Have you ever heard of a strong middle-aged or young friend who died suddenly? You must be wondering what disease caused him to die. Unfortunately, because the vast majority of Chinese people are unwilling to do the pathological autopsies due to the influence of traditional ideas, the causes of death for these people are often unclear. However, according to data published abroad, about half of these young and middle-aged men who are seemingly healthy but suddenly die of ventricular tachycardia or ventricular fibrillation caused by arrhythmogenic right ventricular cardiomyopathy (ARVC).

The disease was first reported by a French doctor named Fontaine in 1978. It is mainly characterized by the enlargement of the right ventricle, the disappearance of some parts of the myocardium and replaced with fat or fibrous tissue. What’s more, some children also suffered this kind of disease. At first, doctors believed that it was due to congenital hypoplasia of the right ventricle, but later in 1994, an international expert team commissioned by the World Health Organization (WHO) conducted an advanced investigation of this disease, which confirmed that it is a postnatal gradually developed and aggravated cardiomyopathy. That is, it should be classified as cardiomyopathy rather than congenital dysplasia. But today, due to the lack of sufficient understanding of the disease, a considerable number of doctors still call it right ventricular dysplasia (ARVD).

Why should we emphasize the differences in the name? That is, if it belongs to congenital dysplasia, it means that there is nothing doctors can do about it; However, if it continues to develop, although there are congenital genetic defects, it is still possible for the doctors and patients to take some measures to reduce or delay the development of the cardiomyopathy as much as possible. In fact, it has been found that the disease is caused by defects in some genes related to desmosomes, and acquired physical labor, stress, and physical exercise might accelerate and aggravate the development of the disease. Therefore, the incidence rate of men in the population is significantly higher than that of women, and people who love sports (especially athletes) or people who have been engaged in heavy manual work for a long time are more likely to develop ARVC. The time of the first onset also varies from person to person, most of which occur between the ages of 20 and 40, but there are also cases in children and the elderly.

The incidence of this disease ranges from 2/10000 to 44/10000. Such a big difference in the incidence mainly reflects the level of understanding of the disease among doctors in different regions. That is to say, if doctors lack understanding of the disease, it is easy to lead to misdiagnosis, especially for patients in the early and middle stages. Studies have shown that about 20% of patients with ARVC may have sudden death. At present, the diagnosis of ARVC still mainly depends on echocardiography, cardiac magnetic resonance, electrocardiogram, right ventriculography and myocardial biopsy.

The diagnosis of typical advanced-stage patients is relatively easy, but early and middle stage patients are often misdiagnosed because the right ventricular lesions are not typical. I would like to remind the majority of patients that if you have a sudden tachycardia or even syncope, you should call the emergency number or go to the hospital as soon as possible, and try to record the electrocardiogram at the time of onset, which is very important to judge whether it is ventricular tachycardia caused by ARVC. If your doctor tells you that it is the non-outflow ventricular tachycardia of the right ventricle (some doctors will call it the right ventricular inflow ventricular tachycardia), there is a chance that you have ventricular tachycardia caused by ARVC. Of course, some patients with ARVC may also have outflow ventricular tachycardia. In addition, it is worth pointing out that although this disease is called arrhythmogenic cardiomyopathy, not all patients will get ventricular tachycardia. A considerable number of patients only have ventricular extrasystole, especially in the early stage. Some patients will not have arrhythmias all their lives. Moreover, left ventricle could also be involved in some patients. The situations are totally different. If the lesion only involves the right ventricle, the patient can often tolerate it for a long time and have time to go to the hospital for rescue or treatment, but if it is complicated with left ventricular disease, the patient is more likely to faint or even die suddenly.

