儿童先天性矫正型大动脉转位行形态三尖瓣置换术结果研究

Clinical outcomes of the morphologic tricuspid valve replacement for children with congenitally corrected transposition of the great arteries

目的研究14岁及以下儿童矫正型大动脉转位(ccTGA)患者行形态三尖瓣置换术的手术适应证及术后近远期结果。方法2007年7月至2017年8月,阜外医院为14岁及以下ccTGA患者行形态三尖瓣置换术4例,男3例,女1例;年龄5.3~14(11.1±3.9)岁,体重20~44(30±10)kg。两例合并内脏反位,1例合并单冠畸形,1例合并形态二尖瓣关闭不全;术前形态右心室射血分数为38%~50%(44.3±5.1)%,心功能分级(NYHA)Ⅱ级1例,Ⅲ级3例。1例为二次手术,1例为三次手术;4例患者均施行形态三尖瓣置换术,1例同期行主动脉瓣置换术+升主动脉成形术,1例同期行二尖瓣成形术。分析术后一周及术后随访患者的心功能,形态右心室射血分数和瓣膜状态等。结果无手术死亡,无瓣周漏,1例术中先行三尖瓣成形后仍有中度以上反流改行三尖瓣置换术,1例术后3周安装永久起搏器;体外循环时间72~245(143±73)分钟,主动脉阻断时间41~174(89±63)分钟;置换机械瓣膜型号25~27mm;呼吸机辅助时间16~86(39±31)小时,ICU时间16~168(93±72)小时。术后一周时形态右心室射血分数与术前比较(43.8±6.8)%vs(44.3±5.1)%,差异无统计学意义(P>O.05);随访4例,随访时间36~156(71±57)个月,随访时形态右心室射血分数与术前比较(43.0±12.2)%vs(44.3±5.1)%,差异无统计学意义(P>O.05);随访心功能分级(NYHA)I级1例,Ⅱ级2例,Ⅲ级1例。结论对形态三尖瓣大量返流儿童ccTGA患者,早期行瓣膜置换术能防止形态三尖瓣关闭不全对形态右心室功能的进一步损害;形态右心室功能明显下降后行三尖瓣膜置换对心功能改善不明显。

Objective To investigate the surgical indications and the clinical results of morphologictricuspid valve replacement(TVR)for children with congenitally corrected transposition of the great arteries(ccTGA).Methods From July 2007 to August 2017,4 cases under or equal to 14 yeas wih CCTGA weretreated in Fuwai Hospital.There were 3 male and l female,aged from 5.3 to l4 years(11.1±3.9)years andweighed from 20 to 44 kg(30±10)kg.There were 2 cascs complicated with sistus inversus viscerum,1complicated with single coronary artery and 1 complicated with morphologic mitral insufficiency.The preoperativemorphologe right ventricle ejection fraction was 38%-50%(44.3±5.1)%.Of the 4 cases,Ⅰ was in gladeⅡand 3were in gradeⅢaccording to Ncw York Heart classification(NYHA).The prior opcration were performed in 2patients,1 was the second and 1 was the third operation.4 patients had undergone morphologic TVR,associatedprocedures included aortic valve replacement+aortoplasty in l case and mitral valvuloplasty in l casc.The state of the prosthetic valve,cardiac function and morphological right ventricle cjection fraction were followed up.Results There was no death or perivalvular lcak in hospital.In l case,tricuspid valvuloplasty was initiallyattempted,but the regurgitation could not be sufficiently controlled,so the operation was swiched to TVR.Apermanent pacemaker was implanted in l case 3 weeks after the operation.The cardiopulmonary time and aortic-clamp time was 72-245 minutes(143±73)minutcs and 41-l74 minutes(89±63)minutes,respectively.Themechanical prosthesis diameter was 25-27 mm,the ventilator assist time was 16-86 hours(39=31)hours and thetime in ICU was 16-168 hours(93=72)hours.All the 4 patients survived with normal function of the prostheticvalve.There was no statistical significance between preoperative and postoperative mean morphologic rightventricle ejection fraction both 1 week after surgery or during the follow-up.During the follow-up of 36-156months(71±57)months.1 was in grade 1,2 were in gradc Ⅰand Ⅱ was in grade Ⅲ according to NYHA.Conclusion To preserve right ventricular function,early morphologic TVR may be a reasonable option even inthe management of serious tricuspid insufficiency in patients with ccTGA during childhood.Morphologic TVRdoes not improve right ventricular function in patients with a poor right ventricular function.