Diagnosis of the aortopulmonary window and one pulmonary artery: To be differentiated from arteriovenous ductus arteriosus, ventricular septal defect, permanent arterial trunk and ruptured lack of sinus aneurysm. Chest radiographs suggest cardiac enlargement and pulmonary hemorrhage. Ultrasound can localize and quantify the defect, but the diagnosis of concomitant malformations is more difficult, especially when differentiated from a permanent truncal artery by the presence or absence of two sets of semilunar valves. The electrocardiogram suggests a large or biventricular left ventricle. Cardiac catheterization is required to estimate pulmonary vascular resistance and to identify concomitant anomalies. Surgical treatment: Early surgery is indicated in most patients when the diagnosis is clear; in rare patients, it may be deferred until after infancy if the defect is small and the symptoms are not obvious. Concomitant malformations should be corrected at the same time. From April 2000 to January 2006, 17 children, 9 males and 8 females, with a main pulmonary window and right pulmonary artery originating from the ascending aorta were admitted to our center. All of them were diagnosed by cardiac catheterization, angiography, cardiac ultrasound, cardiac MRI, cardiac CT, etc. Among them, 4 cases had right pulmonary artery originated from ascending aorta, 5 cases had simple aortic window (all type I), and 8 other cases had aortic arch interruption, ductus arteriosus, tetralogy of Fallot, ventricular septal defect, atrial septal defect, mitral regurgitation and tracheal stenosis, etc. One case was not indicated for surgery. The remaining 16 cases were treated with one-stage surgical anatomical correction of the associated malformations. The average age at surgery was 1.7±1.7yr (21d-6yr), and the average weight was 8.5±3.9kg (3.5-18 kg). The duration of extracorporeal circulation diversion for the whole group was 62.2±17.8 min, aortic block was 39.0±13.5 min, and 6 cases were repaired by deep hypothermic stoppage of circulation, with a mean stoppage of 37.8±15.2 min. Results: There was no surgical death, and the survival rate was 100%. 2 infants had delayed chest closure 3 days after surgery, and 1 case had postoperative bleeding, and the chest was opened again 3 hr later to stop bleeding. Mean ventilator use was 20 hr, and CICU stay was 3 d. Follow-up: Fourteen cases were followed up from 6 months to 4 years after surgery with ECG, X-ray chest radiograph, cardiac ultrasound-Doppler and MRI. There was no supra-aortic stenosis or pulmonary stenosis in the whole group, and only one case of mitral regurgitation had residual mild regurgitation. Discussion: The embryonic arterial trunk is located in the right upper and left lower wall in a pair of corresponding arterial trunk cushions fused to form the proximal part of the aortic and pulmonary artery ductal septum, and the distal part is formed by the fusion of the fourth pair of arterial arches with the aortic channel and the sixth pair of arterial arches with the pulmonary channel, and the distal aortic and pulmonary artery septum fused with the proximal arterial trunk cushions is The distal main and pulmonary artery septum, which is fused with the proximal trunk cushion, is formed by the wall between the fourth and sixth arterial arch pairs. If the arterial trunk pad fusion is abnormal resulting in a proximal defect in the main pulmonary septum, abnormal migration of the sixth pair of arterial arches results in a distal arterial window or the origin of one pulmonary artery in the aorta. The aortopulmonary window is overwhelmingly a single lesion and is predominantly located in the left wall of the aorta. The aortopulmonary window or the two sets of semilunar valves on one side of the pulmonary artery originating from the aorta are normal, with a large variation in defect diameter and aneurysmal dilatation seen in larger defects. One side of the pulmonary artery originates from the ascending aorta, and the pulmonary artery (often the right pulmonary artery) often originates abnormally above the sinotubular junction in the posterior wall of the proximal ascending aorta, directly above the lateral wall of the aortic arch, with no defect between the ascending aorta and the pulmonary trunk. The hemodynamics of the aortopulmonary window are similar to those of the patent ductus arteriosus, ventricular septal defect, and permanent arterial trunk. The fractional flow is mainly related to the size of the defect and pulmonary vascular resistance. When the defect is small, the fractional flow is low and the symptoms are insignificant or asymptomatic. With larger defects, the fractional flow is high, leading to congestive heart failure, pulmonary hypertension, and early obstructive pulmonary vascular lesions. Survival time does not exceed infancy. In contrast, the right pulmonary artery originates from the aorta with a large left-to-right