Chapter 2458： 【2458】is this disease

Chapter 2458 [2458] This is the disease

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

The doctor can only help the family recall the important content related to the child's life word by word: "Zhu Xing's heart has expanded to the size of a ball. In his case, the condition can only get worse and worse. In that case we have to consider giving him a heart transplant."

Heart dilation, at first glance it sounds like the dilated cardiomyopathy we talked about earlier. Dilated cardiomyopathy is a type of cardiomyopathy.

Cardiomyopathy is divided into primary and specific (secondary), and the classification concept of primary and secondary is consistent with other diseases and will not be discussed.

Dilated cardiomyopathy is a primary, but there are many categories of primary cardiomyopathy far beyond dilated cardiomyopathy. Zhu Xing's heart dilation is a primary cardiomyopathy, but it is not the dilated cardiomyopathy we talked about before, but a genetic cardiomyopathy called congenital insufficiency of myocardial compaction.

This disease is relatively rare.

Why is it rare, because it is either very insidious and has been present in the patient's body without developing the disease, or the patient's condition is already very serious once it occurs. This disease cannot be detected through routine physical examinations such as electrocardiogram or routine auscultation. To find out, need to rely on more sophisticated equipment, including echocardiography, magnetic resonance CT and other means. If the patient is asymptomatic, who will do these further examinations, there will be none, which makes it difficult for doctors to detect it at an early stage.

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 many patients with family history, in which the patient's heart has problems during embryonic development.

Our heart does not develop coronary arteries in the first month of embryonic development. At this time, the main components of the myocardium are trabeculae and recesses, and the blood supply of the heart depends on the recesses. These structures belong to the non-densified layer of the ventricular wall pathologically. In normal embryonic development, in the fifth and sixth weeks, the non-densified layer will densify and grow out of the epicardium and endocardium, and the crypt will compress into the coronary system.

Like in patients with noncompaction of myocardium, myocardial densification fails at this time point in embryonic development, resulting in pathologically visible thickening of the non-compacted layer of the ventricular wall. In this way, the patient's myocardium is always mainly composed of muscle trabeculae and recesses, and the grooves and grooves are interlaced like a network, which is like a spongy shape, so this disease is also called spongiform cardiomyopathy.

Spongiform cardiomyopathy It can be an isolated disease with only this symptom, or it can be complicated by other cardiac malformations. In pediatrics, isolated myocardial noncompaction is the main disease.

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

In summary, the most damning thing about this disease is that if it has a small lesion, the patient can have normal heart function for a long time, confusing the patient and the doctor. When it occurs, the lesions are very large, and the patient will develop heart failure, and then it deteriorates into intractable heart failure and severe arrhythmia. Like Zhu Xing, the dilated heart cannot be reversed. Like dilated cardiomyopathy, the original heart surgery can only change the heart structure and cannot correct the diseased myocardium. The only way to save life is heart transplantation.

(end of this chapter)