Cardiomyopathy treatments and therapies: medical therapy

In any case, the choice of treatment varies from individual to individual. It must be made by an expert cardiologist, based on symptoms and an evaluation of numerous factors, including age, symptoms, ECG, ventricular function, and, above all, the specific disease causing the cardiomyopathy. Treatment can improve with appropriate medications.

However, patients with significant and persistent symptoms, or with atrial arrhythmias (e.g. atrial fibrillation) or ventricular arrhythmias (e.g. non-sustained ventricular tachycardia), or progressive left atrial dilation, who do not respond to medical therapy, are candidates for elimination of the obstruction and the often associated mitral regurgitation with appropriate interventions.

Beta-blockers slow the heartbeat and reduce its contraction force, but at the same time protect the heart, especially if it is dysfunctional. In medical practice they are also widely used for other types of heart disease and for arterial hypertension. Their dosage must be established on the basis of various factors, mainly heart rate, which must be maintained during the day between 50 and 65 beats per minute; if the heart rate is excessively slow (below 50 beats per minute) it is advisable to contact the cardiologist again.

In HCM contribute to reducing angina, shortness of breath, palpitations and especially obstruction. In CMD they have been used for over 30 years, with excellent results, especially in patients with a relatively high heart rate of over 80 beats per minute.

One of the main contraindications is cardiac asthma or asthmatic bronchitis, but also psoriasis and intolerance to cold of the hands and feet (e.g. chilblains).

There are many types of beta-blockers, with different effects depending on the dosage and their potency. Due to their specific characteristics, they are used preferably Bisoprolol or Carvedilol for CMD and patients with heart failure, Nadolol for HCM , Atenolol for arterial hypertension.

Diltiazem e Verapamil are calcium channel blockers that are now rarely used in CMI to reduce anginaThey have little effect on obstruction. If combined with beta-blockers, they can cause excessive slowing of the heart rate and their effects must therefore be carefully monitored.

When there is heart failure, even of a mild degree, are indicated diuretic drugs drugs, which increase the leakage of accumulated liquids, with the emission of large amounts of urine.

The use of diuretics can reduce sodium and potassium levels, which should be periodically monitored.

These drugs have been used successfully for many years in the therapy of decompensation. Dosages are variable. Much attention must be paid to the simultaneous adequate intake of fluids (water, juices, etc.) in order to avoid significant lowering of blood pressure (hypotension). They are contraindicated in obstructive CMI HCM, even if the patient has associated arterial hypertension.

These drugs (Spironolactone, Canreonate and Eplerenone) have the function of preventing the reduction of potassium levels, which could promote even life-threatening arrhythmias.

Their association with vasodilator drugs requires careful monitoring of potassium.

It is a selective inhibitor of the slow sodium current, which has demonstrated significant anti-ischemic efficacy in patients with coronary artery disease. In addition to its anti-ischemic effect, ranolazine has shown a good anti-arrhythmic effect, documented in some patients with HCM and in laboratory studies on heart cells extracted during myectomysurgery. It is used to control symptoms of angina at rest in non-obstructive HCM.

Amiodarone It is the most used antiarrhythmic drug in HCM above all after the age of 40It is highly effective in controlling arrhythmias, both atrial (AF) and ventricular (SVT), although it is less effective in preventing cardiac arrest and sudden death. However, it has several potential side effects, including skin hypersensitivity to sunlight, requiring avoidance of sun exposure or use of a high-SPF sunscreen. Furthermore, periodic monitoring of thyroid hormones (FT4 and TSH) is necessary, every four to six months at most, to identify a reduction (hypothyroidism) or increase (hyperthyroidism) in thyroid function.

Amiodarone can be combined with beta-blocker drugs, but the heart rate must be carefully monitored to avoid excessive slowing of the heartbeat (bradycardia), unless a pacemaker or ICD is present.

When using Amiodarone, potassium values ​​should always be above 4 mEq / L.

Other antiarrhythmic drugs used, more rarely, are Sotalol, Propafenome and Flecainide, but their effects must be carefully monitored. These drugs should never be combined with Amiodarone.

The therapy Antiarrhythmic therapy in patients with Cardiomyopathsmust be decided by an expert cardiologist and its positive or negative and/or potentially harmful effects must be carefully evaluated.

La Atrial Fibrillation (AF) and the much more serious episodes of Sustained ventricular tachycardia (SVT) or ventricular fibrillation (VF) can be interrupted with an electric shock to the chest with restoration of sinus rhythm, by means of a External defibrillator, under temporary anesthesia, or from Implantable Defibrillator (ICD)

The discharge of the External defibrillator is almost always effective in restoring normal rhythm; it is essentially risk-free, with the necessary precautions.

Disopyramide It is a drug, initially used as an antiarrhythmic, which may be useful in controlling obstruction in HCM, often in association with Beta-blockersIt can improve symptoms, at least temporarily. Its effectiveness is greater in older people.

Mavacamten It is a new drug, still under investigation, that modulates the contraction force of heart cells by reducing the activation of myosin. Preliminary results seem to indicate good efficacy in obstructive HCM.

When obstructive HCM is present, certain medications are contraindicated: Vasodilators, digitalis derivatives, and diuretics, which can increase obstruction.

For about 20 years it has been possible to intravenously administer the enzyme α-galactosidase every XNUMX weeks, more effective especially in the non-advanced stages of the disease and at an adequate dosage (1 mg / kg) . For some years now, an oral therapy has also been available with “Migalastat”, to be taken daily and used when the enzymatic activity is only reduced and not absent. Several new therapies are also being studied, including gene therapy.

Until a year ago, therapy was only aimed at reducing symptoms and avoiding events.

In 'Transthyretin TTR Amyloidosis, both genetic and “senile”, the effectiveness of a new drug has recently been demonstrated (“Tafamidis”), which works by stabilizing the amyloid substance deposited between cardiac cells, slowing down the accumulation and progression of the disease.