Cardiomyopathy: What is it, how does it manifest? Causes in childhood and adulthood

Cardiomyopathy: What is it, how does it manifest? Causes in childhood and adulthood
Photo source: Getty images

Cardiomyopathy refers to a group of diseases that affect the heart muscle, the myocardium. We know several forms, they have different symptoms and also different treatments.

Characteristics

Cardiomyopathy is the name for a group of diseases affecting the heart muscle

Myocardium = heart muscle. 
Cardio = heart related, cardiac. 
Myo = related to muscle, muscles. 
Patia = disease ending. 

These diseases are divided into several forms. Each may have a different cause, manifested by different symptoms or necessary treatment.

In cardiomyopathy, the heart enlarges.

This results in a malfunction of the muscle. The roughened fabric has different properties and its elasticity does not meet the necessary requirements.

Disease changes are also affected by the conductive system of the heart, which results in heart rhythm disorders. We also know these as arrhythmias

The heart is a pump that pumps blood to the human body for life.

In the case of a blood supply disorder, the brain and the heart itself mainly suffer. Of course, so do other cells, tissues, and organs. With the sudden interruption of blood circulation and the failure of the heart as a pump, unconsciousness, respiratory arrest, and, after a few minutes, death.

Most often you are interested in: What is cardiomyopathy and why does it occur? What is the hypertrophic, dilated, restrictive, stressed or alcoholic form? How does it manifest and heal? Answers to these questions and other interesting information in the article.

Myocardium = heart muscle

The heart pumps blood to the whole body. And without a break for life.

This function is provided by the heart muscle. This is the thickest layer of the heart wall. Muscle with regular movements ensures the expulsion of blood into the body and its suction back.

Upon withdrawal, blood is expelled into the aorta. This phase is also called systole.

The weakening of the heart muscle then ensures the return of blood from the large veins to the heart. And this phase is called diastole.

The thickest layer of muscle is located in the left ventricle
The left ventricle blows blood into the aorta , and thus into the whole body. 
It must therefore overcome the highest pressure
Thanks to which oxygenated blood from the heart travels to the body.

The heart muscle is made up of muscle cells called cardiomyocytes. There is a combination of transversely striated and smooth muscle. 

Although it contains a type of transversely striated muscle fibers, its function cannot be controlled by will. Its influence has an autonomic nervous system, and frequency

The sympathetic nerve accelerates and the parasympathetic nerve slows down heart activity.

The very control of contraction, ie contractions and muscle weakness, is ensured by the heart's own transmission system.Disruption of this system results in various cardiac arrhythmias.

Like all cells in the human body, heart cells must be oxygenated and supplied with nutrients. Blood flows to them through blood vessels, also known as coronary arteries or coronary arteries.

This ensures trouble-free function and operation of the heart like a pump. If the supply of oxygen and nutrients is disrupted, then, depending on the extent of restriction or blockage of blood flow to the muscle, ischemic heart disease or myocardial infarction of the heart muscle occurs.

Layers and envelope of the heart
The inner layer of the heart = endocardium
- the inner lining of the heart cavities and also forms the heart valves
Middle layer - myocardium
The outer layer of the heart = epicardium
Pericardium = a bag in which the heart is stored. 

 +

The heart has 4 cavities: the right atrium - the entry of large veins and the inflow of deoxygenated blood from the body.
The right ventricle - the outflow of blood to the lungs, where it is re-oxygenated. 
Left atrium - entry of oxygenated blood back into the heart. 
Left ventricle - expels oxygenated blood into the aorta, and thus into the whole organism. 

The left ventricle has approximately three times the muscle mass of the right ventricle.

In the rest of the article, you'll learn: 
What is cardiomyopathy and how it divides
Sudden deaths in athletes and young people. 
Why cardiomyopathy occurs . What are the symptoms and treatment available

A closer look at cardiomyopathy

Cardiomyopathies are diseases of the heart muscle. The basis is a change in the size of the heart.

This disease change results in a change in the function of the heart muscle and transmission system. This is negatively reflected on the work of the heart as a pump and also on the heart rhythm.

In addition to cardiac arrhythmia, there is a risk of developing heart failure.

