With the introduction of various advanced instruments, especially echocardiography, congenital heart disease (congenital heart disease) is very easy to detect, however, the treatment is more complicated. At present, congenital heart disease is mainly treated by surgery, including interventional (minimally invasive treatment) surgery and surgical open-heart surgery, but both have certain requirements for age, here is a brief introduction to the best timing of congenital heart disease surgery. When it comes to the timing of surgery, in fact, the medical community has been exploring it. For some diseases, the timing of treatment varies with the advancement of understanding and the emergence of new technologies, but it depends more on the type of lesion, the severity of the disease and the accompanying complications, so the so-called optimal timing of surgery is only for typical cases.
I. Congenital heart diseases that can be treated by intervention
1.Atrial septal defect (atrial defect for short, excluding single atrium and atrial septal defect): theoretically, atrial septal defect before adulthood does not cause severe pulmonary hypertension, especially in small defects, so the age requirement for surgery is relatively relaxed. If a patient with atrial septal defect is combined with severe pulmonary hypertension at an early age, especially if the defect is small, it indicates that this pulmonary hypertension is related to the patient’s pulmonary vascular disease itself and not so much to the defect, and preserving the atrial septal defect at this time is instead beneficial for the patient to live longer. In general, for both interventional and surgical procedures, it is more appropriate to operate on atrial septal defects before school age, unless other complications, such as heart failure and recurrent infections, require early surgery. Surgery at too young an age is risky, and interventional treatment is usually required beyond 3 weeks of age. Complications increase with age, with the main complications being pulmonary hypertension, right heart failure and atrial fibrillation. The incidence of atrial fibrillation (referred to as atrial fibrillation), which can cause many serious complications such as cerebral embolism, increases significantly if the procedure is performed after the age of 25 years (or 40 years according to some). In addition, the defect itself can also cause complications such as migraine and cerebral embolism. Therefore, even in the absence of pulmonary hypertension and right heart failure, surgery is recommended before the age of 25. In some children with large defects who wish to undergo interventional procedures, an older age is required, otherwise a blocker cannot be implanted, but the exact timing of this requirement can only be determined by regular ultrasound examinations. It should be noted that if the child is still not suitable for interventional treatment after the childhood period, then basically only surgery can be considered and one should not wait any longer.
Ventricular septal defect (ventricular septal defect): the best time for surgery is similar to that of atrial septal defect, i.e. before school age, when interventional and surgical procedures can be chosen according to the situation. If interventional treatment is desired, it is more appropriate after at least 3 weeks of age. It is important to note that the timing of ventricular septal defect surgery depends on the size of the defect and the severity of pulmonary hypertension. If pulmonary hypertension is present at a very young age, the defect is large and not suitable for interventional treatment, and one should not wait for interventional treatment to avoid losing the timing of surgery, but should perform surgery within 1-2 weeks of age. Small ventricular septal defects have no significant impact on cardiac function and development of the child, and both the myocardial and membranous defects have the possibility of automatic closure within 6 weeks of age, so the time of surgery can be appropriately postponed, and both types of ventricular septal defects are suitable for interventional treatment. For pulmonary subvalvular and post-tricuspid valve defects, regardless of size, are not suitable for interventional treatment, and surgery should be performed in a timely manner, otherwise valve function may be affected, but there is no specific time requirement, and patients without pulmonary hypertension can only be roughly considered more suitable in childhood.
3, unclosed ductus arteriosus: theoretically, if there are no accompanying symptoms, such as heart failure and pulmonary hypertension, there is no clear requirement for the age of unclosed ductus arteriosus surgery, but the actual situation is quite the opposite, unclosed ductus arteriosus can easily cause heart failure, intrapulmonary infection, and affect growth and development, and pulmonary hypertension has a high incidence and early onset, so once detected, early treatment is recommended. Interventional treatment has now replaced surgical open-heart surgery as the treatment of choice for patent ductus arteriosus. Unlike interventions for atrial septal defect and ventricular septal defect, which require an age of more than 3 years, interventions for patent ductus arteriosus can be performed as early as 3 months after birth.
4, pulmonary stenosis: There is no clear age requirement for surgery, and the timing of surgery depends on the degree of stenosis. In some children, severe pulmonary stenosis is present after birth and should be operated immediately, otherwise it may be life-threatening; the smallest patient with pulmonary stenosis treated with intervention in our unit was 15 days after birth. It is important to understand that children with such severe pulmonary stenosis are very dangerous, both interventionally and surgically, and must be psychologically prepared, but relatively speaking, interventional treatment is safer and can start with balloon catheter dilation to relieve symptoms, and if stenosis occurs again at an older age, interventional treatment can be performed again, usually with very good results.
5.Aortic stenosis: There is no age limit, and severe stenosis in childhood is relatively rare. The timing of surgery depends on the degree of stenosis and should be considered for the following indications.
(1) Symptoms of angina or syncope.
(2) The presence of left ventricular hypertrophy on the electrocardiogram, indicating a corresponding change in the left ventricle.
(3) Transaortic pressure step difference >60 mmHg.
