Zhu Xing's mother struggled to recall what the attending doctor 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 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 primary cardiomyopathy, but there are many classifications of primary cardiomyopathy that are far more than just dilated cardiomyopathy. Zhu Xing’s child’s heart dilation is primary cardiomyopathy, but it is not the dilated cardiomyopathy we talked about before, but a kind of dilated cardiomyopathy.
The genetic cardiomyopathy is called congenital noncompaction of the myocardium.
This disease is relatively rare.
Why is it rare? 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 detect it,
More sophisticated instruments are needed, including echocardiography, magnetic resonance CT, and other means. If the patient has no symptoms, who will go for these further examinations? 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 family histories. During the embryonic development of the patient's heart,
A problem has occurred.
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. The blood supply of the heart depends on the crypts. Pathologically, these structures belong to the ventricular wall.
The non-compacting layer. During the fifth and sixth weeks of normal embryonic development, the non-compacting 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 pathological thickening of the non-compaction layer of the ventricular wall. 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, so 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 main cause.
Zhu Xing was born with a typical case of isolated myocardial noncompaction. Not only that, his father died suddenly, and it cannot be ruled out that he also died because of 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 the patient and the doctor. When it attacks and the scope of the disease is large, the patient will
Heart failure occurs, and then deteriorates into refractory heart failure and severe arrhythmia. Like Zhu Xing, the heart expands into a ball and cannot be reversed. Like dilated cardiomyopathy, surgery on the original heart can only change the heart structure.
The diseased myocardium cannot be corrected, and the only way to save life is heart transplantation.
