1、Ebstein畸形的外科治療策略,1,學習交流PPT,Ebstein畸形是罕見復雜的心臟先天畸形,發(fā)生率1:40,000-200,000 先天性心臟病中: 1% 疾病譜寬： 輕型 無癥狀 重癥 新生兒期死亡率極高 手術(shù)死亡率高,Wilhelm Ebstein 1866 年首先描述形態(tài) Helen Taussig 1950 年描述臨床特點,2,學習交流PPT,解剖學特點,Displacement of the septal and posterior leaflets of the TV toward the apex of the RV. Although the anterior leafl

2、et 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,學習交流PPT,3. The segment of the RV from the level of the true tricuspid annulus to the level of attachment of the septal and posterior leaflet

3、s 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,學習交流PPT,5. The infundibulum is often obstructed by the redundant tissue of the an

4、terior leaflet as well as by the chordal attachments of the anterior leaflet to the infundibulum.,5,學習交流PPT,臨床分型（分級）,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

5、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 c

6、ommissure of the tricuspid valve.,6,學習交流PPT,超聲評估分級,面積比值右房房化右室/功能右室左心房室 心臟舒張期四腔心軸面 1級： 1.5,7,學習交流PPT,病理生理特點：,1. 三尖瓣關(guān)閉不全 右房明顯擴大，卵圓孔右向左分流，右室擴大 2. 右室功能不良 有效收縮部分減少，心室膨脹 3. 肺動脈發(fā)育不良 三尖瓣前葉、乳頭肌阻擋，生理性PAA 4. 左室受壓，呈“夾心餅”，功能受限 5. 可伴有室上性或室性心律,8,學習交流PPT,臨床表現(xiàn)：,容易疲勞 ，活動后呼吸困難、心悸，紫紺 Giuliani 67例非手術(shù)， 12年觀察： 39% NYHA 1-

7、2級 61% NYHA 3-4級 21%病人死亡 死亡病人有一項或多項特點： 1.NYHA 3-4級 2.心胸比大于0.65 3.發(fā)紺或動脈氧飽和90%以下 4.明確診斷時處于嬰兒階段,9,學習交流PPT,術(shù)前基礎(chǔ)治療：,1.保持PDA開放，增加肺內(nèi)血供，改善氧合：PGE1 2.糾正酸中毒 3.充分鎮(zhèn)靜，過度通氣，降低肺血管阻力,10,學習交流PPT,治療原則：,1.盡可能恢復三尖瓣功能 2.右房減容，改善呼吸功能 3.根據(jù)右室功能決定： 雙心室矯治 右室曠置 右室減負荷 4.房化心室是否去除（折疊或切除）？ 5.右室流出道充分疏通,11,學習交流PPT,外科技術(shù)：,三尖瓣成形（包括心室成形）

8、技術(shù) 1.Danielson修復 2.改良Carpentier技術(shù) 3.Devega技術(shù) 4.前葉單瓣技術(shù),12,學習交流PPT,三尖瓣成形技術(shù),1.Danielson 修復,Ebstein畸形的治療,13,學習交流PPT,2.改良Carpentier修復,Ebstein畸形的外科治療,14,學習交流PPT,3.改良Devega技術(shù),runing both ends of the pledgetted suture in and out along the annulus separating the atrialized from the functional right ventricle

9、 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

10、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,學習交流PPT,4.前瓣單葉修復,Ebstein畸形的外科治療,16,學習交流PPT,重癥Ebstein畸形的定義,目前不明確 參考標準 Pr

11、edictors 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 infan

12、cy) Knott-Craig CJ et al. Ann Thorac Surg 2002,76:1786,17,學習交流PPT,新生兒Ebstein畸形的治療,Starnes矯治(J Thorac Cardiovasc Surg 1991:101;1082-7) 5 consecutive newborn infants Age: 1-9 days. Weight : 3.61.8 kg Mean PH: 7.20.05 Mean oxygen tension: 29.62.3 mmHg Mean cardiothoracic ration: 0.810.02 ECHO: severe t

