[Abstract] OBJECTIVE: To review and summarize the method and efficacy of one-stage correction of interrupted aortic arch via median sternotomy. METHODS: Among the 8 patients, 5 were male and 3 were female, the age at surgery was 1-11 years old, and the weight was 9-21 kg. 7 cases were type A and 1 case was type B. All 8 cases were combined with other cardiovascular malformations and severe pulmonary hypertension, and all were treated by median sternotomy in one stage. RESULTS: One case of death in the whole group died of severe postoperative low cardiac output syndrome and renal failure, and 6 cases were followed up for 6-14 years without late death. In one case, calcification and stenosis of the same vessel occurred 12 years after surgery, with a pressure difference of 12 kPa, and artificial vessel was performed again to connect the ascending aorta to the abdominal aortic bypass graft, with a pressure difference of 5.3 kPa at the aortic anastomosis in one case and 1.3-3.3 kPa at the aortic anastomosis in the rest, with NYHA class I cardiac function. Conclusion: One-stage surgical correction of aortic arch interruption via median sternotomy, especially direct aortic anastomosis, is easy to operate, well exposed, less traumatic, and conducive to postoperative recovery. Xu Jinxing, Department of Cardiovascular Surgery, Air Force General Hospital [Keywords] Surgery; Aorta, thorax; anastomosis, surgery [Citation] R654.3 [Ref ID] A [Article ID] 061145 One-stage repair of interrupted aortic arch through midline sternotomy XU Jin-xing, YANG Jun-min, YU Lu-fen, CHEN Yuan-heng, HOU Mai. (Department of Cardiovascular and Thoracic Surgery, Air Force General Hospital, Beijing 100036, China) [ABSTRACT] Objective: To summarize and review the experience with one-stage repair of interrupted aortic arch(IAA) through midline The operative age was 1-11 years and body weight was 9-21 kg, five patients were tape A IAA and 1 was tape B IAA. The operative age was 1-11 years and body weight was 9-21kg, five patients were tape A IAA and 1 was type B. All patients had associated cardiac anomalies and severe pulmonary hypertension. The 6 survivors were followed up from 0.5-14 years, and no late death. One patient required a bypass with artificial graft between ascending aorta and descending aortic artery who had aortic homograft calcification and One had residual pressure gradient 5.3 kPa. The other 4 patients had 1.3-3.3 The other 4 patients had 1.3-3.3 kPa, but their cardiac function was NYHA Ⅰ. Conclusion The method of one-stage repair of IAA especially with direct end-end anastomosis through midline sternotomy can achieve good exposure, less The method of one-stage repair of IAA especially with direct end-end anastomosis through midline sternotomy can achieve good exposure, less trauma and excellent recuperation. [KEYWORDS] surgical procedures, operative; aortic arch, thoracic; anastomosis, surgical Interrupted aortic arch (IAA) is a rare congenital IAA is a rare congenital malformation of the aortic arch, accounting for about 1% of congenital heart disease and reported as less than 1 per 1,000 in China. It is a complex combined malformation, difficult to diagnose and has a high mortality rate, with about 80% of children dying within a few months after birth [1]. The author summarizes the surgical experience of IAA in our hospital, which helps to improve the diagnosis and surgical efficacy. 1 Data and methods 1.1 Clinical data From March 1991 to April 2005, eight cases of IAA were admitted to our hospital, five males and three females, aged 1-11 years, weighing 9-21 kg, all with a history of recurrent respiratory infections before surgery, and six with a history of recurrent pneumonia and heart failure. There were 2 cases of differential cyanosis on physical examination. All patients had blood pressure and oxygen saturation measured in all extremities; blood pressure in the upper extremity was higher than that in the lower extremity in 5 cases, with no significant change in 3 cases, and oxygen saturation in the right upper extremity was higher than that in the lower extremity in 7 cases, with no significant change in 1 case. One case of IAA was diagnosed by echocardiography, and the rest were misdiagnosed. 