From a broader point of view, it is divided into primary or secondary

Primary = has no known cause. 
Secondary = specific disease with a known cause.

Cardiomyopathy is also referred to as KMP.

Cardiomyopathies are divided into several types, which are listed in the table

Type of  cardiomyopathy Description
1. Dilated
cardiomyopathy
  • dilatation = diseased enlargement, increase in volume, dilation
  • pathological enlargement of the heart = dilation of the heart compartments
  • the most common type
  • it affects the left ventricle to a greater extent
  • blood pumping disorder - reduction of the ejection fraction of the heart below 40%
  • various causes
    • ischemia and massive myocardial infarction 
    • unknown cause
    • viral disease - myocarditis = inflammation of the heart muscle
    • alcohol
    • heredity or impaired immunity and others
2. Hypertrophic
cardiomyopathy
  • hypertrophy = increase in cell, tissue, organ volume
  • enlargement of the heart muscle cells - especially the left ventricle and septum, ventricular compartments
  • the heart cavities do not expand
  • the expulsion of blood is not as disturbed as its filling into the ventricles of the heart
  • slowing of the filling of the heart chamber
  • increase in pressure inside the chamber
  • consequently, the pressure in the lungs also increases
  • obstructive form = disruption of filling and expulsion of the chamber
  • a common cause of sudden death in young people and athletes
  • genetic defects and hereditary factor
3. Restriction
cardiomyopathy
  • rare form
  • the heart is hardened - rigid
  • behind the formation are infiltration mechanisms  =
    pathological  deposition of substances in the wall of the heart
  • for example in diseases:
    • Amloidosis
    • Hemochromatosis
    • Sarcoidosis
  • the pumping and expulsion of blood is impaired
4. Arrhythmogenic dysplasia of the
right ventricle
  • ADPK
  • congenital genetic disease
  • fibrous damage of the right ventricle + fatty tissue remodeling
    • normal tissue is morbidly replaced by non-valuable adipose and fibrous tissue
  • change of electrical properties and creation of electrical excitations,
    which control the activity of the heart,
    come out and run through the area of ​​the right atrium and ventricle
  • often asymptomatic
  • possibly with the first symptoms in young people
    • palpitations = palpitations
    • apostasy and syncope = short-term disorder of consciousness
    • sudden death in a hitherto healthy person
  • already in people under 35 years of age
  • genetic basis
  • risk of malignant lethal arrhythmias
  • is not the cause of chronic heart failure
Other
rare forms
Tako Tsubo Cardiomyopathy  - TTK
  • stress cardiomyopathy -  stress KMP
  • you may have heard of broken heart syndrome
  • especially in women
  • named after the pitchers in which  Japanese fishermen catch squid 
    • these are called Takotsubo
  • the cause is excessive stress such as:
    • death in the family
    • divorce
    • or severe chronic mental stress
  • change the shape and size of the left ventricle
    • + disorder of the kinetic properties of the apex and base of the left ventricle
  • the condition usually resolves after a few weeks
  • however, during this time there is an increased risk of heart rhythm disorders
  • maybe confused with myocardial infarction
  • postpartum cardiomyopathy
  • non-compact myocardium
  • diabetic cardiomyopathy
  • and other

Sudden death in young people and athletes

Death of a young person may be the first sign of a previously unspecified heart defect. 

The fact that a now healthy person is experiencing a change in health leading to death is devastating. 

This is largely due to unrecognized hypertrophic cardiomyopathy or arrhythmogenic dysplasia of the right ventricle of the heart. And also other causes.

Severe - malignant heart rhythm disorderventricular tachycardia → progresses  to ventricular fibrillation .

These two arrhythmias cause the blood to fail to fill the heart and pump blood from the heart.

There is heart failure as with a pump. Death occurs without immediate assistance, defibrillation and resuscitation.

Therefore, it is important to pay attention to every dropout and collapse in young people, especially during sports activities or increased physical activity.

Important: Early detection of warning - alarming signs.

