Hypertrophic cardiomyopathy is a disease characterised by an increase in the thickness of the heart muscle

The different forms of hypertrophic cardiomyopathy

Thickening of the heart muscle may affect only the wall dividing the two ventricles (septum) and be described as ‘asymmetrical’.

It can affect both the septum and the free wall of the left ventricle and be called ‘symmetrical’.

It can affect only the free wall of the left ventricle and be called ‘inverted’, or it can affect both ventricles and be called ‘biventricular’.

In addition, thickening of the heart muscle can in some cases create an obstruction to the exit of blood from the ventricle to the vessels that carry blood throughout the body, in this case being called ‘obstructive’.

The frequency in the general adult population is about 1 in 500 cases, but in children it is considered a rare disease and its frequency is estimated at about 1 in 30 000 children per year.

Hypertrophic cardiomyopathy may be primary (with no apparent haemodynamic cause of increased thickening) or with underlying forms secondary to other factors or other structural alterations of the heart (chronic arterial hypertension or valvulopathy).

In around 60-70% of sufferers, isolated hypertrophic cardiomyopathy is caused by alterations (mutations) in our DNA genes (genetically determined hereditary forms).

Once the genetic cause is established, screening of all first-degree relatives is strictly recommended.

Not all children with hypertrophic cardiomyopathy show symptoms

Children usually have no symptoms until adolescence, but in some cases these symptoms may appear earlier.

Possible symptoms include:

• Difficulty breathing, with shortness of breath and/or shortness of breath, usually during exercise;
• Chest pain;
• Heart palpitations;
• Fatigue;
• Transient loss of consciousness (syncope);
• Sudden death.

Hypertrophic cardiomyopathy may be suspected when it affects a first-degree relative of the child

The disease may also be suspected during a regular check-up by the treating paediatrician or during cardiological examinations such as an electrocardiogram (sports doctor’s assessment).

Once the disease has been diagnosed, doctors will conduct investigations to identify the underlying cause.

When there is a suspicion of hypertrophic cardiomyopathy, it must be performed:

• Measurement of heart rate, blood pressure, respiratory rate, peripheral oxygen saturation and body temperature;
• Cardiological examination;
• Chest X-ray;
• Electrocardiogram (ECG);
• Echocardiogram;
• Exercise test;
• 24-hour heart rhythm recording (Holter ECG);
• Blood tests;
• Molecular genetics examinations;
• Cardiac magnetic resonance imaging;
• Cardiac catheterisation, at least in some cases.
• There are currently no therapies that can cure this condition.

We do, however, have treatments that can control and improve the symptoms and quality of life of these children.

Medications that control the heart’s neurohormonal metabolism and help it relax, allowing the ventricle to fill up better with blood;

Implantable defibrillators, which interrupt malignant arrhythmias in patients at high risk of sudden death;

Surgery, necessary in a small number of patients to remove the thickened part of the septum that blocks the escape of blood from the heart;

Heart transplantation, in cases that are refractory and do not respond to standard therapies.

Currently, there are no therapies that can cure this condition.

We do, however, have treatments that can control and improve the symptoms and quality of life of these children

• Drugs that control the heart’s neurohormonal metabolism and help it relax, allowing the ventricle to fill up better with blood;
• Implantable defibrillators, which interrupt malignant arrhythmias in patients at high risk of sudden death;
• Surgery, necessary in a small number of patients to remove the thickened part of the septum that blocks the escape of blood from the heart;
• Heart transplantation, in cases that are refractory and do not respond to standard therapies.

On the whole, many children lead fairly normal lives, especially when the diagnosis is made early and specific treatments are used in good time.

The long-term course depends on the causes and severity of the disease.

However, all children with hypertrophic cardiomyopathy remain at risk of sudden death.

For this reason, competitive or competitive sport is contraindicated as the risk of haemodynamic instability under extreme physical stress is high.

Restrictions on physical activity should be customised on a case-by-case basis and recommended by the treating cardiologist

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Source:

Bambino Gesù