Surgical Treatment And Follow-up Research For Total Anomalous Pulmonary Venous Drainage

OBJECTIVE:We retrospectively analyzed the surgical treatment and outcome of 139 patients of total anomalous pulmonary venous drainage (TAPVD), comparing the survival rate after surgical programs, and to explore the most appropriate surgical procedure;and further analysis of the factors affecting the survival and good surgical outcome, providing evidence for optimizing the perioperative management; and finally preliminary discussion on the diagnosis and treatment of postoperative complications pulmonary venous obstruction (PVO) to raise the long-term survival.Methods:From October 1993 to December 2009,157 patients with TAPVD were repaired in our department.18 cases with complex malformation such as CoA,TGA,DORV were removed. A total of 139 simple TAPVD deformity were included. Review the information of patients since admission, collect the demographic data, preoperative treatment, surgical approach postoperative follow-up data. Statistical analysis of the causes of death and perioperative risk factors, medium and long term survival, the relevant factors that affect the early surgical results and follow up results.Results:A total number of 139 patients with TAPVD underwent surgery at our center during a periord of over sixteen years.81 cases of boys and 58 girls, The age ranging from 10 days to 13 years,mean 15.92±30.38 months,23 patients (16.5%) were less than 1 month of age,73 patients (52.8%) were less than 3 months. the weight ranging from 1.8 kg to 20 kg,mean 7.15±5.51 kg,61 patients (44.8%)were less than 5 kg.59 patients belong to supracardiac (46%),55 cardiac type (46%),17 infracardiac type (12%) and 8 mixed type (4%). Most patients had heart failure performance such as shortness of breath, sweating, growth retardation before surgery.36 patients exhibited signs of PVO.29 patients were given a preoperative dose of inotropic drugs, including dopamine, milrinone, etc.Mechanical ventilation was given to 21 cases because of severe respiratory acidosis to improve oxygenation. the emergency operation in 43 (30.9%), Asian emergency surgery in 34 patients (24.5%), elective surgery in 62 cases (44.6%). The repair was performed under moderate hypothermia in 117 and deep hypothermic arrest in 23.Before the year 2000 TAPVD of supracardiac type was repaired by transversing the right atrium and through atrium septum to perform anastomosis between the left atrium and the common vein, and after that the superior anastomosis approach was used. Coronary sinus unroofing was preferred for cardiac type. the infracardiac was repaired by lifting the heart. Mixed with concrete drainage sites, integrated use of the above methods. Mean duration of cardiopulmonary bypass time and crossclamp time were 85.23±36.48 min and 48.45±23.47 min. The ventilation time,intense time and hospital stay were 70.04±70.58h,7.31±4.73d and 20.09±11.31d, respectively.There were 13 (9.4%) perioperative deaths, of which 5 died before the year 2000, the death rate was 20%。And 8 deaths after 2000, the death rate was 6.3%. The main perioperative complications were pulmonary hypertensive crises in 21,low output in 18,transient arrhythmia 17 and so on.The reason of periopetative death were six cases of low cardiac output, three cases of severe arrhythmia, two cases of residual obstruction, two cases of postoperative bleeding, one patient with severe pulmonary infection. The statiscially significant risk factor for early death including preoperative PH value (p=0.01), and preoperative PVO(p =0.000), preoperative arrhythmia (p=0.004), preoperative mechanical ventilation(p=0.001), preoperative cardiac function(p=0.000), preoperative application of inotropic drugs (p=0.025), earlier operation year and so on.126 cases were discharged uneventfully.109 patients had follow-up data.. The average follow-up time was 39.33±38.57m (1 month to15 years). Echocardiographic, chest radiograph, ECG were underwent during this period. Six later death happened during the medium follow up, three of which died within 1 year after discharge, the main reason of postoperative death was PVO. No long-term death. Kaplan-Meier was used to estimate the survival condition, later survival ratio was 93.8%, and 95% CI was 86.3%-97.3%. Log-rank test way statistical analysis revealed that surgical strategy and various of anatomical type had no apparent effect on the long-time survival. Ten patients had postoperative PVO, the occurrence ratio was 7.2%, Reoperations were performed for four patients at 2 monthes to 2 years after surgery,3 patients died finally and only 1 survived.Conclusions To prevent PVO and severe pulmonary hypertension, the correction of TAPVD should be made once the diagnosed was confirmed. Prognosis depends upon adequate anastomosis between the common vein and left atrium and no obstruction of pulmonary venous return.The mortality after TAPVD repair had decreased by improved surgical techniques and perioperative care but seemed remain highest in the patients with poor preoperative condition. PVO is the most serious complications and the main cause of postoperative death, how to reduce the mortality after PVO is still a great challenge that we have to face.