Ebstein畸形的外科治疗

单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,Ebstein,畸形的外科治疗策略,1,Ebstein畸形是罕见复杂的心脏先天畸形,发生率,1:40,000-200,000,先天性心脏病中,: 1%,疾病谱宽：,轻型 无症状,重症 新生儿期死亡率极高,手术死亡率高,Wilhelm Ebstein 1866 年首先描述形态,Helen Taussig 1950 年描述临床特点,2,解剖学特点,Displacement of the,septal,and posterior leaflets of the TV toward the apex of the RV.,Although the anterior leaflet is attached at the appropriate level of the tricuspid annulus, it is larger than normal and may have multiple,chordal,attachments to the ventricular wall.,3,3. The segment of the RV from the level of the true tricuspid annulus to the level of attachment of the septal and posterior leaflets is unusually thin and dysplastic. The tricuspid annulus and the RA are extremely dilated.,4. The cavity of the functional RV is reduced in size, usually lacks an inlet chamber, and has a small trabecular component.,4,5.,The infundibulum is often obstructed by the redundant tissue of the anterior leaflet as well as by the chordal attachments of the anterior leaflet to the infundibulum.,5,临床分型（分级）,type A:,the volume of the true RV is adequate.,type B：,there is a large atrialized component of,the RV, but the anterior leaflet moves freely.,type C:,the anterior leaflet is severely restricted in its movement and,may cause signficant obstruction of the RVOT.,type D:,there is almost complete atrialization of the ventricle with the,exception of a small infundibular component.The only communication,between the atrialized ventricle and the infundibulum is through the,anteroseptal commissure of the tricuspid valve.,6,超声评估分级,面积比值右房房化右室/功能右室左心房室,心脏舒张期四腔心轴面,1级： 1.5,7,病理生理特点：,1. 三尖瓣关闭不全,右房明显扩大，卵圆孔右向左分流，右室扩大,2. 右室功能不良,有效收缩部分减少，心室膨胀,3. 肺动脉发育不良,三尖瓣前叶、乳头肌阻挡，生理性PAA,4. 左室受压，呈“夹心饼”，功能受限,5. 可伴有室上性或室性心律,8,临床表现：,容易疲劳 ，活动后呼吸困难、心悸，紫绀,Giuliani 67例非手术， 12年观察：,39% NYHA 1-2级,61% NYHA 3-4级 21%病人死亡,死亡病人有一项或多项特点：,1.NYHA 3-4级,2.心胸比大于0.65,3.发绀或动脉氧饱和90%以下,4.明确诊断时处于婴儿阶段,9,术前基础治疗：,1.保持PDA开放，增加肺内血供，改善氧合：PGE1,2.纠正酸中毒,3.充分镇静，过度通气，降低肺血管阻力,10,治疗原则：,1.尽可能恢复三尖瓣功能,2.右房减容，改善呼吸功能,3.根据右室功能决定：,双心室矫治 右室旷置 右室减负荷,4.房化心室是否去除,（折叠或切除）,？,5.右室流出道充分疏通,11,外科技术：,三尖瓣成形（包括心室成形）技术,1.Danielson修复,2.改良Carpentier技术,3.Devega技术,4.前叶单瓣技术,12,三尖瓣成形技术,1.Danielson 修复,Ebstein畸形的治疗,13,2.改良Carpentier修复,Ebstein畸形的外科治疗,14,3.改良Devega技术,runing both ends of the pledgetted suture,in and out along the annulus separating the,atrialized from the functional right ventricle,from A to B,the anterior leaflet is not large or if the posterior leaflet,is well developed or if both the anterior and posterior,leaflets are functional but dysplastic,The “play it where it lies” approach involves limited plication,of the tricuspid valve. Points A and B are approximated,with 1 or 2 mattress sutures at the level of the native valve,not to the level of the true tricuspid annulus. This results in,approximating the apical aspects of the septal and anterior,leaflets, effectively creating a bicuspid valve.,15,4.