Cardiomyopathies: what they are and what are the treatments
Cardiomyopathies, our heart is a tireless muscle that never stops working: thanks to its beats, blood is pumped throughout the body, carrying the oxygen and nutrients we need to the brain and all the other organs
Having a healthy heart is essential for a good quality of life, so we need to take care of it, paying attention to our lifestyle and learning to pick up on those alarm bells that might indicate the need for medical attention.
Diseases that can affect the heart include cardiomyopathies, which affect the heart muscle (myocardium)
Cardiomyopathies can be of various types, the most relevant being dilated, hypertrophic, arrhythmogenic, or restrictive, and are a severe disorder that, if not treated in time, can lead to breathlessness, decompensation, and potentially fatal arrhythmias.
They can affect patients of any gender and age, including children and adolescents, most frequently between the ages of 20 and 40.
Dilated cardiomyopathy: what are the symptoms?
The most common cardiomyopathy is dilated cardiomyopathy, which affects the left ventricle, which, as its name implies, dilates causing an impairment of the blood pumping function that we call systolic/low ejection fraction heart failure.
The main complication it causes is heart failure, a very serious condition that can lead to death.
Among the various causes of dilated cardiomyopathy are previous myocardial infections, the use of chemotherapy drugs and alcohol abuse.
In about 40 per cent of cases, the cause is a DNA mutation involving genes involved in normal cardiac function; these forms may be familial, i.e. present in several individuals in the same family.
In other cases, however, the causes may not yet be clear and it is defined as idiopathic.
Its symptomatology, however, is similar to that of heart failure and includes weakness, generalised fatigue, shortness of breath, dry cough, swelling of the abdomen and legs, a feeling of dizziness and fainting.
The occurrence of arrhythmias can cause palpitations and fainting.
Hypertrophic cardiomyopathies: what are the causes?
In hypertrophic cardiomyopathy we always see an impairment of cardiac function, but this is due to a thickening of the myocardium, which compromises the elasticity of the left ventricle and the amount of blood that the heart can take in and pump.
Sometimes the thickening of the heart causes a true obstruction to blood flow (obstructive form), further compromising the function of the heart.
It is a rarer condition than the dilated form and is mainly due to genetic causes, thus arising in individuals predisposed from birth.
Hypertrophic cardiomyopathy is often asymptomatic, especially in the early stages.
The most common clinical manifestations are those related to heart failure (chest pain, shortness of breath, oedema) or the presence of arrhythmias (palpitations, fainting, sudden death), which most frequently occur during physical exercise.
Arrhythmogenic cardiomyopathies: a rare condition
Arrhythmogenic cardiomyopathy mainly involves the right side of the heart and increases the risk of cardiac arrhythmias.
The disease is genetically determined in 40% of cases and patients are often asymptomatic or paucisymptomatic for years.
The first symptoms usually appear between the ages of 30 and 50 and are due to arrhythmias that can also cause sudden death, especially in young people and athletes.
In advanced stages of the disease, the progression of heart dysfunction can lead to heart failure.
Restrictive cardiomyopathy: a condition often linked to systemic diseases
Restrictive cardiomyopathy involves a stiffening and loss of elasticity of the ventricular walls: here, too, the heart is unable to receive or pump an adequate amount of blood.
Moreover, in more advanced stages of the disease, the systolic function, i.e. the heart’s ability to contract, is also impaired.
In some cases, these are idiopathic forms, involving only the heart.
More frequently, it is linked to multisystem diseases such as amyloidosis and Fabry disease, rare diseases that interfere with organ function.
The onset of symptoms of restrictive cardiomyopathy may be gradual or sudden.
These are similar to those of other cardiomyopathies and include difficulty breathing, feeling short of breath, particularly during physical exertion, heart palpitation, and swelling of the legs and abdomen.
Other symptoms related to multisystem involvement such as gastro-intestinal and neurological disorders may be present.
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Cardiomyopathies: what tests to do for diagnosis?
Cardiomyopathy is usually diagnosed in the emergency room or when the patient, complaining of a particular symptomatology, requests a cardiological examination, perhaps on the advice of their general practitioner (but investigations are also frequent in patients who may be asymptomatic but are familiar with cardiological problems).
Tests to diagnose cardiomyopathy are generally non-invasive and include blood tests, electrocardiogram and echocardiogram.
If the specialist deems it appropriate, second-level examinations such as cardiac magnetic resonance imaging and cardiopulmonary testing may be necessary, which are essential to obtain more detailed information on the pathology.
If a genetic disease is suspected, genetic testing may be indicated.
How are cardiomyopathies treated?
As far as the treatment of cardiomyopathies is concerned, the route may, depending on the characteristics of the pathology, be pharmacological and/or involve the implantation of devices.
In general, for dilated cardiomyopathy, drugs inhibiting the renin-angiotensin axis, beta-blockers, angiotensin receptor antagonists and the new SGLT2 inhibitors are prescribed.
For hypertrophic and arrhythmogenic cardiomyopathy, the drugs used are always beta-blockers, but also antiarrhythmics and calcium channel blockers.
In the field of restrictive cardiomyopathy, on the other hand, it is essential to look for the presence of secondary forms of multi-system diseases in order to set the specific therapy.
In some cases, when the pathology is more severe or the risk of arrhythmias higher, pacemaker or defibrillator implantation is used.
When the disease is advanced and continues to progress, surgical action must be taken with the implantation of a ventricular assist device and, in extreme cases, heart transplantation.
The importance of genetic testing
As we have said, at the root of some forms of cardiomyopathy are hereditary genetic alterations, potentially transmissible within families.
In this context, genetic testing is of fundamental help in confirming a clinical suspicion and identifying individuals at increased risk within the family unit.
This makes it possible to plan specific prevention paths, which include periodic checks in order to make a rapid diagnosis and possible early therapies, and to learn about the possible risk of transmitting the identified variant to one’s children.
Given the complexity of cardiomyopathies, it is essential to take them to a specialised centre where second-level examinations can be carried out (cardiac resonance imaging, cardiopulmonary test, genetic test) and the presence of various specialists (decompensation cardiologist, electrophysiologist, geneticist, internist) enables patients to be correctly assessed and treated.
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