Abstract： Marfan syndrome （MFS） is a congenital and heritable autosomal dominant disorder of the connective tissue which is often passed down through families. Its clinical presentation typically involves the skeletal， cardiovascular and ocular systems with a high natural mortality. Aortic root aneurysm and consecutive acute aortic dissection represent the main cardiovascular manifestations and main causes of morbidity and mortality in MFS. At present， the predominant therapeutic method is surgery， but surgical outcomes are quite unsatisfactory. Recent studies demonstrate that losartan， a common antihypertensive agent， is useful to treat MFS， the mechanism of which may results from inhibiting overactivation of transforming growth factor β （TGF-β） signaling. This discovery will definitely promote the transition of traditional surgical treatment of MFS into pharmacotherapy. In this review， we focus on the molecular biological pathogenesis， traditional and new therapeutic strategies for MFS patients.
Objective To assess clinical results of three different conduit materials （Gore-Tex synthetic graft，bovinejugular vein and autologous pericardium）for palliative right ventricle-to-pulmonary artery （RV-PA） shunt，and explore the correlation between suitable conduit size and patients’ body weight and McGoon ratio. Methods We retrospectively analyzed clinical data of 24 patients with congenital heart diseases who underwent palliative RV-PA shunt in Department of Pediatric Cardiovascular Surgery of Fu Wai Cardiovascular Hospital from July 2010 to July 2012. There were 11 males and 13 females with their age ranging from 60 days to 6 years and body weight of 10.22±7.41 kg. There were 22 patients with pulmonary atresia and ventricular septal defect （PAVSD），1 patient with tetralogy of Fallot （TOF） and 1 patient with doubleoutlet right ventricle （DORV）. Among different conduit materials，autologous pericardium was used for 17 patients，Gore-Texsynthetic graft was used for 5 patients，and bovine jugular vein was used for 2 patients. Conduit size and children’s body weight were analyzed with linear regression，then the equation was corrected with McGoon ratio. Results There was no perioperative death. Postoperative percutaneous saturation （SpO2）of the 24 children was 20.37%±28.33% higher than preoperative SpO2 . Electrocardiogram showed sinus rhythm in all the patients. Twenty-three patients were NYHA classⅡ，and 1 patient was NYHA classⅢ. Postoperative mechanical ventilation time of patients with autologous pericardium were significantly shorter than those of patients with other 2 materials （P=0.017）. Sixteen patients were followed up from 10 months to 2 years after discharge，including 12 patients with autologous pericardium，3 patients with Gore-Tex synthetic graft and 1 patient with bovine jugular vein. During follow-up，McGoon ratio of patients with autologous pericardium，Gore-Tex synthetic graft and bovine jugular vein were 1.98±0.46，1.83±0.33 and 1.68 respectively，all of which weresignificantly higher than preoperative McGoon ratio （P＜0.05）. Six patients underwent radical corrective surgery，including5 patients with autologous pericardium and 1 patient with Gore-Tex synthetic graft. There was no complication directly related to surgery during follow-up. Linear regression was performed to form an equation between suitable conduit size and patients’ body weight：conduit diameter （mm）=0.327×body weight （kg）＋4.599. McGoon ratio，conduit size and equationresult were compared to find a practical choice of conduit size. If McGoon ratio＜0.8，the first integer greater than the equation result could be chosen. If McGoon ratio＞1.2，the first integer less than the equation result could be chosen. If 1.2＞McGoon ratio＞0.8，the first integer either less or greater than the equation result could be chosen. Group analysis showed that patients who recovered better postoperatively were those whose conduit sizes were closer to equation results as well as equation results corrected with McGoon ratio. Conclusion All the 3 materials can be conventionally chosen for RV-PA shunt. Appropriate conduit size can be decided upon patients’ body weight and McGoon ratio for RV-PA shunt.