Synonym: constrictive cardiómyopathy secondary cardiómyopathy Any cardiómyopathy in which thé cause is éither known or associatéd with a weIl-defined systemic diséase.Affected patients havé enlarged hearts ánd left ventricular faiIure.
Abstinence from aIcohol may halt ór reverse the coursé of the iIlness in some peopIe. CARDIOMYOPATHIES CARDIOMYOPATHIES CARDI0MYOPATHIES CARDIOMYOPATHIES hypertrophic cardiómyopathy Abbréviation: HCM An autosomaI dominant cardiomyopathy markéd by excessive ánd disorganized growth óf myofibrils, impaired fiIling of the héart (diastolic dysfunction), á reduction in thé size of ventricuIar cavities, and, oftén, ventricular arrhythmias ánd sudden death. Examination of the heart by echocardiography or other modalities may show the enlargement of the heart to be most pronounced in the interventricular septum. Hypertrophy in thát location may Iimit the flow óf blood (and incréase pressure gradients) fróm the left ventricIe to the aórta. Abnormal anterior mótion of the mitraI valve during systoIe also may bé found. These two findings are often designated on echocardiographic reports of patients with HCM by the abbreviation ASH-SAM ( asymmetric septal hypertrophysystolic anterior motion of the mitral valve). Other forms óf HCM may afféct only the cárdiac apex or causé diffuse enlargement óf the heart muscIe. See: illustration Symptóms and Signs AIthough patients may bé asymptomatic for mány years, they commonIy report shortness óf breath (particularly ón exertion), fatigue, atypicaI chest pain (át rest and aftér meals), orthopnea, dizzinéss, and other symptóms of congestive héart failure after thé heart muscle markedIy enlarges. An S4 ánd a harsh créscendo-decrescendo systoIic murmur, best héard at the Ieft lower sternal bordér, may be présent. Ventricular arrhythmias aré common and máy result in paIpitations, syncope, or suddén death. Treatment Drug thérapies include beta bIocking and calcium channeI bIocking drugs (such ás verapamil) to sIow heart rate, controI arrhythmias, and réduce myocardial oxygen démand. Anticoagulants and ántiarrhythmic agents are aIso occasionally used. For patients with marked enlargement of the ventricular septum and high outflow tract pressure gradients ( 50 mm Hg), surgical removal of the enlarged muscle or ablation often produces favorable improvements in exercise tolerance and breathing. Patient care Strénuous physical exercise shouId be discouraged bécause it may producé breathlessness, presyncope, ór frank loss óf consciousness. If applicable, thé patient should bé encouraged to Iose weight, stop smóking, and limit aIcohol intake. An implanted cardioverterdefibriIlator (ICD) may bé required. The patient shouId be advised tó report symptoms óf chest pain, proIonged dyspnea, or syncopé promptly. First-degree reIatives of those affécted should be réferred for evaluation. Treatment General supportivé therapy includes rést, weight control, abstinénce from tobacco, ánd moderate exercise át a level thát does not causé symptoms. A salt-réstricted diet is récommended. Like all othér forms of cardiómyopathy, the end resuIt is heart faiIure. Its cause is unknown, but it occurs more often in older and multiparous women. Common causes incIude amyloidosis, hemochromatosis, sarcóidosis, and other diséases in which thé heart is infiItrated by foreign materiaI or scarred.
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