Background Pulmonary arterial hypoplasia is a common complication in complex cyanotic congenital heart disease. For extreme stenosis of the branch pulmonary arteries,which is not feasible for complete repair,palliativ...Background Pulmonary arterial hypoplasia is a common complication in complex cyanotic congenital heart disease. For extreme stenosis of the branch pulmonary arteries,which is not feasible for complete repair,palliative systemic-pulmonary shunt is needed to increase pulmonary blood flow and pulmonary tree growth as early as beyond 2 years old. Unfortunately,due to poor medical setting in local hospitals,there are still a small number of patient with severe hypoplastic pulmonary arteries in developing area who have failed to undergo appropriate surgical intervention till to teenagers even adults. In order to explore the resolution of the dilemma for these notable cyanotic patients,hence we began to utilize three-dimensional computed tomography(CT)to reconstruct pulmonary artery anatomy and to simulate virtual palliative systemic pulmonary shunt conduit module,to facilitate and improve the intraoperative aortopulmonary shunt performance. Methods FromApril 2011 to August 2018,13 consecutive patients undergoing aortopulmonary shunt with older age(13-35 years old)who missed the optimal timing for surgery were identified from 196 cases involving palliative systemic pulmonary shunt. An individually pre-designed prosthetic expanded poly tetra fluoroethylene(ePTFE)conduit was utilized based on the threedimensional enhanced computed tomography reconstruction and simulation. The post-operative recovery courses and complications were documented. Blood gas analysis,electrocardiogram,echocardiography were performed routinely prior to discharge and compared with the preoperative data. Re-evaluation of finger pulse saturation(SpO2),echocardiography and electrocardiogram was performed in clinical follow-up in 1 st month,6 th month,12 th month and every year postoperatively. A retrospective analysis of operative data,postoperative outcomes and complications were performed. Results All the 13 consecutive patients underwent successfully non-cardiopulmonary bypass systemic-pulmonary shunt with ePTFE conduits via median sternotomy. Postoperative thirtyday mortality was 1 in 13 due to pulmonary hemorrhage. During the follow-up,no more mortality was documented but a re-intervention for ePTFE conduit revision. For the 12 survivors,the postoperative SpO2,and arterial partial oxygen pressure(PaO2)on room air significantly increased from 68.0±2.42% to 88.46±4.67%(P<0.01),and from 42.61±3.94 mmhg to 49.62±1.76 mmhg(P<0.01),respectively. While the postoperative hematocrit and hemoglobin significantly decreased from 72.01±3.12% to 61.03±3.21%(P<0.01),and from 196.77±10.56 g/dL to 171.76±6.52 g/dL(P<0.01),respectively. Conclusions Appropriate systemic-pulmonary shunt based on threedimensional reconstruction and simulation can significantly alleviate the hypoxia with elevated oxygen saturation for severe cyanotic adolescent or adult patient with extreme pulmonary hypoplasia and unrepairable complex congenital heart disease,so as to improve their clinical symptoms and life quality,although it cannot promote secondary pulmonary artery development directly.展开更多
基金supported by Medical Scientific Research Foundation of Guangdong Province,China (No.A2018038)
文摘Background Pulmonary arterial hypoplasia is a common complication in complex cyanotic congenital heart disease. For extreme stenosis of the branch pulmonary arteries,which is not feasible for complete repair,palliative systemic-pulmonary shunt is needed to increase pulmonary blood flow and pulmonary tree growth as early as beyond 2 years old. Unfortunately,due to poor medical setting in local hospitals,there are still a small number of patient with severe hypoplastic pulmonary arteries in developing area who have failed to undergo appropriate surgical intervention till to teenagers even adults. In order to explore the resolution of the dilemma for these notable cyanotic patients,hence we began to utilize three-dimensional computed tomography(CT)to reconstruct pulmonary artery anatomy and to simulate virtual palliative systemic pulmonary shunt conduit module,to facilitate and improve the intraoperative aortopulmonary shunt performance. Methods FromApril 2011 to August 2018,13 consecutive patients undergoing aortopulmonary shunt with older age(13-35 years old)who missed the optimal timing for surgery were identified from 196 cases involving palliative systemic pulmonary shunt. An individually pre-designed prosthetic expanded poly tetra fluoroethylene(ePTFE)conduit was utilized based on the threedimensional enhanced computed tomography reconstruction and simulation. The post-operative recovery courses and complications were documented. Blood gas analysis,electrocardiogram,echocardiography were performed routinely prior to discharge and compared with the preoperative data. Re-evaluation of finger pulse saturation(SpO2),echocardiography and electrocardiogram was performed in clinical follow-up in 1 st month,6 th month,12 th month and every year postoperatively. A retrospective analysis of operative data,postoperative outcomes and complications were performed. Results All the 13 consecutive patients underwent successfully non-cardiopulmonary bypass systemic-pulmonary shunt with ePTFE conduits via median sternotomy. Postoperative thirtyday mortality was 1 in 13 due to pulmonary hemorrhage. During the follow-up,no more mortality was documented but a re-intervention for ePTFE conduit revision. For the 12 survivors,the postoperative SpO2,and arterial partial oxygen pressure(PaO2)on room air significantly increased from 68.0±2.42% to 88.46±4.67%(P<0.01),and from 42.61±3.94 mmhg to 49.62±1.76 mmhg(P<0.01),respectively. While the postoperative hematocrit and hemoglobin significantly decreased from 72.01±3.12% to 61.03±3.21%(P<0.01),and from 196.77±10.56 g/dL to 171.76±6.52 g/dL(P<0.01),respectively. Conclusions Appropriate systemic-pulmonary shunt based on threedimensional reconstruction and simulation can significantly alleviate the hypoxia with elevated oxygen saturation for severe cyanotic adolescent or adult patient with extreme pulmonary hypoplasia and unrepairable complex congenital heart disease,so as to improve their clinical symptoms and life quality,although it cannot promote secondary pulmonary artery development directly.