前瓣单叶修复,Ebstein畸形的外科治疗,16,重症,Ebstein,畸形的定义,目前不明确,参考标准,Predictors of Death in neonates with Ebsteins Anomaly,cardiothoracic ration greater than 0.85 ( 100% fatal),Echocardiography score grade 4/4 ( 100% fatal),Echocardiography score grade and cyanosis(100%fatal),Severe tricuspid regurgitation (mostly fatal),Echocardiography score grade (45% fatal in infancy),Knott-Craig CJ et al. Ann Thorac Surg 2002,76:1786,17,新生儿,Ebstein,畸形的治疗,Starnes,矫治,(J,Thorac,Cardiovasc,Surg,1991:101;1082-7),5 consecutive newborn infants,Age: 1-9 days.,Weight : 3.6,1.8 kg,Mean PH: 7.20.05,Mean oxygen tension: 29.62.3 mmHg,Mean cardiothoracic ration: 0.810.02,ECHO: severe tricuspid regurgitation,functional pulmonary,atresia,in all patients,All patients were resuscitated with,intubation,and mechanical,ventilation, acidosis was corrected, and therapy with PGE1.,18,Preoperative echo assessment,patient No.,1 2 3 4 5,RV,dysplasia,+ + 0 0 +,tethered anterior leaflet,0 0 + 0 +,Echo score ratio 1.3 0.9 0.8 0.6 1.01,severe TR,+ + + + +,functional pulmonary,atresia,+ + + + +,19,Cardiac catheterization assessment in one neonates,20,Operative technique,The tricuspid orifice was closed with,autologous,pericardium.,The coronary sinus beneath the patch,to reduce the risk of AV block.,An ASD was created to ensure mixing,at the,atrial,level.,21,The right atrium was reduced in size,by removing a segment of the right,atrial free wall.,A A-P shunt was established with,a 4mm Gore-Tex vessel.,22,Results,No perioperative and late deaths.,No postoperative arrhythmias.,Mechanical ventilation time 10.2,0.3days.,Po,2,: 42.2,0.9mmHg, SO,2,: 83.21.9%,23,Follow-up,One received a Glenn operation,after 11 mo.,Two received Fontan procedures,at 23 and 22 mo of age.,24,双心室矫治,(,Knott-Craig CJ.,Repair of,Ebsteins,anomaly in the symptomatic neonate: an evolution of technique with 7-year follow-up .,Ann,Thorac,Surg,2002:73;1786-93),8 symptomatic patients,6 neonates ( 2-19d, 2.8-3.2kg ),1 young infant (2mo, 3.8kg) had undergone,a,starnes,operation elsewhere,1 infant (4mo, 6.4kg),新生儿Ebstein畸形的治疗,25,Preoperative assessment,Severe (4/4) TR was present in all except 1,(Starnes operation),Cardiothoracic ratio exceeded 0.85 in all patients,Echocardiography severity scores were,1.5 in 6 (grade 4/4),and 1.3 in 1 (grade 3/4),3,patients had anatomical PA,2 had functional PA,新生儿Ebstein畸形的治疗,26,Operative technique,Repair consisted of,TV repair,Reduction atrioplasty,Relief of RVOT obstruction,Partial closure of ASD,Correction of all associated cardiac defects,新生儿Ebstein畸形的治疗,27,Tricuspid valve repair,(,3 had Danielson-type repairs, 3 had,DeVega,-type repairs, and 2 had complex repairs),1.modified Danielson technique,placing a pledgetted suture at the A-P commissure,and bringing this down to the CS,thus creating a,double orifice valve.,The lateral orifice,containing the atrialized RV, which be closed by,plicating it vertically.,If the large anterior leaflet does not coapt well,with the ventricular septum, a pledgetted suture,from the anterior papillary muscle to the ventricular,septum may be used to correct this,新生儿Ebstein畸形的治疗,28,2.DeVega-type annuloplasty,(the anterior leaflet is,not large or if the posterior leaflet is well developed or if both the,anterior and posterior leaflets are functional but dysplastic ),runing both ends of the pledgetted suture,in and out along the annulus separating the,atrialized from the functional right ventricle,from A to B,新生儿Ebstein畸形的治疗,29,In the more severe forms of EA in the neonate,1.The orifice of the TV is toward the apex,of the RV.,2.The commissure between the anterior and,septal leaflets may be imperforate or patent,only through small fenestrations.,3.The