stable. When patients complain of symptoms, these include: dyspnea, dizziness, syncope and angina. HCM can lead to sudden cardiac death (SCD), mainly due to ventricular tachyarrhythmia or ventricular tachycardia. High-risk patients benefit from implantable cardioverter-defibrillators. Left ventricular outflow tract obstruction is not a rare feature in HCM, especially in symptomatic patients, and procedures that abolish that obstruction provide positive and consistent results that can improve long-term survival. HCM is the most common cause of sudden death in young competitive athletes and preparticipation screening programs have to be implemented to avoid these tragic fatalities. The structure of these programs is a matter of large debate. Worldwide registries are necessary to identify the full extent of HCM-related SCD.
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PMID:Hypertrophic cardiomyopathy and sudden cardiac death. 2116 Jun 5


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