Abstract: OBJECTIVE: To retrospectively summarize the experience of surgical treatment of aortic constriction combined with intracardiac malformations in infants and children. METHODS: From January 2000 to December 2006, 84 infants and children with aortic constriction combined with intracardiac malformations underwent surgical treatment, aged 1 month to 3 years (mean 13.5 months) and weighing 3.3 kg to 15 kg (mean 7.3 kg). 12 of the 84 cases were combined with complex intracardiac malformations Of the 84 cases, 12 cases were combined with complex intracardiac malformations, 72 cases were combined with ventricular septal defect and other simple intracardiac malformations, and 23 cases were combined with arch dysplasia. The first stage surgery was performed in 62 cases, 49 cases were treated with both aortic stenosis and intracardiac malformation by median opening, 13 cases were treated with left-sided opening and intracardiac malformation was repaired by median opening, and 22 cases were operated in stages. The surgical procedures for aortic constriction included patch plication in 42 cases, resection end-to-end anastomosis in 30 cases, subclavian artery reversal in 6 cases, vascular bypass in 3 cases, and balloon dilation in 1 case. Among 49 cases of median incision stage I surgery, selective cerebral perfusion plus inferior stop circulation was applied in 43 cases, systemic low-flow perfusion in 4 cases, and deep hypothermic stop circulation in 2 cases. Results There were 8 cases of in-hospital death, with a mortality rate of 9.5%. Three of the deceased cases were misdiagnosed. Conclusion: The surgical treatment of aortic constriction combined with intracardiac malformation in infants and children can achieve good near-term results, and the vast majority of children can be operated in a median stage, and selective cerebral perfusion and inferior hemiplegic stop circulation can provide effective protection of the brain and vital organs. Keywords: aortic constriction; cardiac defect; surgery Surgical treatment of aortic coarctation with intracardiac anomaly in infants and toddlers Abstract: Objective To review the To review the experience in repair of aortic coarctation with intracardiac anomaly in infants and toddlers retrospectively. Methods: From Jan 2000 to Dec 2006, 84 infants and children diagnosed as aortic coarctation with intracardiac anomaly in infants and toddlers were treated with aortic coarctation. The mean age of the patients was 13.5 months, ranging from 1 month to 3 years. Mean body weight was 7.3 kilogram, from 3.3 kg to 15 kg. Among 84 cases of aortic coarctation, twelve of them complicated Among 84 cases of aortic coarctation, twelve of them complicated with complex intracardiac anomaly, seventy-two of them with ventricular septal defect and other simple anomaly, twenty-one of them had hypoplasia of the aortic arch . the aortic arch . Median sternotomy was used to simultaneously repair coarctation and intracardiac defect in 49 cases. Thoracotomy and median sternotomy were applied to repair aortic coarctation and intracardiac anomaly respectively in 13 cases. Twenty- two cases had staged repair. Operational techniques for aortic coarctation include 42 cases of patch aortoplasty, 30 cases of resection and end-to-end anastomosis, In all 49 cases of one-stage operation through median sternotomy, selective cerclage was performed. sternotomy, selective cerebral perfusion was used in 43 patients, deep hypothermia low flow was applied in 4cases, deep hypothermia circulatory arrest The mortality is 9.5%. Among 8 deaths, 3 cases were misdiagnosed. Conclusion: Surgeries for aortic coarctation with intracardiac anomaly have satisfactory short-term One-stage repair through median sternotomy can be applied to most of the cases. Selective cerebral perfusion with deep hypothermia and circulatory arrest Selective cerebral perfusion with deep hypothermia and circulatory arrest in lower body can protect the brain and other vital organs. key words：Aortic coarctation; Cardiac defect; Surgery Aortic constriction is a congenital stenosis at the beginning of the thoracic descending aorta, usually near the junction of the ductus arteriosus and the aorta. In severe aortic constriction, the aortic lumen at the site of stenosis may be nearly atretched, but there is continuity in the aortic wall above and below the stenosis. Infantile aortic stenosis differs significantly from adult aortic stenosis in terms of clinical presentation and surgical approach, and is often combined with ventricular septal defect or other intracardiac malformations, with early onset of symptoms of cardiac insufficiency and pulmonary hypertension, requiring early