Alarming symptoms:

  • loss of consciousness, collapse, syncope, apostasy
    • about 18% have a cardiac cause
    • quick return to consciousness within 20 seconds
      • in uncomplicated syncope, a good sign
  • persistence of  impaired consciousness - unconsciousness 
    • when one does not take consciousness
  • body cramps in a person who is not being treated for epilepsy
    • onset of convulsions until loss of consciousness
    • in epilepsy, mostly with loss of consciousness
  • difficult to absent breathing
    • growl
    • catching breathing - fish breathing
    • irregular and ineffective breathing
  • discoloration of the skin of the face and lips
    • Gray color
    • bluish to purple color
    • beware heart disorder

Warning! Pulse measurement may have an incorrect result.The rescuer can feel his own pulse!  And not the pulse of the affected person.

You ask:

How to proceed?

We proceed as follows:

  1. secure access
  2. control of consciousness - by voice, touch
  3. does not answer?
  4. call for help 155 / in case of accident 112
    • the emergency operator asks questions
    • answer
    • will advise you on what to do in a situation immediately threatening your health and life 
  5. respiratory control
  6. not breathing normally? 
  7. his chest and abdomen are not raised
  8. airway clearance
    • head tilt
  9. still not breathing normally?
  10. revival
    • chest compression 5 - 6 cm to depth
    • 100 times per minute
    • center of chest
    • does the rescuer have a first aid course
      • +  mouth-to-mouth breathing
      • ratio of 30 chest compressions and 2 breaths - 30:2
      • inhalation takes about 1 second
    • if the rescuer does not have a first aid course, he only performs chest compressions!
  11. children have their first breaths - anyway!
    • 5 introductory breaths
    • subsequently compressing the chest to approximately one-third of the height
    • in children, the most common respiratory (respiratory) cause is circulatory arrest

Remember: In children, 5 first breaths, then chest compressions. Don't have a first aid course? = "ONLY" COMPRESS your chest.100 * 1 minanimation - resuscitation will not hurt. Broken ribs heal.Death is irreversible. Therefore, even imperfect resuscitation is BETTER  than DOING NOTHING!

Early defibrillation is important in heart rhythm problems. It could be described as resetting the heart's transmission system. And it should restore normal heart rhythm function.

The defibrillation public are designed automatic external defibrillators abbreviation AED

They are available in some  public places :stations,stadiums,sports halls, shopping mallsor workplaces. They are marked: White heart with lightning on a green field.

AED marking. Photo: Getty images

BUT...

In some cases, the error is detected accidentally even before the first symptoms occur. Or the first symptoms are less severe, such as palpitations, or palpitations.

In this case, preventive implantation of a cardioverter-defibrillator in a person at risk may be chosen. Another method is the treatment method, and thus catheter ablation, which interrupts the pathological conduction of stimuli through the heart and prevents the onset of arrhythmias.

Causes

The causes of cardiomyopathies are diverse. In some cases, the exact reason will never be found. This is referred to as primary cardiomyopathy.

The second group is secondary cardiomyopathies. These are specific forms of the disease, as listed in the table above. Their causes are usually different.

The table shows the distribution of causes according to the form of KMP

Form Causes
Dilatation KMP
  • ischemia - decreased myocardial blood flow
  • massive myocardial infarction
  • unknown cause
  • myocarditis  = inflammation of the heart muscle
    • viral disease
    • protozoa
  • drugs
  • alcohol
  • heredity and genetic factors
  • immune system disorder and autoimmune causes
  • pregnancy
  • Beri-Beri, a vitamin B1 deficiency disease
Hypertrophic KMP
  • HKMP
  • in most cases the genetic basis 
  • and thus also the familial occurrence
  • up to 50% of cases
  • approximately 150 gene mutations are known to which it is associated
  • Fabry disease , Danon's disease, and others
Restriction KMP
  • RKMP
  • based on the pathological deposition of substances in the heart in other diseases
  • Amyloidosis - metabolic disease, storage of protein, amyloid in organs, lungs, heart, liver, joints
  • Hemochromatosis - a disorder of iron metabolism, the storage of iron in organs and their subsequent damage, in addition to the heart, such as the liver and other
  • Sarcoidosis , also the deposition of small nodules in organs, is an unknown cause
  • post radiation damage
  • unknown cause
  • the heart is stiff, rigid
Arrhythmogenic dysplasia of the right ventricle
  • ADPK
  • genetically determined disease
  • fibrous damage of the right ventricle and fat remodeling
  • physiological tissue is replaced by non-functional adipose and fibrous tissue
  • this will cause a change in electrical properties and the generation of electrical excitations
Some other causes
  • rheumatoid arthritis 
  • scleroderma
  • heart valve defects
  • muscular dystrophy
  • thyrotoxicosis
  • diabetic KMP
  • in case of insufficient nutrition
  • for chronic  tachycardia
  • toxic causes
  • long-term and severe stress
  • unknown cause
  • and other