7 cases were clearly diagnosed by right heart catheterization, and 1 case was found by intraoperative exploration, 7 cases of type A and 1 case of type B. All 8 patients were combined with other cardiovascular malformations (Table 1). Table 1 Diagnosis, surgical procedures and results of 8 cases with IAA Tab1 diagnosis, surgical procedures and results of 8 patients with IAA No. Age (y) Wt (kg) Diagnosis Surgical procedures CPB/Aortic Clamping Time (min) Results 1 3 12 IAA(A),VSD,PDA,PH(severe) PDA closure via PA,VSD repairing,ADB 185/125 Alive 2 6 20 IAA(A),VSD,PDA,PH(severe) PDA closure via PA,VSD repairing,ADB 185/125 Alive 2 6 20 IAA(A),VSD,PDA,PH(severe) closure via PA,VSD repairing,ADB 208/105 Alive 3 5 15 IAA(A),VSD,PDA,PH(severe) PDA closure via PA,VSD repairing,ADB 305/220 Death 4 6 15 IAA(B),VSD, PDA,PH(severe) PDA closure via PA,VSD repairing,ADB 184/125 Alive 5 11 21 IAA(A),VSD,PDA,MI,PH(severe) PDA closure via PA,VSD repairing,ADB,MVP 180 /137 Alive 6 7 18 IAA(A),VSD,PDA,PH(severe) PDA closure via PA,VSD repairing,ADB 195/122 Alive 7 6 15 IAA(A),VSD,PDA,PH(severe) Division and suture of PDA,VSD repairing,end-end aortic anastomosis 158/85 Alive 8 1 9 IAA(A),VSD,PDA,PH(severe),TS Division and suture of PDA,VSD repairing,end-end aortic anastomosis aortic anastomosis,trachotomy 130/56 Alive IAA.interrupted aortic arch,VSD.ventricular septal defect,PDA.patent ductus arteriosus,PH. pulmonary hypertension, MI.mitral incompetence, TS.tracheal stenosis, PA.Pulmonary artery, ADB.ascending to descending aorta bypass with homograft, MVP. mitral valvuloplasty, CPB. cardiopulmonary bypass 1.2 Surgical approach All were performed in one stage using a median sternotomy (Table 1). The first 6 procedures were homozygous ascending to descending aortic diversion grafts, and the last 2 procedures were end-to-end aortic anastomotic connections. 1.2.1 Homozygous ascending to descending aortic transplantation under extracorporeal circulation Median sternotomy approach, free thoracic aortic anastomosis, early 2 femoral artery cannulation, rest of cannulation methods as below, blocking the aorta under moderate hypothermia, repairing the ventricular septal defect (VSD) after suturing the pulmonary artery to close the arterial catheter, descending and ascending aorta were blocked with lateral forceps, taking the homozygous thoracic aorta and descending aorta respectively. The posterior walls of the descending and ascending aorta were sutured with 5-0 Prolene sutures continuously and the anterior walls were sutured intermittently. 1.2.2 Direct end-to-end anastomotic connection of the aorta: median sternotomy approach, excision of bilateral thymus, freeing the right and left pulmonary arteries, and placing control strips, with one arterial perfusion tube inserted in each of the ascending aorta and pulmonary artery, and blocking the ascending aorta at an anal temperature of 18-20°C. In one case, the aorta was anastomosed under stopping circulation, with a stopping time of 35 min; in one case, the procedure was performed under low-flow (30-50) ml・kg-1・min- The arterial conduit was resected and the posterior wall was interrupted with continuous anterior sutures and the VSD was repaired. 2 Results There were differences in the surgical diversion and blocking times between the two corrective methods, with aortic blocking time (70.5±20.5) min for direct end-to-end anastomotic connection and (139±41.0) min for homozygous ascending to descending aortic diversion graft. One case of low cardiac output syndrome, one case of cardiac insufficiency, and one case of pulmonary hypertension crisis occurred early. One case of tracheal stenosis was found during intraoperative anesthetic intubation, and tracheotomy was performed. One case of intraoperative death was caused by preoperative missed diagnosis of IAA, suture closure of life-dependent arterial catheter and repair of VSD followed by anuria and low cardiac output unable to stop the extracorporeal circulation machine, and the disease was found on exploration, which was ineffective after restoration of elevated aortic blood flow connection with homogeneous aorta. The blood pressure of the upper extremity was higher than that of the lower extremity in five cases after surgery, and the blood pressure of the lower extremity improved significantly, from a systolic blood pressure difference of 50-90 mmHg to a similar blood pressure in the upper and lower extremities. Six cases were followed up for 6 months-14 years [mean (8.5±2.5) years] and all survived. In one case, elevated blood pressure and dizziness in the upper extremity occurred 12 years after surgery, and examination revealed calcified stenosis of the same vessel with a pressure difference of 12 kPa between the upper and lower extremities, and the ascending abdominal aorta was connected again with an artificial vessel through a combined median thoracoabdominal incision. 5.3 kPa pressure difference at the anastomosis was observed in one case with no obvious symptoms, and the remaining four cases had a pressure difference of 1.3-3.3 kPa and NYHA class I cardiac function. 