A link is established between the mutation γ-субъединицы CIDA and development SCOP. Classification Depending on the presence or absence of a gradient systolic pressure in the cavity of LV SCOP divided into obstructive and neobstruguuyu, which has practical significance in the choice of tactics treatment. There are three options gemodinamicakih obstructive SCOP : * subaortalina with obstruction at rest (the basal obstruction); * Labilna with obstruction, which is characterized by significant fluctuations spontaneous vnutrijeludochkovogo pressure gradient for no apparent reason; * Conjunction with obstruction, which is only under load and provocative clinical samples (such as fertilizer amilnitrita, to the reception of nitrates or intravenous izoproterenola introduction). Morphology typical morphological changes : anomaly архитектоники сократительных of heart (hypertrophy and disorientation muscle fibers), the changes фибротических heart muscles, pathology small интрамиокардиальных receptacles. Clinical symptoms of the disease varied and malospecificna relate to the violations of haemodynamic (diastolical dysfunction, dynamic obstruction of the outflow, mitralnaya regurgitation), heart disease, cardiovascular disease gene disorders and a violation elektrofiziologicak their processes in the heart. The range of clinical manifestations, it is great, from asymptomatic to steadily progressing and the difficult medication forms, accompanied by severe symptoms. The first and only manifestation of the disease can be sudden death. Clearly identified five basic options currents and outcomes : * stable, benign form of the disease; * Sudden death; * Progressive course : increased breathlessness, weakness, fatigue, pain syndrome (abnormal pain Drinking), the emergence of presincopalnah sincopalnah and conditions of sistolicescoy LV dysfunction); * "Final phase" : the further progression of events congestive heart failure the ремоделированием and систолической LV dysfunction; * development of atrial fibrillation and its associated complications, in particular thromboembolitic. Diagnosis There is a widespread increase in the number of reported cases of the disease, both through introduction of modern methods of diagnosis, and likely to be associated with the real increase in the number of patients SCOP. According to the latest studies, the incidence of disease in the general population is greater than previously believed, and is 0.2%. SCOP is diagnosed at any age from the early days of the last decade of life, regardless of gender, race, but the disease is diagnosed in young people of working age. Ekg-priznaki SCOP : signs of atrophy turned left, the emergence of zubza Q, recovery zubza T, widening or increase tension zubza R, WPW syndrome, fibrillace predserdy, Av-blokada. ЭХО-критерии SCOP : 1. Plus : sistolicescoe the front door Mitral Valve forward and asymmetric hypertrophy left heart; 2. Small : normal or small size of the left heart of the increasing fraction of release, bringing the front door mitral valve to mejjeludockova resting during diastola; Reducing the amplitude of early diastolic of mitral valve; Pressure gradient between the aorta and the left heart of more than 50 mm. Hg. Art.
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