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-Blockers should be the initial drug in symptomatic individuals, especially when dynamic outflow obstruction is noted on the echocardiogram The resulting slower heart rates assist with diastolic filling of the stiff LV Dyspnea, angina, and arrhythmias respond in about 50% of patients Calcium channel blockers, especially verapamil, have also been effective in symptomatic patients Their effect is due primarily to improved diastolic function, but their vasodilating actions can also increase outflow obstruction and cause hypotension Disopyramide (Norpace) is also used because of its negative inotropic effects; it is usually used in addition to the other therapies Diuretics are frequently necessary due to the high diastolic pressure and PCWP Patients do best in sinus rhythm, and atrial fibrillation should be aggressively treated with antiarrhythmics Dual-chamber pacing may prevent the progression of hypertrophy and obstruction Nonsurgical septal ablation has been performed by injection of alcohol into septal branches of the left coronary artery with good results in small series of patients Patients with malignant ventricular arrhythmias and unexplained syncope in the
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The most frequent symptoms are dyspnea and chest pain (Table 10 12) Syncope is also common and is typically postexertional, when diastolic filling diminishes and outflow obstruction increases Arrhythmias are an important problem Atrial fibrillation is a long-term consequence of chronically elevated LA pressures and is a poor prognostic sign Ventricular arrhythmias are also common, and sudden death may occur, often in athletes after extraordinary exertion Features on physical examination are a bisferiens carotid pulse, triple apical impulse (due to the prominent atrial filling wave and early and late systolic impulses), and
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presence of a positive family history for sudden death are probably best managed with an implantable defibrillator Excision of part of the outflow myocardial septum (myotomy myomectomy) by surgeons experienced with the procedure has been successful in patients with severe symptoms Some experts advocate mitral valve replacement, as this results in resolution of the gradient as well, and prevents associated mitral regurgitation
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The natural history of HOCM is highly variable Several specific mutations are associated with a higher incidence of early malignant arrhythmias and sudden death, and definition of the genetic abnormality provides the best estimate of prognosis Some patients remain asymptomatic for many years or for life Sudden death, especially during exercise, may be the initial event The highest risk patients are those with a family history of sudden death, those with marked hypertrophy, and those that do not increase their systemic blood pressure with exercise HOCM is the pathologic feature most frequently associated with sudden death in athletes Pregnancy is generally well tolerated Endocarditis prophylaxis is indicated A final stage may be a transition into dilated cardiomyopathy in 5 10% of patients
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Harris KM et al Prevalence, clinical profile and significance of LV remodeling in the end-stage phase of hypertrophic cardiomyopathy Circulation 2006 Jul 18;114(3):216 25 [PMID: 16831987] Ho CY et al A contemporary approach to hypertrophic cardiomyopathy Circulation 2006 Jun 20;113(24):e858 62 [PMID: 16785342] Sherrod MV Pathophysiology and treatment of hypertrophic cardiomyopathy Prog Cardiovasc Dis 2006 Sep Oct;49(2): 123 51 [PMID: 17046437]
finding diagnostically The condition is relatively uncommon, with the most frequent causes being amyloidosis In Africa, endomyocardial fibrosis, a specific entity in which there is severe fibrosis of the endocardium, often with eosinophilia (L ffler s syndrome), is seen Other causes of restrictive cardiomyopathy are infiltrative cardiomyopathies (eg, hemochromatosis, carcinoid syndrome) and connective tissue diseases (eg, scleroderma) Amyloidosis results from deposition of various proteins within the myocardium Primary amyloidosis is caused by deposition of immunoglobulin light chains (AL) by monoclonal plasma cells, often as a consequence of multiple myeloma The heart may be the only organ involved at times Systemic amyloidosis is due to production of AA light chains Familial amyloidosis results from production of a carrier protein called transthyretin Systemic amyloidosis may affect any organ but is particularly associated with significant pulmonary and renal involvement and with a peripheral neuropathy (that often results in orthostatic hypotension)
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