Feldt R H, Driscoll D J, Offord K P, Cha R H, Perrault J, Schaff H V, Puga F J, Danielson G K
Section of Pediatric Cardiology, Mayo Clinic, Rochester, Minn 55905, USA.
J Thorac Cardiovasc Surg. 1996 Sep;112(3):672-80. doi: 10.1016/S0022-5223(96)70051-X.
Patients were observed after the Fontan operation to determine the frequency and severity of protein-losing enteropathy. A total of 427 patients who survived for 30 days after the Fontan operation, performed between 1973 and January 1987, were analyzed and, thus far, protein-losing enteropathy has developed in 47 of 427. The cumulative risk for the development of protein-losing enteropathy by 10 years was 13.4% among 30-day survivors, and 5-year survival after the diagnosis was 46%. Hemodynamic studies done coincident with the diagnosis of protein-losing enteropathy have shown increased systemic venous pressure, decreased cardiac index, increased pulmonary vascular resistance, and increased ventricular end-diastolic pressure. Medical management of protein-losing enteropathy was only partially successful. Statistical analysis has shown that factors related to protein-losing enteropathy were ventricular anatomy, increased preoperative ventricular end-diastolic pressure, longer operative bypass time, increased length of hospital stay, and postoperative renal failure. This study suggests that scrupulous selection of cases for the Fontan operation is mandatory and that certain perioperative factors may predispose to this serious complication of the Fontan procedure.
对接受Fontan手术的患者进行观察，以确定蛋白丢失性肠病的发生率和严重程度。分析了1973年至1987年1月期间接受Fontan手术后存活30天的427例患者，到目前为止，427例中有47例发生了蛋白丢失性肠病。30天存活者中10年内发生蛋白丢失性肠病的累积风险为13.4%，诊断后5年生存率为46%。与蛋白丢失性肠病诊断同时进行的血流动力学研究显示，体静脉压升高、心脏指数降低、肺血管阻力增加和心室舒张末期压力增加。蛋白丢失性肠病的内科治疗仅部分成功。统计分析表明，与蛋白丢失性肠病相关的因素有心室解剖结构、术前心室舒张末期压力升高、手术体外循环时间延长、住院时间延长和术后肾衰竭。本研究提示，对Fontan手术病例进行严格筛选是必要的，某些围手术期因素可能易导致Fontan手术的这一严重并发症。