Interesting:
Normally, the left ventricle has a muscle thickness of approximately 12 millimeters.
In hypertrophic cardiomyopathy, it can be as much as 60 millimeters

You ask: At what age does it develop?

The age category is not precisely limited, as it can develop in children, young and adults.

Symptoms

Each form of cardiomyopathy may have different symptoms. However, it happens that it takes place asymptomatically, ie asymptomatically.

In some cases, serious heart problems occur first. An example is a heart rhythm disorder characterized by palpitations.

Sudden death may also be the most serious first symptom. The information resonates especially in the case of the death of a young athlete. 

In this case, mainly two forms of KMP are considered: 

  • arrhythmogenic right ventricular dysplasia 
  • hypertrophic cardiomyopathy

Until then, they run asymptomatic, are not diagnosed and the trigger is physical exertion.

Thus, the symptoms of cardiomyopathies cannot be precisely specified in one set. Sometimes KMP does not show up at all, other times it has typical symptoms of a cardiac problem.

The conditions with loss of consciousness and respiratory disorder are especially dramatic, as described at the beginning of the article in the section on the Death of young people and athletes.

From general symptoms may occur such as:

  • fatigue and weakness
  • fainting, collapse, syncope
  • increased fatigue and reduced performance
  • dizziness
  • swelling, first of the feet, ankles and later higher
  • heart rhythm disorders, irregular heartbeat, palpitations - palpitations
  • shortness of breath
  • chest pain

Initially, symptoms can only occur with more severe physical activity. Depending on the degree and extent, the required load rate decreases. Severe myocardial damage is manifested by minimal effort.

+

The disproportion in the size of the heart cavities results in the formation of blood clots (thrombi). Similar to cardiac arrhythmia. 

The basis is disruption of blood flow. The risk is the expulsion of clots from the heart to the body. A serious complication is a stroke.

Thrombus in the heart → after its release → embolus  →
risk of clogging of a vessel in another part of the body → embolization.

A common cause of brain non-blood flow is a thrombus expelled from the heart. Examples are atrial fibrillation, valve defects, and others.

Athlete's heart

The heart can adapt to long-term sports load and activity. Therefore, it is common for athletes, and especially in elite sports, for the heart to enlarge to some extent.

It is also hypertrophy.

This is usually an increase in chamber thickness to 13 millimeters.

Myocardial thickness has been reported to be up to 12 millimeters under normal circumstances.

Possibly.

The so-called gray zone is also mentioned. It is a thickening of the muscle up to 15 mm. In this case, further examinations and condition monitoring are required.

Diagnostics

Diagnosis focuses on the anamnesis and clinical picture, ie the manifestations of the disease. At a higher stage of the development of the disease, the symptoms that lead to the heart cause are usually present.

Of course, in addition to the physical examination, other specific examinations are also required.

An example is the ECG, which records the electrical activity of the heart. Possible arrhythmias are also detected here, and a specific ECG image is determined. A 24-hour recording, ie an ECG Holter, can be helpful.

It complements the ECHO. During echocardiography, ie the examination of the heart by ultrasound, the dimensions of the heart, its cavities and the thickening of the heart muscle wall, the enlargement of the heart sections, the condition of the valves, and other parameters can be determined.

MRI - magnetic resonance imaging can be performed to exclude structural changes. And to recognize coronary artery disease and atherosclerosis, coronary angiography is added

The basic diagnostics also include X-rays, CT, lungs and heart - chest, blood collection for laboratory examination, of course, blood pressure, pulse frequency, and its regularity are measured. Possibly genetic testing.

