Standardize the treatment of precocious heart disease in China

  Congenital heart disease refers to the following two conditions: one is a local anatomical structure abnormality caused by the formation disorder of heart and large blood vessels during human embryonic development, and the other is a normal physiological channel in fetal period that should have closed automatically after birth but failed to close in time, resulting in the long-term retention of this channel, referred to as congenital heart disease. According to the data, congenital heart disease is the first malformation of birth defects in China, the incidence accounts for between 6.87‰ and 14.39‰ of newborn babies, and there are 150,000 to 200,000 new cases of congenital heart disease in China every year. Li Tianchang, Department of Cardiology, Naval General Hospital Congenital heart disease is a general term for a series of congenital heart, blood vessel development and morphological and structural abnormalities, the site and form of local morphological and structural abnormalities are different, their impact on hemodynamics is also different, the early and late appearance of clinical manifestations, the severity and impact on growth and development and prognosis are also different, and cannot be treated equally; some congenital heart diseases do not cause any adverse impact on the growth and development and health of the children. Some of them do not have any adverse effects on growth and health and do not require treatment for life; others require emergency treatment within 1-2 days or even hours after birth to save the child’s life; and the vast majority of patients with precocious heart disease require elective treatment. The vast majority of precardiac diseases can be completely cured with appropriate surgical or interventional treatment at the right time of the disease; otherwise, they can become incurable or incurable.  In the past, the treatment of congenital heart disease mainly relied on surgical correction, which has wide indications and high technical maturity, but there are defects such as the need for extracorporeal circulation, large surgical trauma, and the legacy of surgical scars. In the past two decades, interventional treatment of congenital heart disease has achieved satisfactory results, and is popular among patients and medical personnel because it does not require extracorporeal circulation, is less traumatic, and has fast postoperative recovery. Epidemiological data show that simple cardiac malformations such as atrial septal defect, ventricular septal defect and patent ductus arteriosus account for about 70%-80% of all congenital heart diseases, and most of them can be cured by interventional methods. However, interventional treatment requires peripheral vascular puncture and placement techniques, and the small size of the child significantly increases the incidence of complications such as vascular injury, which limits its use.  Surgical correction of precardiac disease using thoracoscopy and hybrid surgical treatment (surgery combined with interventional treatment) provide more options for patients with precardiac disease and have become a hot spot in the treatment of precardiac disease in the last decade. The hybrid surgical treatment is mainly applied in the field of treatment of some complex precardiac diseases, which can reduce the difficulty and risk of surgery, shorten the time of extracorporeal circulation, reduce the number of open-heart surgery, effectively improve the treatment effect, and increasingly show its superiority.  Compared with the western developed countries, China’s precocious disease treatment work started late, the lack of professional physician team, and there is an uneven geographical development; due to the lag in the development of China’s previous period of precocious disease treatment technology, a considerable part of the accumulation of patients with precocious disease missed the best time for treatment, or even completely lost the time of cure; we should also see that, at present, the treatment of precocious disease in many places in China is not by We should also see that many places in China are currently treated by doctors who do not have systematic expertise in precocious diseases. At present, with the rapid promotion and popularization of the treatment technology of precocious heart disease at home and abroad, China is still facing the phenomenon of “three big and three shortages” in the field of treatment of precocious heart disease, including the large number of patients, large differences in conditions, large span of disease, as well as the lack of professional physicians, the lack of industry norms and the lack of popular science education. In spite of the increasing attention to precardiac disease in China, in the face of the rapid emergence of new technologies for the treatment of precardiac disease and the fragmented practice of doctors specializing in the surgical and interventional treatment of precardiac disease, most non-precardiac doctors still adhere to the traditional concept of “early detection, early diagnosis and early treatment” in a general way, without differentiating between different types and conditions. The phenomenon of “internal medicine can be plugged if they see a hole, surgery can be mended if they see a hole” occurs from time to time. Happily, in the last decade or so, under the correct leadership and attention of the government, the domestic academic community has relied on the Cardiovascular Branch of the Chinese Medical Association and the Cardiovascular Physicians Branch of the Chinese Medical Association to carry out a series of activities for the treatment of precocious heart disease, along with various health education activities on the theme of prevention and treatment of precocious heart disease, tens of thousands of children with precocious heart disease have been treated, which has greatly promoted the diagnosis and treatment of precocious heart disease in China. The author believes that in order to further improve the quality of life of our country, we need to improve the quality of life of our children. The author believes that in order to further improve the overall level of treatment of precocious heart disease in China, it is necessary to further standardize the treatment of precocious heart disease in China from the following aspects.  