Rare heart disease tako tsubo brugada syndrome and tank heart

The layman can still imagine something under the term heart valve malformation or ventricular fibrillation. But what are tako tsubo, Brugada syndrome or armored heart? The exotic names hide rare heart diseases – with symptoms that are often misinterpreted.

Cardiac specialist Professor Thomas Meinertz explains why its diagnosis can present challenges to cardiologists


Brugada syndrome: difficult to diagnose

The Brugada syndrome has only been known since the beginning of the 90s. The rare heart disease was discovered by Spanish cardiologists Josep and Pedro Brugada. Patients with Brugada syndrome appear to be heart healthy. Imaging procedures such as ultrasound, MRI and CT show no abnormalities.

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"Brugada syndrome can only be diagnosed with the help of an electrocardiogram (ECG), as it is a primarily electrical heart disease", says Professor Thomas Meinertz, cardiologist and pharmacologist from Hamburg and member of the Scientific Advisory Board of the German Heart Foundation e.V. "Abnormal discharges of damaged muscle cells occur in the right heart. Diagnosing it is not easy. Brugada syndrome is often overlooked."

Brugada syndrome: symptoms can be life-threatening

Brugada syndrome is based on a genetic defect that can be passed on. Sufferers suffer from seizure-like heart palpitations of a frequency of up to 200 beats per minute. Since the heart beats so fast that no more blood can be pumped into the circulation, there is a risk of unconsciousness and cardiovascular failure. The abnormal discharge of electrical impulses in the heart can be fatal if the cardiac arrhythmia leads to life-threatening ventricular fibrillation.

Often it is sports, high fever or certain medications that trigger the rare heart disease. "It is important that patients avoid triggering factors. If the heart palpitations recur, a defibrillator should be implanted or the damaged heart muscle cells sclerosed using catheter technology," says Meinertz.

Tako Tsubo: risk of confusion with heart attack

Tako Tsubo cardiomyopathy, or Tako Tsubo syndrome, or broken heart syndrome, presents similarly to a heart attack. Sufferers experience chest pain, shortness of breath, cold sweats and nausea. However, unlike myocardial infarction, Tako Tsubo does not involve a blocked coronary artery. Nevertheless, parts of the heart muscle die. It can become life-threatening.

"Rare heart disease is triggered by psychological trauma. This is why doctors also speak of "stress cardiomyopathy", explains Meinertz. "The intense psychological stress puts the heart into a state of shock. This means that there is no longer sufficient pumping capacity of the heart."

Tako Tsubo is often associated with losses such as death of a loved one, divorce, or loss of a job. Women are affected five times more often than men. "Tako Tsubo, just like a heart attack, can be acutely fatal.", says Meinertz. "Many patients have permanent damage to the heart. For others, however, the rare heart disease partially or completely heals on its own."

Armored heart: the "walled-in" heart Heart

The armored heart also belongs to the rare heart diseases . In this case, the pericardium is either enclosed by a layer of calcium or a solid layer of tie and can no longer expand properly. The heart is "walled in. Cardiac output is severely impaired. Today, the classic form of the armored heart, which is accompanied by calcification, is rarely found.

"Calcification is mainly associated with tuberculosis, which is almost non-existent in our latitudes", says Meinertz. "In our case, it is more likely to be the connective tie shell surrounding the heart caused by inflammation. The scarring is usually caused by viral infections, but can also be caused by tumors." The armored heart must be operated on in a high-risk operation and the firmly grown tie shell must be separated from the heart muscle. There is a risk of the heart muscle rupturing.

Recognizing rare heart disease: When to see a doctor?

The cardiologist advises to consult a doctor in case of any previously unknown complaints in the chest area. "It is always a warning signal when symptoms are newly added, do not subside or even increase", says Meinertz. "This should always be clarified. Often the cause is harmless – but sometimes not."

Possible indications of heart disease include:

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Warning signals from the chest:With angina pectoris, it gets tight around the heart

Narrowed coronary arteries:Coronary heart disease often goes undetected for a long time

– sudden drop in performance – difficulty breathing (often first with exertion, later at rest) – feelings of prere in the chest – chest pain – dizziness – swelling of the legs

The earlier a diseased heart is detected, the better the treatment options and the better the chances of therapy.

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