posterior leaflet may be reasonably,well developed and mobile.,新生儿Ebstein畸形的治疗,30,Detaching the entire anterior and,posterior leaflets from the annulus,Freeing the leaflets from their,muscularized attachments and,reducing the annulus in size posteriorly,Reattaching the leaflets to the smaller annulus,not only corrects the defect but also effectively,changes the orientation of the TV back to the RVOT,and the functional RV. Fenestrating the A-S,commissure and leaflet prevents tricuspid stenosis,31,Correction of all associated cardiac defects,PA,、,PS or RVOTS:,RVOT patch or a small homograft or other,valved,conduit,VSD: more complex,Unloading the RV,Fenestrated ASD closure,Adding the hemi-,Fontan,connection (in older patients),Reduction,atrioplasty,Open right pleural cavity and leave a drain in the,peritoneal cavity,新生儿Ebstein畸形的治疗,32,Results,One patient died in hospital,no late deaths,All are in sinus rhythm and in functional class I,4 patients had trace to mild TR and 2 had mild to,moderate regurgitation,33,外科矫治新观点,（,Sunil P.,Malhotra,MD, Selective Right Ventricular Unloading and Novel Technical Concepts in,Ebsteins,Anomalys,San Francisco, CA, Jan 2628, 2009.,）,New conecpts:,Using of valve reconstructive techniques that differ substantially from those in the literature:,1 A “play it where it lies” approach to the tricuspid valve in which the reconstruction is performed at the functional orifice instead of moving the valve to the anatomic tricuspid annulus;,2 Avoidance of detachment and reimplantation of valve leaflets; and,3 A limited plication performed only at the level of the displaced valve rather than complete plication of the entire atrialized RV.,34,New conecpts:,Depending specific physiologic and anatomic criteria for selective use of the BDG in conjunction with repair of Ebsteins anomaly.,35,Patient Characteristics,93.12-08.12 57consecutive patients outside of the neonatal period,The diagnosis of severe Ebsteins anomaly of the tricuspid valve was established by echocardiography in all patients.,Echocardiography was used to characterize the degree of apical displacement of the tricuspid annulus, the severity and nature of TR, and the degree of mobility of the anterior leaflet.,TR was classified on a scale of 1 to 4 (1, trace; 2, mild; 3, moderate, and 4, severe).,Echocardiography also was used to assess right and left ventricular function and to identify any atrial level shunts.,36,Patient Characteristics,Age: 7 months to 40.4 years,exercise intolerance in 40,cyanosis in 26,RV failure in 18,atrial dysrhythmias in 8,TR was moderate or severe in 50 patients (87.7%).,37,Approaches to the Tricuspid Valve,1,The detrimental effects of a very large tricuspid annulus,38,Approaches to the Tricuspid Valve,2,The goal of plication of the atrialized RV,The “play it where it lies” approach involves limited plication of the tricuspid valve. Points A and B are approximated with 1 or 2 mattress sutures at the level of the native valve, not to the level of the true tricuspid annulus. This results in approximating the apical aspects of the septal and anterior leaflets, effectively creating a bicuspid valve.,39,3,Selective use of the BDG,using the BDG in two separate and independent circumstances.,The first is physiologic. Cyanosis at