Course

Enlargement of the heart can be asymptomatic. And especially in the initial period. The asymptomatic course is interrupted by occasional difficulties, especially during exercise

Examples are palpitations, increased tiredness or dizziness, and a feeling of falling away.

Higher levels of physical exertion and provocative mechanisms initially cause milder difficulties. However, over time, symptoms of the disease may occur even after mild exercise.

Heart failure develops.

A high level of danger to health and life is conditions when a full acute deterioration occurs in full health. Rhythm disorders are mainly risky, Which can lead to unconsciousness, respiratory disorders, and even death. 

The table lists some of the symptoms that occur during dilated or hypertrophic KMP

Dilatation KMP Hypertrophic KMP
gradual heart failure arrhythmia
leads to cardiac decompensation chest pain
to  swelling of the lungs exertional dyspnea
commonly occurring: palpitations
arrhythmia to orthopnoea
palpitations - palpitations there are syncopations - falls off, especially during exercise
chest pain - angina pectoris, 
but with a normal finding in the coronary arteries
or milder pre-collapse states - a feeling of falling off
shortness of breath - dyspnoea, first after exertion muscle weakness
orthopnoea - objectively visible
labored breathing
fatigue 
night cough + the chamber of the heart fills worse due to its increased muscle mass, the expulsion of blood into the body circulation is not disturbed. The chamber fills more slowly and increased pressure is present
a cough
+ the left ventricle is normally filled, but there is a disorder of its emptying = expulsion of blood into the aorta

And ...

There is also swelling of the lower limbs, which develops over time and according to the degree of heart involvement. It progresses higher, from the legs, through the ankles, and to the forelegs to the thighs and abdomen. Thus, it may be a symptom of chronic heart failure.

People diagnosed with KMP are also at risk for developing malignant arrhythmia = a serious heart rhythm disorder. Such as ventricular tachycardia and fibrillation. 

How it is treated: Cardiomyopathy

Treatment of cardiomyopathy - medications and healthy lifestyle

Show more
fshare on Facebook

Interesting resources

  1. "What Are the Signs and Symptoms of Cardiomyopathy?". NHLBI. 22 June 2016. 
  2. "Who Is at Risk for Cardiomyopathy?". NHLBI. 22 June 2016.
  3. "Types of Cardiomyopathy". NHLBI. 22 June 2016. 
  4. "What Causes Cardiomyopathy?". NHLBI. 22 June 2016. 
  5. "How Is Cardiomyopathy Treated?". NHLBI. 22 June 2016. 
  6. GBD 2015 Disease and Injury Incidence and Prevalence, Collaborators. 
  7. "What Are the Signs and Symptoms of Cardiomyopathy? - NHLBI, NIH". www.nhlbi.nih.gov
  8. Bakalakos, Athanasios; Ritsatos, Konstantinos; Anastasakis, Aris (1 September 2018). "Current perspectives on the diagnosis and management of dilated cardiomyopathy Beyond heart failure: a Cardiomyopathy Clinic Doctor's point of view". Hellenic Journal of Cardiology59 (5): 254–261.
  9. Rath, Anika; Weintraub, Robert (23 July 2021). "Overview of Cardiomyopathies in Childhood". Frontiers in Pediatrics9: 708732. 
  10. Gorla, Sudheer; Raja, Kishore; Garg, Ashish; Barbouth, Deborah; Rusconi, Paolo (December 2018). "Infantile Onset Hypertrophic Cardiomyopathy Secondary to PRKAG2 Gene Mutation is Associated with Poor Prognosis". Journal of Pediatric Genetics07 (04): 180–184. 
  11. ^ Law, Michelle L.; Cohen, Houda; Martin, Ashley A.; Angulski, Addeli Bez Batti; Metzger, Joseph M. (February 2020). "Dysregulation of Calcium Handling in Duchenne Muscular Dystrophy-Associated Dilated Cardiomyopathy: Mechanisms and Experimental Therapeutic Strategies". Journal of Clinical Medicine9 (2): 520.