surgical correction [1]. From January 2000 to December 2006, a total of 84 children under 3 years of age with aortic constriction combined with intracardiac malformations were admitted to our hospital, and a retrospective analysis of their clinical manifestations, diagnostic methods, surgical options, and recent outcomes is presented. I. Clinical data There were 84 children in the group, including 52 males and 32 females. The average age was 13.5 months, including 56 infants and 28 toddlers, with an average weight of 7.3 kg (3.3-15 kg). Clinical examination revealed a difference in blood pressure between upper and lower extremities (systolic upper extremity was higher than lower extremity by more than 20 mmhg) in 60 cases, accounting for 71%. All cases were screened by echocardiography, 56 of them were then examined by UFCT, 23 cases were confirmed by cardiovascular angiography, and 6 cases of aortic constriction were missed preoperatively. Among the 84 children with aortic constriction, 60 cases (71%) had simple constriction and 24 cases (29%) had constriction with arch dysplasia. Among them, there were 5 cases of right ventricular double outlet, 2 cases of complete transposition of the great arteries, and 1 case each of corrected transposition of the great arteries, tetralogy of Fallot, complete endocardial cushion defect, single ventricle, and tricuspid atresia. Surgical method 1. One-stage surgery with median incision: 49 children were operated in this way. The aortic arch was opened through a median incision, and the descending part of the aortic arch was freed, and aortic constriction and intracardiac malformation were corrected simultaneously under extracorporeal circulation. Selective cerebral perfusion plus inferior hemiplegic stop circulation was used in 43 cases, ascending aorta and femoral artery were cannulated for deep hypothermic low-flow perfusion in 4 cases, and deep hypothermic stop circulation was used in 2 cases. The operation of selective cerebral perfusion plus inferior hemiplegia was as follows: after general anesthesia, the chest was opened medially, the thymus was subtotally removed, the three major brachial vessels and the arterial catheter were fully freed, and the blocking band was set. Establish extracorporeal circulation, drainage through the upper and lower vena cava, and block the arterial catheter as soon as the transfer is started. Slowly and evenly lower the temperature, and then fully free the descending part of the aortic arch during the cooling process, and the descending aorta is generally free to the first intercostal vessel far away, paying attention to avoid injury to the recurrent laryngeal nerve and septal nerve. When the rectal temperature drops below 20 degrees, the ascending aortic perfusion tube is inserted into the unnamed artery, the three major head arm vessels are blocked, selective cerebral perfusion and inferior hemiplegia stop circulation are started, the cerebral perfusion flow is controlled at 15-20 ml/kg.min, the arterial catheter is cut off, and the pulmonary artery is sutured laterally. The distal descending aorta is blocked on the clamp, the arterial catheter and narrowed aorta are completely removed, and the proximal vessels are end-to-end anastomosed or patched for angioplasty. After completion of aortoplasty, the distal blocking clamp of the descending aorta is opened for adequate venting, and the unnamed artery is intra-arterially cannulated to withdraw the ascending aorta and open the three major capillary vessels to restore normal systemic perfusion. Correction of intracardiac malformations is accomplished during cooling or rewarming depending on the specific intraoperative situation. The mean time of selective cerebral perfusion and lower body stopping circulation using the median incision phase I surgery was 49 minutes, the time of extracorporeal circulation was 172 minutes, and the time of aortic block was 90 minutes 2. Left posterior plus median incision phase I surgery: 13 children were operated using this procedure. Under general anesthesia, the left posterior lateral incision was opened, the descending part of the aortic arch and intercostal vessels were fully freed, the arterial catheter was cut, and the descending part of the aortic arch was shaped under narrowing and blocking at both ends, and the intracardiac malformation was corrected immediately after the median open-heart hypothermic extracorporeal circulation. In 20 cases, descending aortic arch angioplasty was performed through a left posterior external incision under general anesthesia, or pulmonary artery annuloplasty was performed at the same time, and then intracardiac malformation was corrected in the second stage. 