Résumé
@#Objective To retrospectively reviewed our experience of the surgical and perioperative treatment of patients suffering from critical Marfan syndrome with severe left ventricular dysfunction and to evaluate its therapeutic effect and prognosis. Methods Between January 2012 and October 2016, 15 patients diagnosed with Marfan syndrome combined with severe left ventricular dysfunction (left ventricular ejection fraction≤40% or left ventricular end diastolic diameter≥75 mm) underwent operations for aortic root aneurysm in Zhujiang Hospital and Guangdong General Hospital. Among them, 11 were males and 4 were females with a mean age of 32.9±8.7 years ranging from 19 to 55 years. Five patients with aortic dissection underwent Bentall procedure and total arch reconstruction with stent graft implantation. Two patients underwent Bentall procedure and hemi-arch replacement, seven patients underwent Bentall procedure and one patient underwent Cabrol procedure. Concomitant procedures included mitral valve repair in 12 patients, mitral valve replacement in 3 patients and tricuspid valve repair in 12 patients. Results There were 11 patients (73.3%) receiving intra-aortic balloon pumping implantation. One (6.7%) in-hospital death occurred. The left ventricular end diastolic diameter decreased from 80.5±7.4 mm to 58.3±6.0 mm (P<0.05) and the left ventricular ejection fraction improved from 37.3%±5.2% to 46.3%±4.4% 3 months postoperatively (P<0.05). The left ventricular end diastolic diameter decreased from 80.5±7.4 mm to 53.7±3.6 mm (P<0.05) and the left ventricular ejection fraction improved from 37.3%±5.2% to 57.7%±4.2% after one year (P<0.05). No death and reoperation occurred in the follow-up. Conclusion Although the patients with Marfan syndrome and severe left ventricular dysfunction usually have a high surgical mortality, the key to satisfactory outcomes of severe Marfan syndrome is adequate preoperative preparation, complete correction of all vascular lesions during the operation, application of circulatory auxiliary device and perioperative strict and long-term ICU monitoring.