In terms of treatment, ARVC has two main manifestations. First, it causes ventricular tachycardia and ventricular fibrillation, which is characterized by palpitation, fainting and even death, which is the most important and dangerous symptoms of the disease. Second, it leads to right ventricular dysfunction, which is rare and the general symptoms are not that serious. Therefore, the first clinical treatment is to prevent sudden cardiac death from ventricular tachycardia or ventricular fibrillation. For patients with rapid ventricular rate during ventricular tachycardia or who had the history of syncope, the most reliable treatment is to implant an implantable cardioverter defibrillator (ICD), which does not cure the disease itself, but can deliver electric shock to save the patient's life when malignant ventricular tachycardia or ventricular fibrillation occurs. Unfortunately, this treatment is expensive, generally requires 100,000 to 200,000 RMB, and can be used for only 5-7 years. It is financially unbearable for many young patients. Some others worry about social discrimination in education, employment and marriage after the implantation. Moreover, the implanation of an ICD does not guarantee the 100% safety, and ICD itself sometimes also bring some complications and adverse effects.

Therefore, some doctors try to use catheter ablation to treat ARVC. Although it is theoretically possible to eliminate the lesion that causes ventricular tachycardia in ARVC, it is difficult to do so in practice. The main reason is that the risk of the operation itself, especially in the middle and late stage patients, because the right ventricle itself has been enlarged, and the part of the right ventricle has become thin and fragile, the operation may cause cardiac rupture or cardiac death. In addition, it is difficult to induce all types of VT in one operation, or although they have been induced, these VTs cannot be completely eliminated at one time, and two or more operations might be needed. Another possibility is that the lesion continues to develop after the operation, with the emergence of new ventricular tachycardia in other locations. Despite these difficulties, because the disease is one of the most dangerous heart diseases in clinic, and patients tend to be young, it has inspired some doctors to study it. At present, there are mainly a small number of hospitals in the United States, Europe and Japan that had carried out ablation of ARVC. However, no more than 30 cases has been reported even in the largest center at present. The number of cases of professor Yao Yan's team in Fuwai Hospital, China, is far more than 10 times that of other hospitals. After more than 10 years of painstaking research, This team have explored a whole set of unique mapping and ablation strategies for ventricular tachycardia in ARVC. They had not only completed the largest series of cases, but also achieved the highest success rate in the world. In the case of less than 1.3 operations on average, more than 80% of the patients no longer developed ventricular tachycardia during the follow-up period (up to 16 years). And the rest of the patients also improved significantly, that is, the incidence of ventricular tachycardia was significantly reduced, and the effect of medication was significantly better. In the past, the success rate of other well-known hospitals in the world was generally less than 50%, and the lowest was only 15%. In recent years, as the technique of epicardial mapping ablation by pericardial puncture has matured, the short-term success rate of ablation of the disease has significantly increased to about 80%. However, considering that the disease will gradually develop over time, the possibility of recurrence still exists in the future. Nevertheless, epicardial ablation often can only be done for once, and once recurrence, we have to go back to the less damaged endocardium for further ablation, but it may be more difficult because of pericardial adhesion.

It is worth pointing out that patients in developed countries often have implanted with ICD, even if the ablation surgery is not successful, the safety of patients is relatively guaranteed. However, the vast majority of patients in our country are unwilling or unable to implant an ICD. Although we have carried out this work for 10 years, but with a scientific attitude, we cannot say that we have completely conquered the disease, and long-term research and observations are still needed.

Therefore, after surgery, patients are still recommended:

1. Long-term use of beta blockers, you can add propranolol or sotalol;

2. Take vasodilators, ACEI or even diuretics if necessary;

3. Try to avoid heavy physical labor and physical exercise, adjust the psychological state to avoid excessive tension and other mental stimulation, in order to delay the development of the disease. This is particularly important. According to international multicenter observation, the earlier you start professional physical exercise or high-intensity physical exercise, the earlier and more serious the disease will occur. Once the disease is diagnosed, as long as you stop high-intensity exercise, the attack of ventricular tachycardia and fibrillation can also be significantly reduced and postponed.

4. Regular PE examination (echocardiography, electrocardiogram), usually at least once a year;

5. For early ARVC, if it is difficult to judge if the local doctor is inexperienced, intracardiac electrophysiological examination and even biopsy can be considered.