三尖瓣置换术治疗Ebstein心脏畸形的临床研究

The Clinical Research of Tricuspid Valve Replacement in the Treatment of Ebstein's Anomaly

目的评价三尖瓣置换术(TVR)治疗重症Ebstein心脏畸形的效果,分析手术适应证及个体化手术方案的选择。方法回顾性收集安贞医院1993年5月至2007年6月32例Ebstein心脏畸形患者施行TVR的临床资料,采用Kaplan-Meier生存曲线计算中长期生存率和免除血栓栓塞率;对比分析人工瓣膜高位或原位缝合、房化心室折叠与否、三尖瓣保留与否的临床效果;并对死亡原因进行单因素分析和logistic回归分析。结果住院死亡2例(6.25%)。30例患者随访4.2±3.5年,3例发生瓣膜毁损,其中生物瓣2例,机械瓣血管翳形成1例,均再次施行TVR治疗。晚期死亡2例(6.67%),全组患者术后5年、10年生存率分别为92.1%±4.6%和86.5%±5.8%。发生血栓栓塞事件2例次,均经溶栓治疗成功,5年、10年无血栓栓塞率分别为91.3%±4.5%和82.3%±4.2%。生存28例患者术后6个月跨人工瓣膜压差为12.7±3.2mmHg;心功能级24例,级4例。人工瓣膜原位缝合者发生结性心律的比率显著高于高位缝合者(5例vs.1例,P=0.022),房化心室不折叠者的心室矛盾运动比率显著高于折叠者(6例vs.1例,P=0.024),保留三尖瓣与否对心功能的影响差异无统计学意义。单因素和多因素logistic回归分析发现,术前固有右心室/房化心室比率<1、腹水为早期死亡的危险因素(P=0.023,0.025);术前腹水、下肢水肿为晚期死亡的危险因素(P=0.026,0.019)。结论TVR是治疗重症Ebstein心脏畸形的有效方法,应根椐患者病理解剖情况个体化决定房化心室折叠与否、保留与不保留自体三尖瓣,但原位缝合患者发生结性心律的比率高于高位缝合者。

Objective To evaluate the effect of treating Ebstein＇s anomaly with tricuspid valve replacement （TVR）, and analyze the indications and personalize the surgical methods of TVR. Methods 35 TVR in 32 cases of Ehstein＇s anomaly not amenable to repair from May 1993 to June 2007 in Anzhen Hospital were retrospectively. The mortality and complication incidence were estimated and the Kaplan-Meier survival curves were constructed to estimate actual survival and freedom from thrombus embolism rate, and comparative studies were undertaken to evaluate the clinical effect between implanting the prosthesis in the original tricuspid ring or above the coronary sinus, and between fold the atrialized ventricle or not, and between preserve the autologous tricuspid or not. The univariate analysis and logistic regression were used to explore the early and late death risk factors. Results There were 2 early hospital death （6. 25%）. Follow-up of 30 patients who survived 30 days ranged up to 14 years （4. 2±3. 5 years）,there were 3 TVR reoperation due to the prosthesis deterioration. There were 2 late deaths（6.67%）, and the actuarial survival at 5,10 year was 92.1%±4.6%, 86.5%±5.8% respectively in Kaplan-Meier curve. There were 2 events of thrombus embolism that were cured through thrombolytic therapy, thus the freedom from thrombus embolism at 5 and 10 yers reached 91.3%±4.5%,82.3%±4.2% respectively. 24 late survivors were in New York Heart Association（NYHA） functional class Ⅰ, and 4 in NYHA functional class Ⅱ. The incidence of atria ventricular node rhythm in patients with the prosthesis implanted in original tricuspid ring was statistically higher than that of prosthesis implanted above the coronary sinus, and the incidence of paradoxical ventricular septal motion in patients without folding the atrialized ventricle was statistically higher that of with folding the atrialized ventricle, but the NYHA functional class was not affected by these factors. The univariate analysis and logistic regression revealed that preoperative functional right ventricle/atrialized ventricle〈1 and ascites were the independent risk factor for the early death （P = 0. 023, 0. 025）, whereas preoperative ascites and edema in lower extremities were the independent risk factors for late death （P = 0. 026, 0. 019）. Conclusion TVR is a good therapeutically option for Ebstein＇s anomaly since the operative risk is low, the functional status improved in all patients and the durability of prosthesis in tricuspid position has been good. In order to maximize the effect of the TVR in Ebstein＇s anomaly, personalized procedure should be adopted in deciding whether to fold the atrialized ventricle or not, to preserve the autologous tricuspid valve or not, and to implant the prosthesis in the original tricuspid ring or above the coronary sinus.