2 cases had pulmonary artery annuloplasty first to reduce pulmonary artery pressure, and then the aortic lesion and intracardiac malformation were corrected in the second stage. 13 of the 22 patients with staged surgery completed the second stage. The time interval of the second surgery was 3 days-3 years. 4. Surgical methods of aortoplasty and correction of intracardiac malformations: Different aortoplasty methods were adopted according to the age of the child, the site and severity of the narrowing, and the extent of the narrowing. Among the 82 children treated for aortic narrowing, 42 cases were treated with patching, 30 cases with end-to-end anastomosis, 6 cases with subclavian artery reversal, 3 cases with vascular bypass, and 1 case with balloon dilation. Aortoplasty materials included homograft in 32 cases, artificial vessel in 8 cases, autologous pericardium in 4 cases, and heterograft in 1 case. Correction of intracardiac malformations included repair of ventricular septal defect in 68 cases, transposition of the great arteries in 2 cases, correction of double outlet of the right ventricle in 2 cases, repair of complete endocardial cushion defect, radical treatment of tetralogy of Fallot, and Damus-kaye-stansel surgery in 1 case each. Results There were 8 deaths in the whole group, with a mortality rate of 9.5%. Among them, there were 7 cases of aortic constriction combined with simple malformation and 1 case of complex malformation. Three of the deaths were due to preoperative omission of aortic constriction. 5 of the 8 deaths were due to respiratory failure, and 1 death each was due to severe infection, postoperative low cardiac output, and gastrointestinal hemorrhage. Important complications occurred in 20 cases, including 15 cases of respiratory complications, 2 cases of low cardiac output, and 1 case each of brain damage, left vocal cord paralysis, renal failure, gastrointestinal hemorrhage, and hemolysis. Nine patients with staged surgery did not complete the second-stage surgery, including two cases of death after aortic stenosis correction, four cases of aortic stenosis without further intracardiac malformation repair, two cases of aortic stenosis combined with intracardiac malformation preceded by pulmonary artery annuloplasty without further second-stage surgery, and one case of aortic stenosis combined with corrected transposition of the great arteries preceded by aortoplasty plus pulmonary artery annuloplasty without further second-stage surgery. All children with aortic stenosis were discharged from the hospital with no further aortic stenosis or aneurysm on review echocardiogram. Discussion Aortic constriction is classified according to the traditional classification into symptomatic infantile type (pre-catheter type) and asymptomatic adult type (post-catheter type) [2]. The two types are different in terms of clinical manifestations and surgical approaches, and this group of cases only includes infants and children under the age of 3. Clinical practice has shown that the stenosis of infantile aortic constriction can also be located in the distal part of the ductus arteriosus, and this traditional classification method appears to lack practical significance. In fact, the site of aortic constriction is always located near the junction of the ductus arteriosus and the aorta, and the stenosis may be caused by the growth of smooth muscle in the wall of the ductus arteriosus into the aortic wall, resulting in thickening of the aortic wall and subsequent formation of fibrous tissue, resulting in narrowing of the aortic lumen [3]. Whether aortic narrowing is combined with arch dysplasia has important implications for the choice of surgical approach. In our group, 23 of 82 children with constriction combined with intracardiac malformation had combined arch dysplasia, accounting for 28%. In infants and children with aortic stenosis combined with intracardiac malformations, the most common one is ventricular septal defect, and the combination of other complex malformations is less common. In ventricular defect combined with aortic stenosis, the left-to-right intracardiac fractional flow is greatly increased because of the obstruction of left cardiac drainage, so the children have early heart enlargement and pulmonary congestion, and the clinical manifestations are recurrent pneumonia and heart failure, and most of them are accompanied by severe pulmonary hypertension. The pressure difference between the upper and lower extremities does not exist in all children, but it exists in 65 out of 93 cases in this group, accounting for 70%. The presence of differential pressure is related to the degree