1.Construct a joint consultation system of cardiac physicians and surgeons oriented to the precocious disease chain. With reference to the international advanced experience, a substantial cardiac center operation mode should be gradually piloted and implemented in the tertiary hospitals, and a joint consultation system should be constructed and implemented with the joint participation of cardiovascular physicians and surgeons to comprehensively evaluate the physiological and pathological parameters of children with precocious heart disease and formulate personalized treatment plans containing the timing and treatment methods of precocious heart disease to ensure the interests of patients Maximize the benefits for patients.  At present, in the majority of medical institutions in China, the medical and surgical departments of preconditioning adopt a relatively independent and fragmented mode of operation, and there is a lack of proper communication and communication mechanisms between disciplines. In some difficult cases, there will be mutual excuses between medical and surgical departments, which will undoubtedly affect the correct treatment of the child and deviate from the people-oriented principle.  In recent years, some large tertiary hospitals in China have set up cardiac centers with cardiovascular disease-oriented disease chain model, which I believe meets the needs of the times and represents an advanced production relationship, but due to the constraints of many factors such as practice policy level, personnel knowledge level and technical level, most hospitals have not fundamentally broken through the traditional medical and surgical model of cardiac medicine and surgery each working on its own in the past. However, due to the constraints of practice policy, staff knowledge and technology, most hospitals have not fundamentally broken through the traditional medical model of separate cardiac medical and surgical practices, and have not been able to bring about the expected productivity gains. In order to change this situation, on the one hand, the government should pay attention to and support this work from the policy level, and increase the government’s supervision of this work; on the other hand, we should fully rely on the academic resources of professional societies and associations, deepen and promote the exchange and integration of disciplines, and fully implement the training, assessment, qualification and legal practice of professional and technical personnel guided by the precardiac disease chain to accelerate the cultivation of professional talents; third In the third aspect, a nationwide comprehensive database system covering interventional treatment and surgical correction of precardiac disease should be launched and implemented, and with reference to the guidelines for the treatment of precardiac disease, industry experts should be organized to conduct random checks and assessments of the treatment of precardiac disease in various medical institutions on a regular basis.  At present, the Ministry of Health has taken the lead in China to initially establish and improve the standardized training program for specialist cardiovascular physicians and cardiovascular interventional physicians, establish a national database system for interventional treatment of cardiovascular diseases, rely on the academic resources of professional societies and physicians’ associations in cardiovascular medicine, and make use of regular quality control meetings of different scopes, etc., to increase the prominent problems in the field of interventional treatment of cardiovascular diseases and However, the database system fails to include data of patients with precardiac disease treated by cardiovascular surgery.  2. Further standardize the selection of various surgical indications: To further standardize the indications for the treatment of different types of precardiac diseases to prevent the expansion and abuse of indications, in view of the current status of the treatment of precardiac diseases in China.  Some precardiac diseases have no adverse effects on hemodynamics and growth and development of the child, and generally do not require treatment. For example, the foramen ovale is a vital channel necessary for fetal development. Before birth, the umbilical vein from the mother enters the left side of the fetal heart along this channel, and then, through the arterial system of the fetal circulation, it is distributed throughout the body to provide the oxygen and nutrients required for fetal development. Foramen ovale is by far the most common congenital heart anomaly in adults, with approximately 1 in 4 people in the normal population being detected with this condition. The vast majority of patients with patent foramen ovale do not present clinically throughout their lives, and therefore are not clinically significant and do not require management. Recent studies have shown a strong association between patent foramen ovale and patients with unexplained stroke and vascular migraine, and closing open foramina in high-risk populations is expected to reduce the incidence of patients.  In addition, tiny patent ductus arteriosus and ventricular septal defects less than 2 mm in diameter and atrial septal defects less than 5-8 mm in diameter generally do not cause cardiac hemodynamic changes and do not affect the growth and development of the affected children, and are usually followed up with close observation without the need for urgent blocking and surgical treatment.  