3 Discussion 3.1 Clinically, IAA presents in a variety of ways depending on the combined malformations. Signs, X-rays, electrocardiograms and general ultrasound examinations are not specific, and it is very easy to miss misdiagnosis clinically [6]. In this group, only one case was questioned by ultrasound, and the rest were missed. All of them were suspected by clinicians after blood pressure and oxygen saturation monitoring of upper and lower extremities, and the diagnosis was confirmed by angiography. Echocardiography is very important for the diagnosis of IAA when the upper sternal fossa is cut to avoid missed diagnosis, but it is closely related to the examining physician’s knowledge and experience of the disease. 3.2 Since the first surgical treatment of this disease in 1955, the surgical mortality rate has remained high, over 35% [6,7], and has only decreased in recent years, to about 12% in advanced cardiac centers [4]. Although it has been argued that staged surgical correction is effective [8], more data show that one-stage correction is superior to staged correction. In our group, all children were treated in one stage by median sternotomy, and except for one case due to missed diagnosis and misdiagnosis, there were no surgical deaths in the remaining seven cases and good cardiac function at follow-up. In terms of IAA correction method, we advocate a one-stage median sternal incision approach. 3.3 Homozygous ascending to descending aortic diversion grafting is less common in the previous correction of IAA. This method has less free surface, less invasion, better compliance with homograft than artificial vascular material, less leakage of blood with anastomotic apposition, no need for anticoagulation, lower incidence of calcification and bacterial endocarditis with homograft material, and longer life span. The shortcomings are that the anastomosis is deep and the operation time is long; there is a possibility of restenosis due to distant calcification and obstruction of the tube, and the tube will be replaced again if necessary. One case of stenosis in our group required surgical intervention, and due to severe adhesions, in situ replacement of the tube was dangerous. A relatively simple artificial vessel bypass graft from the ascending aorta to the abdominal aorta was used to achieve better results. In terms of near and long-term results, there is no better alternative to homografts due to their potential growability and less likelihood of restenosis than other artificial vascular materials. This method can be used if the aortic arch has a wide dissection distance >2 cm, if the aortic arch is old, and if the systemic condition and cardiac function are poor. 3.4 Direct anastomotic connection of the aortic arch is the ideal surgical method for IAA, with fewer anastomoses, better exposure of the surgical field, and less time spent on anastomosis, and this method shortens the time by nearly a factor of 1 compared with the same vessel for ascending to descending aortic transplantation. This method is more suitable if the dissection distance of IAA is short, especially if the large artery is free and elastic in infancy. 3.5 The key points of this group are as follows: ①The ascending aortic cannula must be located on the right side of the ascending aorta, corresponding to the descending aortic connection site, which can reduce the direct perfusion of large amounts of blood to the brain or coronary arteries [1]. ② Place one perfusion tube each in the aorta and pulmonary artery, and start the extracorporeal circulation diversion, immediately block the left and right pulmonary artery collaterals so that the blood flow from the pulmonary artery perfusion tube enters the descending aorta directly via the arterial catheter to ensure the blood supply and cooling of the lower body, while preventing the blood flow from entering the pulmonary circulation to cause perfusion of the lung. ③ Performing direct arterial anastomosis should fully free the ascending aorta, cephalobrachial artery, pulmonary artery, and descending aorta to reduce the tension of the anastomosis, prevent bleeding, and completely remove the arterial conduit tissue to prevent stenosis. ④The ascending aorta to descending aorta transfer graft with homologous vessels does not require excessive freeing, small trauma, deeper anastomosis position with descending aorta, and attention to exact suturing to prevent stenosis. ⑤ The posterior wall of the anastomosis is sutured continuously and the anterior wall is sutured intermittently to facilitate growth. 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