Interventional treatment, i.e., balloon dilatation of the aortic valve, is preferred in patients who are not very old, whereas surgical procedures are mainly performed in patients who are older and not suitable for interventional treatment. Such treatment options are not based on treatment outcomes, but rather on age and late treatment considerations. Usually only after adulthood or near adulthood the aortic annulus is fully developed and the implanted aortic valve does not develop to cause relative stenosis of the implanted valve, in addition, the implanted valve also has a service life problem, and the second valve replacement surgery is still a challenge.
6, aortic stenosis: similar to valve stenosis, there is no specific age requirement, and the timing of surgery depends on the degree of stenosis and the patient’s symptoms, usually the pressure step difference across the stenosis >20 mmHg should be considered for surgery. The surgical approach depends on the age, the degree of narrowing and the length of the narrowed segment. Usually, balloon angioplasty is used under 14 years of age, and stenting is advocated over 14 years of age; simple membranous narrowing can be treated by balloon dilation only, while tubular narrowing, or combined with arterial catheter failure, is treated by overlapping stent implantation; if the narrowing location is high and close to other large vessel branches, only surgical treatment can be performed.
7, coronary artery fistula: a rare malformation, although theoretically a variety of complications can occur and immediate treatment is advocated once detected, but in practice these complications are uncommon. The decision to operate depends on the presence or absence of symptoms and the size of the fistula. Small coronary artery fistulas (less than 3 mm) do not require treatment. Interventional treatment is preferred, but currently there is no clear age requirement and the procedure can be performed on anyone from children to adults.
Congenital heart disease that can only be treated surgically at present
The following complex congenital heart diseases cannot be treated by intervention at present and can only be treated by surgery, so for your convenience, they are listed together.
1, atrioventricular septal defect, including single atrium, partial atrioventricular septal defect and complete atrioventricular septal defect three, including single atrium and partial atrioventricular septal defect surgery timing depends on the severity of pulmonary hypertension, usually 1-2 weeks of age surgery is better; complete atrioventricular septal defect in the past that should be operated within 6 months after birth, at present, it is believed that 3-4 months after birth should be operated.
The timing of surgery depends on the patient’s symptoms. Those with hypoxic attacks (manifested as blue lips and breathing difficulties after crying) should be operated immediately; those with mild symptoms and no hypoxic attacks used to think that surgery can be performed before school age, but in recent years, early surgery is advocated, and even surgery should be performed within 1 week of age in order to obtain the best long-term results.
3.Aortic transposition, including corrective aortic transposition and complete aortic transposition, the timing of surgery varies greatly and the age requirement is strict. Complete transposition of the great arteries should usually be operated within 2 weeks after birth, with large ventricular septal defect can be delayed until 2 months after birth. For corrected transposition of the great arteries, surgery is not necessary if there is no combined malformation. Combined ventricular septal defect without pulmonary stenosis should be operated within 6 months after birth, and surgery with pulmonary stenosis is appropriate at 2-3 years of age. If the corrected transposition of the great arteries is combined with only a small ventricular septal defect or pulmonary stenosis, interventional treatment can also be considered, and the timing of interventional treatment is the same as that of common ventricular septal defect and pulmonary stenosis.
4. Double outlet of right ventricle depends on the presence or absence of pulmonary stenosis. If there is pulmonary stenosis, surgery is appropriate at 4-5 weeks of age, but if the stenosis is severe, early surgery is needed to relieve the symptoms, and surgery without pulmonary stenosis should be performed within 1 week of age to avoid losing the opportunity of surgery due to pulmonary hypertension. In addition, there is a right ventricular double outlet called Taussig-Bing malformation, which is manifested by the pulmonary artery riding on the ventricular septum and requires surgery 3-6 months after birth.
5.Pulmonary vein ectopic connection (also called pulmonary vein ectopic drainage) is divided into two types: partial type and complete type, of which partial type is similar to simple atrial septal defect and depends mainly on the presence of pulmonary hypertension; complete type should be operated as early as possible if there is pulmonary vein obstruction or heart failure, and those without obstruction and heart failure should be operated before pulmonary hypertension is severe, usually at 1-2 weeks of age.
6, tricuspid atresia, the mortality rate is very high, the surgery is very complicated, the timing is difficult to grasp, the approximate essentials are as follows: if there is pulmonary atresia (no pulmonary valve) or no pulmonary stenosis (manifested by a significant increase in pulmonary blood) should be operated as early as possible, currently it is believed that the surgery should be performed within 1 month after birth. If pulmonary stenosis is present, but not atresia, the time window is relatively large and surgery can be performed between 2 and 15 weeks of age, with 2-6 weeks of age being optimal.
7. There are four types of common arterial trunks (also called permanent arterial trunks), of which types I-III all have pulmonary arteries and type IV has no true pulmonary artery. The first 3 types were previously considered to be best operated at 3-4 weeks of age, but recently it is considered best to operate within 1 week of age, otherwise the opportunity to operate may be lost due to pulmonary hypertension. In type IV common arterial trunk, it is difficult to operate because there is no true pulmonary artery, and the possibility of surgery depends on the degree of development of collateral vessels.
There are more types of congenital heart disease and all others are relatively rare, and the timing of surgery is related to the severity of the disease, so I will not dwell on them here.