rest is a marker for an inadequate RV pump. If the patient is fully saturated at rest but becomes cyanotic with exercise, this is a relative marker of an inadequate RV pump, and we will have a low threshold for placing a BDG. Typically, we will separate the patient from cardiopulmonary bypass after valve repair and monitor right and left atrial pressure. If the right atrial pressure exceeds 1.5 times the left atrial pressure under these relatively unstressed conditions of an open chest in an anesthetized patient, we will perform a BDG.,If the patient presents with an intact atrial septum or an atrial septal defect with left-to-right shunting, a BDG is not performed.,40,The second circumstance for placing a BDG is anatomic and relates to the ultimate size of the functional tricuspid annulus after repair. If it is necessary to make the functional tricuspid orifice substantially less than 2.5 cm (in a 70-kg patient) to achieve a competent valve, we will assess inflow velocity across the tricuspid after separation from cardiopulmonary bypass using transesophageal echocardiography. If obstruction is demonstrated, a BDG is placed. We acknowledge that many of the maneuvers used to make a regurgitant valve competent involve reducing the valve opening. This option for BDG use frees us to aggressively reduce the functional valve orifice as much as necessary to achieve a stable, competent valve repair.,41,Concomitant Procedures Performed at Initial Ebsteins Anomaly Repair,Procedures No.,Electrophysiologic procedures 8Ablation of accessory pathway 2Maze,procedures Bilatera l2 With pacemaker 1 Right-sided 3 With pacemaker 1Pacemaker alone 1Partial anomalous pulmonary vein repair 1 Pulmonary valve replacement 1 Relief of RV outflow tract obstruction 2 Supravalvar pulmonary stenosis repair 1,42,Results,No early or late deaths occurred.,Early reoperation was required in 2 patients.,1 patient required pacemaker placement for atrioventricular nodal block and 1 patient required placement of an CRT for recurrent ventricular arrhythmias.,At follow-up echocardiography, RV systolic function was normal in 52 patients, mildly reduced in 3, and moderately reduced in 2 patients.,43,Severity of tricuspid regurgitation is shown before and after repair.,NYHA status improved from 2.3 0.5 preoperatively,to 1.0 0.2 at follow-up (p = 0.0002).,44,Outcomes of “One and a Half Ventricle” Repairs,31 cases,pre: SO2 89.5% 5.9% vs 96.2% 3.9%,p,= 0.01,NYHA 2.5 0.6 vs 2.16 0.4,p,= 0.0025,There were no BDG-related complications,The mean saturation was 96.9% 3.0%,NYHA functional status in this cohort improved,to 1.0 0.2 (p = 0.0002).,45,我院情况,1996,年,10,月,2005,年,10,月,151,例，死亡,7,例,心胸比,0.7,26,例,术前,TV,均为大量返流,5,月,-60,岁，,8.3-58kg,死亡,5,例，,2,例,ECMO,（均存活）,46,手术方法,单纯,Glenn,（常温,1,例）,3,例,Glenn,Danielson,成形,1,例,Glenn,Carpentier,成形,1,例,Glenn,Devega,环缩,1,例,死亡,全腔改,Glenn 1,例,死亡,TVR,（,包括,TVR,Glenn 1,例,）,5,例,死亡,3,例,Danielson,成形,（包括,RVOTS,疏通,2,例,),6,例,Carpentier,成形,5,例,Devega,环缩,3,例,47,2例ECMO,术后早期,TV,返流，右心功能严重衰竭，左心受累,右心,EF:20,25%,、,10,15,ECMO,支持时间,8,天、,5,天,右心明显缩小：,60 39mm,左心功能明显好转，,EF,：,45 70,48,外科策略和原则-总结和得到的启发,RV,功能好尽可能双心室矫治（,ASD,开窗）,新生儿期后可增加,Glenn,手术保证右心有效减压,(,一个半心室）,急诊新生儿,Starnes,或改良矫治是另一种选择（降低死亡率）,双心室和一个半心室矫治，,RVOTS,和,PS,必须解除,对于严重右心衰竭影响左心的患者，可短期,ECMO,支持，待左心功能恢复后可逐渐脱离,晚期患者可考虑心脏移植,49,谢谢,50,