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【2458】is this disease

Zhu Xing's mother recalled what the attending doctor said with difficulty. It was so difficult for her. Her brain was already so tired that she was about to go on strike.

The doctor can only help the family recall the key content related to the child's life: "Zhu Xing's heart has expanded to a ball. In his case, his condition can only become more and more serious. In this case, we must consider a heart transplant for him."

Heart dilation, at first glance, seems to be the dilated cardiomyopathy we have talked about before. Dilated cardiomyopathy is a type of cardiomyopathy.

Cardiomyopathy is divided into primary and specific (secondary), and the classification concepts of primary and secondary are consistent with other diseases.

Dilated cardiomyopathy is primary, but there are many categories of primary cardiomyopathy that are far more than dilated cardiomyopathy. The heart dilation of Zhu Xing’s child is primary cardiomyopathy, but it is not the dilated cardiomyopathy we have talked about before, but a genetic cardiomyopathy called congenital myocardial densification insufficiency.

This disease is relatively rare.

Why is it rare? It is either the symptoms are hidden and there is no disease in the patient's body, or the patient's condition is already very serious once the disease occurs. This disease is particularly difficult to detect through daily physical examinations such as electrocardiogram or routine auscultation. To find out, more precise instruments, including echocardiography, magnetic resonance CT and other means. If the patient is asymptomatic, who will do these further examinations? It will not happen, which leads to the doctor's real difficulty in finding it early.

What kind of disease is this disease? Since it is a genetic disease, the root cause is the dominant or recessive inheritance of human pathogenic genes. It is manifested in that many patients have family history and problems have occurred during the patient's heart during embryonic development.

Our heart does not grow coronary arteries in the first month of embryonic development. At this time, the main components of myocardium are muscle trabecular muscles and crypts. The heart's blood supply depends on crypts. These structures are pathologically non-densified layers of the ventricular wall. Normal embryo development is in the fifth and sixth week. The non-densified layer will densify and grow the epicardial endocardium, and the crypts will compress and turn into the coronary system.

For patients with myocardial densification insufficiency, the myocardial densification failed at this time, resulting in a pathological thickening of the non-densified layer of the ventricular wall. In this way, the patient's myocardial muscles are always mainly composed of muscle trabecular and crypts, and the grooves are interlaced like a sponge, so this disease is also called sponge-like cardiomyopathy.

Sponge-like cardiomyopathy can be an isolated disease with only this symptom, or it can also cause other cardiac malformations. In pediatrics, isolated myocardial densification is the main focus.

Zhu Xing is a typical congenital isolated myocardial incompleteness. Not only that, his father died suddenly, and it is not ruled out that he died because of this disease.

To sum up, the most damn thing about this disease is that if its lesion range is small, the patient can have normal heart function for a long time, confusing the patient and the doctor. When it occurs, the lesion range is large and the patient will develop heart failure, which will worsen all the way to stubborn heart failure and severe arrhythmias. Like Zhu Xing's heart dilated into a ball and cannot be reversed. Like dilated cardiomyopathy, the original cardiac surgery can only change the heart structure and cannot correct the lesion myocardium. The only way to save life is heart transplant.
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