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¡¾2458¡¿This is the disease

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    Zhu Xing¡¯s mother struggled to recall what the attending doctor had said. It was really difficult for her. Her brain was so tired that it was about to go on strike.

    The doctor could only help the family recall the key points related to the child's life word by word: "Zhu Xing's heart has expanded to the size of a ball. In a situation like his, the condition can only get worse and worse, and he is where he is today."  In this case we must consider giving him a heart transplant."

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

    Cardiomyopathy is divided into primary and specific (secondary). The classification concepts of primary and secondary are consistent with other diseases and will not be discussed again.

    Dilated cardiomyopathy is a primary disease, but there are many classifications of primary cardiomyopathies 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 myocardial noncompaction.

    This disease is relatively rare.

    Why it is rare is because either the symptoms are very insidious and persist in the patient's body without the onset of the disease, or the patient's condition is already very serious once the disease occurs.  This disease cannot be detected through daily physical examinations such as electrocardiogram or routine auscultation.  To find out, more sophisticated instruments are needed, including echocardiography, magnetic resonance CT and other means.  If the patient is asymptomatic, who will go for these further tests? There won't be any, which makes it difficult for doctors to detect it early.

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

    Our heart does not have coronary arteries in the first month of embryonic development.  At this time, the main components of the myocardium are muscle trabeculae and crypts, and the blood supply to the heart depends on the crypts. Pathologically, these structures belong to the non-densified layer of the ventricular wall.  During the fifth and sixth weeks of normal embryonic development, the non-compacted layer will densify and grow into the epicardium and endocardium, and the crypts will compress to become the coronary artery system.

    Like patients with myocardial noncompaction, myocardial densification fails at this point in embryonic development, resulting in thickening of the non-compaction layer of the ventricular wall that can be seen pathologically.  As a result, the patient's myocardium is always mainly composed of muscle trabeculae and crypts. The grooves are intertwined like a network and resemble a sponge. Therefore, this disease is also called cavernous cardiomyopathy.

    Cavernous cardiomyopathy 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 predominant condition.

    Zhu Xing is a typical case of congenital isolated myocardial noncompaction.  Not only that, his father died suddenly, and it cannot be ruled out that he died due to this disease.

    To sum up, the most damning thing about this disease is that if the scope of the disease is small, the patient can have normal heart function for a long time, confusing both the patient and the doctor.  When it attacks, the scope of the disease is very large and the patient will develop heart failure, which will then deteriorate into refractory heart failure and severe arrhythmia. Like Zhu Xing, the heart is dilated into a ball and cannot be reversed.  Like dilated cardiomyopathy, cardiac surgery can only change the structure of the heart but cannot correct the diseased myocardium. The only way to save life is heart transplantation.

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