Cardiomyopathy: Types, Symptoms, Treatment

Scientific editor: Strokina OA, therapist, doctor of functional diagnostics.
November, 2018.

Cardiomyopathy - a change in the heart muscle is often unclear reasons.

Condition diagnosis "cardiomyopathy" is lack of (Or exclusion after the test)

• congenital malformations,
• valvular heart disease,
• lesions caused by systemic vascular diseases,
• hypertension,
• pericarditis,
• Some rare variants defeat the cardiac conduction system.

Cardiomyopathies are primary, when the disease process affects only the heart, and secondary, developing as a result of a systemic disease.

There are three main types of damage to the heart muscle with cardiomyopathies, respectively, allocated¹

• dilated cardiomyopathy,
• hypertrophic cardiomyopathy
• restrictive cardiomyopathy.

This separation is usually based on an evaluation of intracardiac blood flow and at an early stage of the disease can, in some cases possible to set the search direction causes process. When an unknown root cause of the defeat of talk about idiopathic forms varying cardiomyopathy.

Dilated cardiomyopathy (DCM)

For dilated cardiomyopathy is characterized by violation of the contractile function of the heart muscle (myocardium) with significant enlargement of the heart chambers. Its occurrence is associated with genetic factors, as occurs familial disease. Equally important are observed and disorders of the immune regulation.

The symptoms of dilated cardiomyopathy

Manifestations of dilated cardiomyopathy are determined by the rise heart failure² :

• dyspnea on exertion,
• fast fatiguability,
• swelling in the legs,
• pale skin,
• bluish fingertips.

The prognosis of dilated cardiomyopathy is very serious. Joining atrial fibrillation worsens the prognosis. In the first 5 years of the disease are dying up to 70% of patients, although regular, controlled by drug therapy, of course, possible to achieve the extension of life of the patient.

Women with dilated cardiomyopathy, pregnancy should be avoided, since the frequency of maternal mortality in this diagnosis is very high. In some cases, it noted the provocative effect of pregnancy on disease progression.

Diagnostics

Laboratory diagnosis gives little valuable data for diagnosis, but it is important to monitor the effectiveness of the treatment in the assessment of water-salt balance, in order to avoid some of the side effects of drugs, such as cytopenia.

The main instrumental method of diagnosis of all types of cardiomyopathy is a heart ultrasound (echocardiography). In most cases, the instrumental diagnosis of dilated cardiomyopathy can be delivered at the first ultrasound.

ECG has no specific criteria for the diagnosis of dilated cardiomyopathy, but you can to detect various types of heart rhythm disturbances up to ventricular arrhythmias (better identified at daily Holter ECG) signs of the load on the left ventricle.

Radiologically determined by an enlarged heart, and later signs of venous congestion in the lungs.

Coronary angiography - to eliminate the causes of ischemic enlargement of the heart chambers.

In vivo myocardial biopsy in theory helps to eliminate the specific causes (viruses, amyloidosis).

The diagnosis of dilated cardiomyopathy - diagnosis of exclusion. He placed when all possible causes of cardiac abnormalities excluded.

DCM treatment

Treatment of dilated cardiomyopathy is aimed at combating heart failure, prevention of complications. In the case of acquired DCM treatment is aimed at the main cause.

Major efforts in therapy directed to the improvement of myocardial contractility and reduction of heart failure symptoms using ACE inhibitors. It is possible to use any of the drugs in this group, but wider than other currently used enalapril (renitek, enap).

If there are contraindications to the use of ACE inhibitors administered Sartai (valsartan, losartan), which have the same properties.

Selection of a particular drug is determined by tolerability, blood pressure response, side effects.

An important place is occupied by the use of small doses of beta-blockers. Treatment starts with the minimum doses. In the case of good endurance dose can be increased, watching for signs of heart failure is increasing.

A promising application of carvedilol drug³ - beta-alpha-blocker with unique for this group of positive antioxidant effects on the myocardium.

As with the treatment of heart failure caused by other diseases, great importance has traditionally use of diuretics. Their effect is monitored patient weight control (preferably several times a week or daily), the measurement of urinary volume, watching the blood electrolyte composition.

Due to the poor prognosis of the disease, patients with dilated cardiomyopathy are considered candidates for heart transplantation.

hypertrophic cardiomyopathy

hypertrophic cardiomyopathy⁴ - a disease characterized by a significant increase in left ventricular wall thickness without expanding its space. Hypertrophic cardiomyopathy can be either congenital or acquired. The probable cause of the disease are genetic defects.

Symptoms of hypertrophic cardiomyopathy

Most often the disease is asymptomatic or mild symptoms. Many patients often are not even aware that they have a serious heart condition.

Manifestations of hypertrophic cardiomyopathy is determined by the complaints of shortness of breath, chest pain, tendency to fainting, palpitations.

Because arrhythmias patients often die suddenly. Hypertrophic cardiomyopathy is often found in young men who died during exercise.

In some patients gradually developing heart failure. Sometimes, especially in older, circulatory failure develops suddenly after long-term favorable course of the disease for many years.

As a result of violation of a relaxation of the left ventricle observed pattern of heart failure, although Left ventricular contractility remains at normal parameters until the beginning of the terminal stage disease.