13、ricuspid regurgitation functional pulmonary atresia in all patients All patients were resuscitated with intubation and mechanical ventilation, acidosis was corrected, and therapy with PGE1.,18,學習交流PPT,Preoperative echo assessment patient No. 1 2 3 4 5 RV dysplasia + + 0 0 + tethered anterior leaflet

14、 0 0 + 0 + Echo score ratio 1.3 0.9 0.8 0.6 1.01 severe TR + + + + + functional pulmonary atresia + + + + +,19,學習交流PPT,Cardiac catheterization assessment in one neonates,20,學習交流PPT,Operative technique,The tricuspid orifice was closed with autologous pericardium. The coronary sinus beneath the patch

15、to reduce the risk of AV block.,An ASD was created to ensure mixing at the atrial level.,21,學習交流PPT,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,學習交流PPT,Results,No perioperative and late deaths. N

16、o postoperative arrhythmias. Mechanical ventilation time 10.20.3days. Po2: 42.20.9mmHg, SO2: 83.21.9%,23,學習交流PPT,Follow-up,One received a Glenn operation after 11 mo. Two received Fontan procedures at 23 and 22 mo of age.,24,學習交流PPT,雙心室矯治(Knott-Craig CJ. Repair of Ebsteins anomaly in the symptomatic

17、 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),新生兒Ebstein畸形的治療,25,學習交流PPT,Preoperative assessment,Severe (

18、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,新生兒Ebstein畸形的治療,26,學習交流PPT,Operative technique,Repair consis

19、ted of TV repair Reduction atrioplasty Relief of RVOT obstruction Partial closure of ASD Correction of all associated cardiac defects,新生兒Ebstein畸形的治療,27,學習交流PPT,Tricuspid valve repair ( 3 had Danielson-type repairs, 3 had DeVega-type repairs, and 2 had complex repairs),1.modified Danielson technique

20、,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 ple

21、dgetted suture from the anterior papillary muscle to the ventricular septum may be used to correct this,新生兒Ebstein畸形的治療,28,學習交流PPT,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

22、 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,新生兒Ebstein畸形的治療,29,學習交流PPT,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 comm

23、issure 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.,新生兒Ebstein畸形的治療,30,學習交流PPT,Detaching the entire anterior and posterior leaflets from the annulus,Freeing the leaflets fr

24、om 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 preve

25、nts tricuspid stenosis,31,學習交流PPT,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 pl

26、eural cavity and leave a drain in the peritoneal cavity,新生兒Ebstein畸形的治療,32,學習交流PPT,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,學習交流PPT,外科矯治新觀點（Sunil P. Malhotra MD, S

27、elective 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 w

28、hich 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

29、 the entire atrialized RV.,34,學習交流PPT,New conecpts: Depending specific physiologic and anatomic criteria for selective use of the BDG in conjunction with repair of Ebsteins anomaly.,35,學習交流PPT,Patient Characteristics,93.12-08.12 57consecutive patients outside of the neonatal period The diagnosis of

30、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 classifi

31、ed 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,學習交流PPT,Patient Characteristics,Age: 7 months to 40.4 years exercise intolerance in 40 cyanosis in 26 RV f

32、ailure in 18 atrial dysrhythmias in 8 TR was moderate or severe in 50 patients (87.7%).,37,學習交流PPT,Approaches to the Tricuspid Valve,1 The detrimental effects of a very large tricuspid annulus,38,學習交流PPT,Approaches to the Tricuspid Valve,2 The goal of plication of the atrialized RV,The “play it wher

33、e 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,

34、effectively creating a bicuspid valve.,39,學習交流PPT,3 Selective use of the BDGusing 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, thi

35、s 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 press

36、ure 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,學習交流PPT,The second circumstance for placing a B

37、DG 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 se

38、paration 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 redu

39、ce the functional valve orifice as much as necessary to achieve a stable, competent valve repair.,41,學習交流PPT,Concomitant Procedures Performed at Initial Ebsteins Anomaly RepairProcedures No.Electrophysiologic procedures 8Ablation of accessory pathway 2Maze procedures Bilatera l2 With pacemaker 1 Rig

40、ht-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,學習交流PPT,Results,No early or late deaths occurred. Early reoperation was required in 2 patients. 1 patient required pacemaker p