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Internal illnesses. Hypertrophic cardiomyopathy (most important)

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19. HYPERTROPHIC CARDIOMYOPATHY

Hypertrophy of the walls of the left ventricle (occasionally right) without expansion of the cavity, with increased systolic function and impaired diastolic function. Hypertrophy is more often asymmetric with a predominance of thickening of the interventricular septum, not associated with increased work of the heart.

Pathogenesis. Asymmetric septal hypertrophy leads to dynamic, then fixed obstruction of the outflow tract from the left ventricle. The ability of the myocardium to relax is reduced. The diastolic function of the left ventricle worsens due to calcium overload of the cytoplasm of cardiomyocytes and a decrease in the elastic properties of the myocardium.

Clinic. Shortness of breath, worsening with physical exertion, pain in the heart, muscle weakness, dizziness, fainting, palpitations, and attacks of loss of consciousness are noted. The disease can be asymptomatic or of the vegetodystonic type.

During auscultation at the apex and at the Botkin point, a systolic murmur of great variability is heard.

Diagnostic study. An ECG examination reveals signs of left ventricular hypertrophy; Atypical Q waves may appear - deep, narrow in leads II, III, aUR, V4, V5. Possible signs of WPW syndrome, repolarization disorders, ST segment reduction, T wave inversion.

To detect ventricular tachycardia, atrial fibrillation, daily Holter ECG monitoring is performed.

EchoCG study reveals changes in the thickness of the interventricular septum, which can reach 1,7-2 cm or more.

Treatment. β-blockers are prescribed in large doses, which increase left ventricular filling, reduce the pressure gradient and have an antiarrhythmic effect.

Calcium channel blockers are used. Verapamil has a negative inotropic effect on the myocardium, is prescribed 40-80 mg 3-4 times a day. Long-term treatment with this drug can lead to inhibition of automatism, deterioration of atrioventricular nodal conduction, and excessive negative inotropic action. You can not prescribe the drug with a large filling of the left ventricle, with orthopnea, nocturnal paroxysmal dyspnea.

Nifedipine is inappropriate, as it increases the pressure gradient due to a pronounced vasodilating effect (syncope).

Antiarrhythmic drugs are used. Cordarone is prescribed for ventricular arrhythmias: in the first week, 600-800 mg / day, then 150-400 mg daily with a two-day break every week. The effect of the drug comes in 1-2 weeks and persists for several months after withdrawal. Disopyramide is indicated for supraventricular and ventricular tachycardia.

Physical activity is limited, with infections, timely antibiotic therapy is carried out.

Cardiotonic agents, diuretics, nitrates, vasodilators are contraindicated in the treatment.

Of the surgical methods of treatment, excision of a part of the interventricular septum and mitral valve replacement are used.

Flow. Relatively favorable, progresses slowly.

Forecast. Adverse.

Author: Myshkina A.K.

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