Results Of Staged Treatment Of Marfan Syndrome With Cardiovascular Disease:A Single Center Retrospective Clinical Study

Objective: To evaluate the treatment and effects of staged operations on patients suffering from cardiovascular diseases and Marfan syndrome.Methods:A retrospective analysis was performed on 132 patients with Marfan syndrome at our hospital from March 2003 to November 2019.Of those patients,114 underwent 149 surgeries.We classified them into 5 categories according to different pathological changes:(1)aortic root dilation,(2)aortic dissection,(3)thoracic descending aortic/abdominal aortic aneurysm,(4)extensive aneurysm,(5)cardiac lesions.Preoperative echocardiography and computed tomography angiography were performed in all patients to determine the pathological type and lesion range,and surgery was performed according to the corresponding surgical indicationsResults:We performed 149 surgeries on 114 patients.There were 61 cases of aortic root dilation,64 cases of aortic dissection,10 cases of thoracic descending aortic/abdominal aortic aneurysm,8 cases of an extensive aneurysm,and 4 cases of cardiac lesions(2 cases of mitral valve insufficiency,2 cases of heart failure),1 case of pseudoaneurysm of ascending aorta caused by graft infection,and 1 case of endoleak after thoracic endovascular aortic repair.147 operations were performed in stages,including 114 in the first stage,29 in the second stage,4 in the third stage and 2 were reoperations.Staged surgery included 58 cases of aortic root replacement,8 cases of aortic root replacement + mitral valve replacement,4 cases of aortic root replacement + coronary artery bypass grafting,1 case of aortic root replacement + repair of an atrial septal defect,23 cases of root replacement + total arch replacement + frozen elephant trunk,1 case of root replacement + total arch replacement + descending thoracic aorta replacement,1 case of root replacement + total arch replacement,1 case of total arch replacement + frozen elephant trunk,3 cases of descending thoracic aorta replacement,22 cases of thoracic and abdominal aortic replacement,4 cases of hybrid surgery,15 cases of thoracic endovascular aortic repair,2 cases of heart transplantation.3 cases of mitral valve replacement.There were 2 cases of reoperation,1 case for occlusion of pseudoaneurysm of ascending aorta and 1 case for Endovascular therapy.There were 6 surgical deaths(4.0%).Among them,20 patients had postoperative complications,including 11 patients with respiratory insufficiency and 1 patient with stroke,2 patients with transient paresis improved when he discharged,4 patients with deep wound infection,4 patients with renal insufficiency and no paraplegia.The 10-year cumulative survival rate was 91.2%.Conclusion:Successful treatment of cardiovascular diseases in patients with Marfan syndrome was achieved,with good early and long-term results.Nevertheless,it is still necessary to pay close attention to the cardiovascular changes in these patients during the operation period to avoid the occurrence of adverse cardiovascular events and improve the long-term survival rate.