of pulmonary hypertension, the thickness of the arterial duct, and the formation of collateral vessels, and the diagnosis can be confirmed by CT or angiography. Compared to cardiovascular imaging, CT has the advantage of being noninvasive and less expensive, and can be the preferred means of confirming the diagnosis. Avoiding preoperative leakage is important to improve the success rate of surgery. In our group of 8 children who died, there were 3 cases of preoperative leakage of aortic lesions. In children with precordial disease, accurate measurement of upper and lower extremity blood pressure and extremity saturation is very important. Although not all patients with aortic constriction have differential upper and lower extremity pressure or differential cyanosis, an accurate examination can provide signs of possible aortic constriction. When CT is not yet a routine test for precordial disease, a thorough and detailed examination and echocardiography are the main tools to prevent missed aortic constriction. Early appearance of oliguria, anuria, and high blood pressure in the upper limbs after intracardiac malformation correction should alert for missed aortic constriction, and prompt further examination and reoperation. In addition to preoperative missed aortic lesions, respiratory failure is the main factor leading to postoperative death. Children with aortic constriction combined with ventricular septal defect, who have severe pulmonary congestion and recurrent pneumonia and heart failure before surgery, are prone to respiratory insufficiency after the blow of surgery. When possible, the preoperative cardiopulmonary function of the child should be adjusted to the optimal state by active medical treatment as much as possible, and the lungs and other important organs should be protected as much as possible intraoperatively to shorten the ischemic time, and the arterial catheter should be blocked in time after the start of extracorporeal circulation to prevent perfusion of the lung. Postoperative respiratory system treatment should be correct and timely to prevent and treat possible lung exudation, infection, pulmonary atelectasis, and pneumothorax. After the improvement of surgical and perfusion techniques and the accumulation of more experience in perioperative management, only 2 cases died in 44 operations after January 2005 in our group, with a mortality rate of 4.5%, which was significantly lower than before. There are still multiple options for the surgical approach to aortic constriction combined with intracardiac malformations, and the experience reported in the literature varies [4, 5, 6]. The choice of surgical approach is related to the type of combined malformation, the severity of the disease, and the age of the child. In adults and older children, or in children with extensive aortic narrowing, the aortic arch can be removed through a median incision because of the deep field and the difficulty of operation. In infants and young children, the operation of freeing the descending part of the aortic arch through the median incision is less difficult, and the majority of patients can be treated in one stage through the median incision. If the child has complex combined malformations or severe preoperative cardiac insufficiency or respiratory failure and is not expected to tolerate complex surgery, staged surgery may be an option. The aortic constriction can be corrected first, or the aortic constriction can be corrected together with pulmonary artery circumferential constriction, and then the intracardiac malformation can be corrected in a second stage after the child has recovered. The advantages of orthodontic phase I correction are obvious, especially for patients with aortic arch dysplasia. In our group, 49 of 84 children were treated with orthotropic one-stage orthodontic treatment, accounting for 58%; 44 cases were operated after January 2005, 33 cases were treated with orthotropic one-stage orthodontic treatment, accounting for 75%; 27 cases were operated after January 2006, 24 cases were treated with orthotropic one-stage orthodontic treatment, accounting for 89%. The orthodontic phase I correction is gradually becoming the preferred surgical approach. If possible, the narrowed section should be completely excised, and the arterial duct tissue on the aortic wall should be removed as much as possible, and the aortic arch, cephalic vessels and descending aorta should be fully freed. In our group of 79 cases of aortic constriction surgery, end-to-end anastomosis was used in 28 cases, accounting for 35%, and end-to-end anastomosis was used in 17 of 24 patients after January 2006, accounting for 70%, indicating that