Abstract： Objective To summarize our experience of surgical treatment for anomalous origin of one pulmonary artery in infants and children. Methods From March 2005 to May 2010，11 patients with anomalous origin of one pulmonary artery and other concomitant congenital cardiovascular malformations underwent surgical repair in Xijing Hospital of Fourth Military Medical University．The mean age of the patients was 11.5 months with a range from 2 months to 36 months．Their mean body weight was 7.1 kg with a range from 4 to 13 kg. Seven patients had anomalous origin of the right pulmonary artery from the ascending aorta, and four patients had anomalous origin of the left pulmonary artery from the ascending aorta. All the eleven patients had other concomitant intracardiac anomalies or vascular malformations as well as pulmonary hypertension, and underwent one stage surgical repair via median sternotomy under hypothermia and cardiopulmonary bypass. Results Their operation time was 169 - 293 （231±55） min, cardiopulmonary bypass time was 87-210 （138±47） min, and aortic-clamping time was 45-133 （86±28） min. There was one postoperative death who had low cardiac output syndrome after repair for tetralogy of Fallot and anomalous origin of the right pulmonary artery. The overall postoperative mortality was 9.1％. Postoperative echocardiography of all the surviving patients showed their left and right pulmonary artery origined from the right ventricle and pulmonary artery with satisfactory malformation correction but no residual shunt and pulmonary stenosis . All the surviving ten patients were followed up with a follow-up rate of 100% and mean follow-up time of 13.5 months with a range from 3 to 32 months. Their echocardiography during follow-up showed that there was no pulmonary stenosis in all the patients, and pulmonary blood pressure significantly decreased in 9 patients. Conclusion Patients with anomalous origin of one pulmonary artery should undergo surgical repair as early as possible with satisfactory short-term outcomes in infants and children. For elder patients with irreversible pulmonary hypertension, the choice of surgical treatment should be more cautious. During the surgery, the anomalous pulmonary artery and ascending aorta should be dissociated fully, and transection of the ascending aorta is helpful to get a satisfactory operating field view for the surgeon. Repairing aortic defect with autologous pulmonary arterial patch can effectively avoid the occurrence of postoperative aortic aneurysm.
ObjectiveTo explore surgical methods and risk factors of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). MethodsClinical data of 28 ALCAPA patients who underwent surgical repair from October 1993 to September 2013 in Beijing Anzhen Hospital were retrospectively reviewed. There were 8 male and 20 female patients with their age of 0.6-l6.8 (4.3±0.7)years including 10 patients less than 1 years old. Surgical procedures included simple ligation of left coronary artery, intrapulmonary tunnel procedure (Takeuchi)and direct coronary reimplantation of the anomalous artery. Postoperative death, complication and cardiac function were observed. ResultsAmong the 28 patients, 1 patient received simple ligation of left coronary artery, and 7 patients received intrapulmonary tunnel procedure (Takeuchi), among whom 2 patients died postoperatively. Twenty patients received direct implantation of the anomalous artery into the ascending aorta, and 3 patients died postoperatively. Five patients who died postoperatively were 10.20±3.27 months old, including 3 patients with moderate mitral regurgitation (MR)and 2 patients with mild MR preoperatively. Preoperative heart function of the patients who died postoperatively was significantly reduced. Preoperative left ventricular ejection fraction of the patients who died postoperatively was significantly lower than that of the patients who survived (36.6%±8.5% vs. 60.9%±10.7%, P=0.000). Low cardiac output syndrome was the reason for all postoperative death. All survival patients were followed up from 1 month to 18 years. One patient who underwent intra-pulmonary tunnel procedure (Takeuchi)received pulmonary artery balloon dilatation for pulmonary supravalvular stenosis 15 years after discharge. None of the other patients received a secondary operation. During follow-up, left ventricular function was improved. Growth and development of all the patients was normal. MR did not significantly aggravate in all the patients. ConclusionPatients with younger age and worse left ventricular function have greater surgical risks of ALCAPA.
Objective To summarize the method and outcomes of surgical treatment for 21 patients with congenital anomalous left coronary artery from the pulmonary artery (ALCAPA). Methods We retrospectively analyzed the clinical data of 21 patients with ALCAPA underwent surgical treatment in our center from January 2010 to January 2015. There were 11 males and 10 females with a mean age of 4.3 years (ranging from 0.5 to 16.0 years) and a mean weight of 19.3 kg (ranging from 5.0 to 97.0 kg). All of 21 patients underwent surgery under cardiopulmonary bypass and corrected malformations. Results There were 2 perioperative deaths and the mortality rate was 9.5%. The mean cardiopulmonary bypass time was 116.6 minutes ranging from 109.0 to 388.0 minutes and the mean aortic cross clamping time was 82.9 minutes ranging from 62.0 to 129.0 minutes. The mean time of hospital stay was 11.1 days ranging from 1.0 to 25.0 days. After surgery, cardiac function improved significantly in all patients. The mean left ventricular ejection fraction (EF), left ventricular fractional shortening (FS), and left ventricular end-diastolic diameter (LVEDD) have significantly improved after surgery (P < 0.05). Conclusions Once patients with ALPACA are diagnosed, they should be treated with surgery and most of them will achieve a satisfactory long term clinical result.