Zhu Xing's mother struggled to recall what the attending physician had said. It was so difficult for her that 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 his case, his condition will only get worse. At this point, we must consider giving him a heart transplant."
At first glance, heart dilation may sound like 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 the same as other diseases and will not be repeated here.
Dilated cardiomyopathy is a primary disease, but there are many types of primary cardiomyopathy, far more than just 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 is it rare? Because either the symptoms are very hidden and the disease never occurs, or once it occurs, the patient's condition is already very serious. This disease is special because it cannot be detected through daily physical examinations such as electrocardiograms or routine auscultation. To detect it, more sophisticated instruments are needed, including echocardiograms, magnetic resonance imaging, and CT. If the patient has no symptoms, who would do these further examinations? No one would, which makes it difficult for doctors to detect it in the early stages.
What kind of disease is this? 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 a family history and problems have already occurred in 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 myocardium is mainly composed of trabeculae and crypts. The heart's blood supply depends on the crypts. These structures belong to the non-compacted layer of the ventricular wall in pathology. In the fifth and sixth weeks of normal embryonic development, the non-compacted layer will compact and grow into the epicardium and endocardium, and the crypts will compress to become the coronary artery system.
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For example, in patients with incomplete myocardial compaction, myocardial compaction fails at this point in embryonic development, resulting in the thickening of the non-compacted layer of the ventricular wall in pathology. As a result, the patient's myocardium is always mainly composed of trabeculae and crypts, and the grooves are like a network, like a sponge, so this disease is also called sponge cardiomyopathy.
Spongy cardiomyopathy can be an isolated disease with only this symptom, or it can be complicated by other heart malformations. In pediatrics, isolated myocardial noncompaction is the main disease.
Zhu Xing is a typical case of isolated congenital myocardial incomplete compaction. Not only that, his father died suddenly, and it is not ruled out that he died of this disease.
To sum up, the most damning thing about this disease is that if the lesion is small, the patient can have normal heart function for a long time, confusing the patient and the doctor. When it occurs, the lesion is large and the patient will suffer from heart failure, which will then deteriorate into refractory heart failure and severe arrhythmia. For Zhu Xing, whose heart has expanded into a ball, it is impossible to reverse it. Just like dilated cardiomyopathy, surgery on the original heart can only change the heart structure but cannot correct the diseased myocardium. The only way to save life is heart transplantation.
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