Some precardiac diseases have a high tendency to heal on their own after birth and can be followed up for a period of time under close observation before deciding whether treatment is needed. It has been reported in the literature that most of the ventricular septal defects at birth will heal on their own within 5 years of age, especially the myocardial (80%) and perimembranous septal defects (40%) have a higher chance of healing on their own. For these patients with a tendency to heal on their own, if the secondary damage caused by hemodynamic changes is not obvious at the time of consultation, follow-up under close observation can be considered to give the children a chance to heal on their own rather than treating them prematurely.  Many congenital heart diseases with left-to-right shunts develop to a certain stage, with the increase of right heart load and pulmonary hypertension, the pulmonary vascular resistance increases progressively, and bi-directional or even right-to-left shunts appear, forming the so-called Eisenmenger’s syndrome, even if interventional or surgical procedures are performed to correct the primary cardiac malformation in these patients, it still cannot improve the prognosis of patients, or even worsen the prognosis of patients, so it is not recommended to perform corrective surgery in these patients. Therefore, corrective treatment is not recommended for these patients.  3, to further standardize the timing of orthopedic treatment: to stop the phenomenon of premature treatment when treatment should be late and delayed treatment when treatment should be early. The best time to operate for congenital heart disease is a question that is the easiest to answer and the most difficult to answer. Theoretically, after the diagnosis of congenital heart disease is established, correction should be performed as early as possible, so as to avoid the impact of heart malformation on the growth and development of the child, and to reduce the mental and economic burden of the parents and family of the child. However, objectively speaking, the newborn’s heart is only the size of a pigeon egg, and the smaller the child is, the less tolerant it is to surgery, the more difficult it is to perform surgery, and the higher the risk of complications; while interventional treatment requires peripheral vascular puncture and placement, and the small size of the child restricts the development of interventional surgery because of the thin diameter of the peripheral vasculature; in addition, performing either surgical or interventional treatment for precardiac disease requires a high level of surgical skill. In addition, the performance of both surgical and interventional procedures for precordial disease is highly demanding, requiring skillful operators and extensive practical experience. In this regard, the timing of correction depends on the location and severity of the heart malformation and its possible impact on the prognosis of the patient; the impact of the disease on the heart structure, function and growth of the child; the tolerance of the child to surgery and the possible impact on learning; and the equipment and technical capabilities of the hospital. As can be seen, the optimal timing of treatment for congenital heart disease is different and cannot be generalized. Generally, cyanotic congenital heart disease should be corrected as early as possible to save the child’s life; for non-cyanotic congenital heart disease, close observation can be made before determining the timing of correction.  As mentioned above, some physiological channels during fetal life will close naturally within a certain period after birth, so there is no need to intervene prematurely. In general, the ductus arteriosus begins to close functionally 10-15 hours after birth, and most of them will close permanently between 2 months and 1 year of age; those who remain unoccluded after 1 year of age are considered to have an unoccluded ductus arteriosus. Of course, if the ductus arteriosus does not close three months after birth, and the diameter is more than 3-4 mm, the possibility of self-closing is very low, and if this causes significant hemodynamic changes, or even adversely affects the feeding and growth of the child, then timely correction is needed. At birth, with the first cry, the pulmonary circulation resistance decreases and the left atrial pressure rises, causing a functional closure of the left primary septum partially adhering to the right secondary septum, which reaches anatomical closure within 1 year. If the foramen ovale remains unclosed in children >3 years of age, it is called foramen ovale nonocclusion. The vast majority of unclosed foramina are not clinically significant and do not require treatment; individual cases of unclosed foramina that require correction should be re-intervened at least after 3-5 years of age.  Currently, internationally, there is a lack of guidelines or consensus guiding the timing of surgical correction for patients with precordial disease, which is generally determined according to the following principles: 1. Emergency surgery (corrective surgery within 2 days to 2 weeks after birth): For life-threatening cardiac malformations, efforts should be made to perform surgical treatment within 2-10 days after birth to save the child’s life. If a child with complete transposition of the great arteries with intact septum is not operated in time, the child will die early, and most of these children can only be operated within two weeks after birth to correct the anatomy (transposition of the great arteries).  2. Early surgery (corrective surgery within 6-12 months after birth): For cyanotic precordial disease (such as Tetralogy of Fallot), if the child has frequent episodes of severe hypoxia, surgery should be performed as early as possible to avoid brain damage caused by hypoxia. If hypoxia is not significant, surgical correction can be done after 6-12 months of life. For huge atrial septal defect, ventricular septal defect, atrioventricular canal malformation, arteriovenous catheter failure and complete pulmonary vein ectopic drainage, etc., children with recurrent pulmonary infections, heart failure and developmental arrest can be treated surgically within 3-6 months after birth; some children without the above symptoms but with obvious heart enlargement and a tendency to develop severe pulmonary hypertension should also be operated early, preferably within 6-12 months after birth The best time for surgery is within 6-12 months after birth.  