shunt, and the right lung receives flow from the corpora arteriosa and develops early pulmonary vasculopathy; the left lung receives all pulmonary circulation flow and often develops pulmonary hypertension. The main pulmonary window and the right pulmonary artery originated from the aorta are clinically difficult to distinguish from the arteriovenous ductus arteriosus, ventricular septal defect and permanent arterial trunk, etc. The main reliance is on cardiac ultrasound, cardiac MRI, cardiac CT and cardiac catheterization, imaging, etc. The usual practice of the authors is: if the child is older than 6 months, cardiac catheterization must be performed to assess the pulmonary artery pressure and resistance, and in children with severe pulmonary hypertension, if the pulmonary small vessel resistance Children with severe pulmonary hypertension who have pulmonary small vessel resistance greater than 10 to 12 Woods units and stiffness of the small pulmonary arteries on imaging, and who are older than 2 years of age, are usually lost to surgery. The main pulmonary artery window and the right pulmonary artery originated from the aorta are prone to early pulmonary hypertension and pulmonary vasculopathy, and once diagnosed, there is a strong indication for surgery. In order to better visualize the aortopulmonary window fistula and coronary artery opening, transaortic patch repair under extracorporeal circulation is now preferred: a median sternotomy is made, the aorta is fully freed, and a high aortic cannula is taken to facilitate placement of a blocking clamp and repair of the defect. Depending on the age and weight of the child, a single right atrial or double vena cava can be cannulated. The right and left pulmonary arteries are trapped after the start of extracorporeal circulation. The repair can be performed under stoppage of circulation or continuous full diversion. After aortic block, the anterior aortic wall is incised vertically and the opening of the coronary artery is carefully inspected to ensure that the opening is on the aortic side of the patch. If there is a coronary artery originating from the pulmonary artery, it must also be isolated on the aortic side. Some authors advocate that one side of the pulmonary artery originating from the aorta should be anastomosed directly from the pulmonary artery with an abnormal origin to the pulmonary trunk. The authors’ unit experience is that to prevent distant anastomotic stenosis, if the anomalous origin of the pulmonary artery is high and far from the aortic valve and coronary artery opening, the Double Flap anastomosis can be used: the right pulmonary artery with anomalous origin is trimmed together with the posterior wall of the aorta and sutured with part of the anterior wall of the pulmonary trunk Flap or the right pulmonary artery with anomalous origin is trimmed together with the anterior and posterior walls of the aorta and sutured with A modified Double Flap approach was used to anastomose the pulmonary artery trunk. The aortic window and the right pulmonary artery originated from the aorta with interrupted aortic arch were cannulated with the same interrupted aortic arch alone, and the ascending aorta was cannulated with a single cannula, and the right and left pulmonary arteries were trapped at the beginning of extracorporeal circulation, and the descending aorta was perfused through the aortic window and arterial catheter. After cooling and stopping the circulation, the cephalic brachial artery is circled, the arterial duct is sutured off, the residual ductal tissue is excised, the distal descending aorta is anastomosed to the lower edge of the aortic arch, and the main pulmonary artery window is closed via an anterior aortic wall incision patch. In neonates or small infants with ascending aorta-aortic arch dysplasia, the main-pulmonary artery can be separated, the pulmonary trunk defect repaired with a patch, the distal descending aorta anastomosed to the aortic arch, and the lateral wall of the ascending aorta and the inferior border of the arch repaired with an expanded patch of the same aortic patch. At present, most of the international precordial treatment centers have good results for the main pulmonary window and one side of the pulmonary artery originating from the aorta, and the operative mortality rate has been close to zero. Moreover, the possibility of recurrence and pulmonary artery stenosis is very low due to the current common use of patch repair. There are limited reports on the long-term outcome of aortic origin of one pulmonary artery, and close follow-up is required for the development of pulmonary stenosis. The prognosis of patients with complex malformations is largely determined by whether the concomitant malformation has been corrected simultaneously. In contrast, the outcome in older children is largely determined by the pulmonary vascular resistance at the time of surgery.