Complex corrective transposition of the great arteries

  In order to avoid the occurrence of postoperative cardiac insufficiency, the right ventricle is separated from the right ventricle and then the superior vena cava is directly anastomosed with the pulmonary artery, which reduces the load on the right ventricle from the surface. This is a new treatment modality that has emerged in recent years in the field of complex congenital heart disease – “one-and-a-half” ventricular correction.  Corrective transposition of the great arteries is a complex congenital heart disease in which the anatomical left ventricle is used as the right ventricle, while the anatomical right ventricle is burdened with a heavier pressure on the body circulation. Conventional surgical treatment only corrects for the intracardiac malformation, and after surgery the anatomical right ventricle remains as the blood pump of the body circulation, unable to bear the pressure load of the body circulation, with a poor long-term prognosis. The incidence of right ventricular insufficiency is 40% at 3 years after surgery and up to 55%-68% at 10 years. In recent years, with the application of anatomic correction surgery – atrioventricular double transfer surgery – the near and long-term outcome of this type of disorder has been significantly improved. However, due to the presence of several complex combined malformations, some right ventricular dysplasia, or combined tricuspid valve malformation, some heart position variants such as right-sided heart or atrioventricular inversion can also affect the operation of double reversal surgery. In the past, such patients often could only undergo single-ventricle corrective surgery, which means that the patient loses the right ventricle that supports pulmonary circulation, and the pattern of their circulatory system resembles the circulatory characteristics of inferior crawling class of arteries, resulting in many postoperative near and long-term complications and unsatisfactory cardiac function and quality of life.  In order to solve this problem, the Pediatric Cardiac Surgery Center of Fu Wai Cardiovascular Hospital in Beijing, under the leadership of its director, Professor Li Shoujun, began to experiment with a “one-and-a-half” ventricular correction strategy, i.e., a simultaneous Rastelli + half Mustard + two-way Glenn procedure, in which the aorta is tunneled intracardially to the In this way, the “one and a half” ventricle is corrected. The advantages include: (1) the semi-Mustard procedure can reduce the number of intra-atrial sutures, which helps to reduce the late complications of the Mustard procedure, such as sinus node dysfunction and pulmonary vein obstruction; (2) for patients with pulmonary artery-external right ventricle connection, the semi-Mustard/bi-directional Glenn procedure reduces the blood flow through the right ventricle-pulmonary artery external conduit, which can (3) The semi-Mustard procedure is simpler than the traditional Mustard, especially for patients with complex cardiac anatomy such as right-sided heart or atrial inversion.      In March this year, a 5-year-old boy accompanied by his parents came to Beijing Fu Wai Cardiovascular Hospital with “shortness of breath with cyanosis” after birth. The aorta was 55% from the right ventricle, the main pulmonary artery was atretic with no obvious traces of subvalvular outflow tract; the right and left pulmonary arteries were still developing with fusion, the descending right aortic arch and 3mm unclosed arterial duct, the left superior vena cava had an internal diameter of about 7mm and entered the coronary sinus. The diagnosis was congenital heart disease: single right-sided heart, corrected transposition of the great arteries, pulmonary atresia, ventricular septal defect, atrial septal defect, unclosed ductus arteriosus, left superior vena cava connected to the coronary venous sinus, and right aortic arch. The child was considered too complex to be treated surgically in the local hospital. The family came to the National Cardiovascular Center Beijing Fu Wai Cardiovascular Hospital with a ray of hope and found Professor Li Shoujun of the Pediatric Cardiac Surgery Center, who carefully examined the child, held several intra-departmental discussions, formulated a thorough surgical plan, and performed the first Rastelli+ on April 7. The surgery went smoothly, and the child recovered well and was discharged from the hospital after the surgery. In the following 3 months, Prof. Li has completed 4 similar cases of “one and a half” ventricular correction surgery, without surgical death or serious complications. In the face of such impressive results, Prof. Li is not satisfied and hopes to go to the next level in terms of technology. He will further improve the indications and surgical techniques for this type of surgery and complete the “one-and-a-half” ventricular correction based on arterial level transfer, so that more children can receive more reasonable treatment.