constrictive resection end-to-end anastomosis is suitable for most cases in infants and children. Although postoperative restenosis cannot be completely avoided regardless of the shaping method used, most of the current literature suggests that end-to-end anastomosis is effective in reducing the incidence of restenosis [7, 8]. In cases of severe and extensive narrowing, where the upper and lower marginal counterparts may be under too much tension after resection of the narrowed segment, patching or subclavian artery reversal shaping may be chosen. After dissection of the narrowed lumen, the abnormal proliferation of membranous and fibrous tissue is removed, and the material used for patch widening may be autologous pericardium, artificial vessel piece, homograft piece, or heterograft piece. If the aortic narrowing of the child is very long, a vascular bypass can be chosen to connect the distal and proximal ends of the narrowing with an artificial vessel, which was used in three cases in our group, all of which were 3 years old children. For patients with mild and limited narrowing, interventional balloon dilation is also a viable option. In this group, of the 56 patients who underwent median phase I surgery, 45 cases were treated with selective cerebral perfusion plus inferior hemiplegia stopping circulation technique. The ascending aortic cannula was transferred for cooling, and when the rectal temperature dropped below 20 degrees, the ascending aortic perfusion tube was inserted into the innominate artery, the blocking band of the three major cephalic arm vessels was tightened, and selective cerebral perfusion was started with flow control at 15-20 ml/kg. min, the upper blocking clamp blocked the distal descending aorta and opened the proximal aorta to facilitate the operation of aortoplasty. Compared with systemic stopping circulation, selective cerebral perfusion is more beneficial to the protection of brain tissue [9], and compared with systemic low-flow perfusion, femoral artery cannulation can be avoided and surgical trauma can be reduced. The mean time of selective cerebral perfusion and inferior hemiplegic stop circulation in our group of 45 cases was 49 minutes, and none of the children had brain damage, spinal cord ischemic damage or renal function damage, indicating that the nervous system and other organs are safe under proper hypothermia and conventional aortoplasty operation time, and selective cerebral perfusion with inferior hemiplegic stop circulation is a safe and effective perfusion technique, which has become a median stage I surgical correction of infantile aortic It has become the preferred perfusion method for the correction of aortic stenosis combined with intracardiac malformations in infants and children. It is concluded that surgical treatment of aortic constriction combined with intracardiac malformations in infants and children can have a good near-term outcome, and the vast majority of children can be treated with median-stage surgery, and selective cerebral perfusion and inferior hemiplegia can provide effective protection of the brain and vital organs. References 1, Kouchoukos NT et al. Coarctation of the aorta and interrupted aortic arch. In: Kouchoukos NT, Blackstone EH, Doty DB, et al, eds, Kirklin / Barratt – Boyes Cardiac Surgery, 3rd Edition. Philadelphia: Elsevier Science. 2003. 1315-1376 2. Sun LZ, Zheng J. Congenital aortic constriction. In: Wu, Qing-Yu, ed. Cardiac Surgery. Jinan: Shandong Science and Technology Press, 2003. 332-339 3. Jonas RA. Coarctation of the aorta. In: Jonas RA, DiNardo J, Laussen PC, et al, eds, Comprehensive surgical management of congenital heart disease. London: Arnold. 2004. 207-224 4. Hirooka K, Fraser CD. One-stage neonatal repair of complex aortic arch obstruction or Recent experience at Texas Children’s Hospital. Tex Heart Inst J, 1997, 24(4): 317-21 5. Sudarshan CD, Cochrane AD, Jun ZH, et al. Repair of the coarctation of the aorta. coarctation of the aorta in infants weighing less than 2 kilograms. Ann Thorac Surg, 2006, 82(1): 158-63 6. Murakami J, Kado H. Surgical treatment of coarctation and interrupted aortic arch complex in infants. Nippon Geka Gakkai Zasshi, 2001, 102(8): 566-72 7. Becker CL, Mavroudis C, Zias EA, et al. Repair of coarctation with resection and extended end-to-end anastomosis. Ann Thorac Surg, 1998, 66(4): 1365-70; discussion 1370-1 8. Thoson JD, Mulpur A, Guerrero R, et al. Outcome after extended arch repair for aortic coarctation. Heart, 2006, 92(1): 90-4 9. Takeuchi T, Harada Y, Morishima K, et al. . One-stage repair of interrupted aortic arch with selective cerebral perfusion. Kyobu Geka, 1998, 51(6): 443-7; discussion 447-50?