The cause of circulatory disorders in hypertrophic cardiomyopathy is a reduction in tensile chambers of the heart (especially the left ventricle). Left ventricle changes its shape, which is determined preferentially localized area of ​​thickening of myocardium.

Diagnostics

For examination of the patient with suspected hypertrophic cardiomyopathy using instrumental and laboratory methods.

laboratory

1. Routine laboratory diagnostics:

• general blood analysis,
• general urine analysis,
• glucose,
• cardiac enzymes - CPK, AST, ALTLDH)
• blood lipids,
• thyroid hormones
• renal and liver function tests,
• electrolytes
• uric acid.

All this reveals the extracardiac condition that can worsen heart failure.

2. Analysis of blood level of brain natriuretic peptide to assess the severity of heart failure. Its highest rate is strongly correlated with the severity of diastolic (relaxing) function of the heart.

instrumental

An electrocardiogram is characterized primarily by signs of thickening of the left ventricle, as well as a variety of cardiac arrhythmias.

Radiologically the disease can not be detected for a long time, since the outer contour of the heart does not change. Later, there are signs of pulmonary hypertension.

ultrasound of the heart (Echocardiography) - reliable method for early diagnosis of this disease, as is able to detect a change in internal contours of the left ventricular cavity.

Magnetic resonance imaging MRI of the heart - more expensive method than ultrasound, but has greater resolution. Thanks to him, specialists get a clearer picture and information about the structure of the body.

The prognosis of hypertrophic cardiomyopathy

The prognosis is more favorable in comparison with other forms of cardiomyopathy.

Patients remain operable for a long time (with regard to their profession). However, in these patients the cases of sudden death are recorded with high frequency.

Clinic Heart Failure formed quite late. Joining atrial fibrillation worsens the prognosis. Pregnancy and childbirth in hypertrophic cardiomyopathy possible.

Treatment

Treatment is aimed at reducing the severity of symptoms, improving the quality of life of the patient and the prevention of attacks of sudden cardiac death and disease progression.

The most commonly used verapamil or diltiazem. Widely used beta-blockers, which help to reduce the heart, prevent the occurrence of arrhythmias and decrease myocardial oxygen demand.

Recommended application disopyramide (ritmodan).

When the detected violations rhythm (atrial fibrillation) amiodarone or sotalol are used. In this case, the need for prevention of thrombus formation in the left atrium, which is administered anticoagulants (warfarin).

Also, there is a surgical treatment of hypertrophic cardiomyopathy:

• excision of part of the interventricular septum at its base;
• moxibustion hypertrophied septum base 96% alcohol by means of a catheter;
• in recent years, it recommended the use of a dual-chamber pacemaker for permanent pacing.

restrictive cardiomyopathy

Restrictive cardiomyopathy - a disease of the myocardium, characterized by the violation of the contractile function of the heart muscle, reduced relaxation of its walls. Myocardium becomes rigid, the walls do not stretch, suffers filling of the left ventricle with blood. Thickening ventricular wall or its extension is not observed, unlike atrial experiencing increased load.

Restrictive cardiomyopathy - most rare form, it exists in an independent embodiment as well as in the defeat of the heart with a sufficiently large range of diseases, which should be excluded in the specification of diagnosis.

it amyloidosis, hemochromatosis, sarcoidosis, Endomyocardial fibrosis, Leffler disease fibroelastosis sometimes defeat the cardiac conduction system (Fabry disease), systemic sclerosis, and others. In children, heart disease occur due to glycogen metabolism disorders.

symptoms

Intrigue of the disease lies in the fact that the complaints of the patient for the first time there are only at the stage of heart failure related to the terminal part of the disease. Usually, the cause of treatment is the appearance of peripheral edema, increase in size of the abdomen (ascites - fluid in the abdominal cavity), swelling of the jugular veins in the neck. A little later, a clinic attached dyspnea.

Diagnostics

At the heart of the X-ray is normal size, but revealed an increase in the atrial and signs of stagnation of blood in the pulmonary circulation.

ECG changes are not specific.

Ultrasound of the heart (echocardiography) Provides valuable information:

• measuring myocardial thickness (expressed wall thickening is observed at a somewhat smaller amyloidosis and their variation in other diseases),
• increase in left atrial size, evaluation of the pericardium (the differential diagnosis of diseases of the pericardium, which can give the same symptoms; the presence of calcification in the pericardium exclude the diagnosis of restrictive cardiomyopathy).

Laboratory diagnosis of restrictive cardiomyopathy has no specific symptoms, but it is important to identify secondary causes of heart disease.

Treatment of restrictive cardiomyopathy

The treatment is a difficult task due to delays in seeking patients, diagnostic difficulties, the lack of reliable methods of stopping the process. Heart transplantation may not be effective because of the relapse process in the transplanted heart.

When the prescribed character secondary lesion are specific ways to influence, e.g. cupping hemochromatosis, sarcoidosis corticosteroids.

The rest of the treatment is symptomatic, aimed at eliminating the heart failure clinic. antiarrhythmic drugs prescribed for heart rhythm disturbances. For the prevention of thromboembolic complications are used anticoagulants.

Forecast

Patients with restrictive cardiomyopathy disabled. Unfavorable prognosis disease, mortality reaches 50% at 2 years.