3. Elective surgery (corrective surgery at the age of 2-5 years after birth) For some types of precardiac disease, if the child’s life is not endangered, elective surgery can be performed, but in order not to affect the child’s schooling and development, it is advisable to operate before school age (2-5 years old).  4.Other. The timing of surgical treatment for some precardiac diseases has its own peculiarities, such as the right ventricular double outlet and other deformities that need to be corrected with extracardiac conduits, or some complex deformities that require full cavity pulmonary artery connection surgery, the child is required to undergo surgery after the age of 3-5.  Interventional treatment for precardiac disease is commonly used in patients with simple cardiovascular malformations, such as pulmonary valve stenosis, simple secondary foramen ovale septal defect, ventricular septal defect and patent ductus arteriosus, which account for about the majority of children with precardiac disease (more than 70%). In terms of the timing of treatment, domestic guidelines for the treatment of precocious heart disease generally recommend that the age of treatment for patent ductus arteriosus be limited to 6 months or older, for atrial septal defect to 1.5 years or older, and for ventricular septal defect to 2.5-3 years or older. If a child with precordial disease has severe hemodynamic changes, pulmonary hypertension, and affects the feeding and growth of the child due to a large defect before the above age, the child is recommended to receive surgical treatment as early as possible.  4, to further standardize the choice of corrective treatment for precordial disease: At present, there are two options for the treatment of precordial disease: medical interventional techniques and surgical procedures. Both treatment methods are mature technologies, different treatment methods, in the selection of indications and timing are also slightly different. Generally speaking, cyanotic precardiac disease and complex malformations require surgical correction, while simple cardiac malformations and combined malformations (ductus arteriosus with atrial septal defect or ventricular septal defect, atrial septal defect with pulmonary valve stenosis, etc.) can often be treated by interventional methods, so most simple cardiac malformations can technically be treated both surgically and by interventional treatment. However, in reality, we should see that due to the inherent characteristics of surgical and interventional techniques, there are differences in the efficacy and safety of surgical or interventional treatment for a significant number of precardiac diseases, that is, some precardiac diseases are better treated with interventional treatment at a particular time, while others are better treated with surgical correction, and vice versa. For example, for the vast majority of ductus arteriosus, both surgical and interventional treatment are more effective and safe, but in the case of adult-detected ductus arteriosus with moderate to severe pulmonary hypertension, interventional treatment may be more appropriate because factors such as ductus arteriosus fibrosus and calcification affect the effectiveness and safety of surgical ligation; however, in the case of giant window ductus arteriosus, surgical treatment would be more effective. In addition, interventional techniques have shown better recent and mid-term results in the treatment of secondary foramen ovale atrial septal defects, and they have the potential to replace traditional surgical correction. Some atrial septal defects with a defect diameter of more than 30 mm or even larger have been reported to be successfully treated with interventional therapy, and the long-term effects of this mega blocking umbrella and the secondary inter-umbrella mechanized thrombus on atrial septal function are worrying. The author believes that in the face of precardiac disease, we have at least two tools: interventional therapy and surgery. It is not enough to simply view the use of a certain technique to be able to correct a certain malformation, but also to compare it with other treatment methods in terms of safety and effectiveness, to find out the best treatment for this type of precardiac disease is our goal. As a specialist in precordial disease, the physician should have a more comprehensive understanding of these two technologies and use them rationally, without prejudice.  In view of the requirements of peripheral vascular conditions for interventional treatment, interventional treatment can be considered for some simple malformations of precordial disease (pulmonary valve stenosis, patent ductus arteriosus, atrial septal defect, ventricular septal defect, etc.) if it is determined that the malformation does not have the possibility of self-healing, the peripheral vascular conditions (age) allow it, and the structural features of the lesion are suitable. However, surgical treatment should be considered if one of the following conditions exists in the case of the above-mentioned simple malformations: 1) the lesion is of large caliber, with significant hemodynamic changes that affect the child’s cardiac function and lead to pulmonary hypertension; 2) the child’s growth is suspected to be arrested due to the lesion; 3) the anatomical features of the lesion are not suitable for intervention; 4) the peripheral vascular conditions (age) do not allow it, etc. As for cyanotic preconditioning, surgical correction is generally required, while some simple malformations (such as patent ductus arteriosus, atrial and ventricular septal defects, lateral branches of the body-pulmonary circulation, etc.) associated with cyanotic preconditioning and complex preconditioning can be treated by intervention at the appropriate time, the latter being essentially the domain of hybrid surgery.