Ebstein畸形的外科治療策略,Ebstein畸形是罕見復(fù)雜的心臟先天畸形,發(fā)生率1:40,000-200,000先天性心臟病中: 1.5 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,Operative technique,Repair consisted of TV repair Reduction atrioplasty Relief of RVOT obstruction Partial closure of ASD Correction of all associated cardiac defects,新生兒Ebstein畸形的治療,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 commissureand bringing this down to the CS,thus creating adouble orifice valve. The lateral orifice containing the atrialized RV, which be closed byplicating it vertically.,If the large anterior leaflet does not coapt wellwith the ventricular septum, a pledgetted suturefrom the anterior papillary muscle to the ventricularseptum may be used to correct this,新生兒Ebstein畸形的治療,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 theatrialized from the functional right ventricle from A to B,新生兒Ebstein畸形的治療,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.,新生兒Ebstein畸形的治療,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 RVOTand the functional RV. Fenestrating the A-S commissure and leaflet prevents tricuspid stenosis,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,新生兒Ebstein畸形的治療,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,外科矯治新觀點(diǎn)（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; and3 A limited plication performed only at the level of the displaced valve rather than complete plication of the entire atrialized RV.,New conecpts: Depending specific physiologic and anatomic criteria for selective use of the BDG in conjunction with repair of Ebsteins anomaly.,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.,Patient Characteristics,Age: 7 months to 40.4 yearsexercise intolerance in 40cyanosis in 26RV failure in 18atrial dysrhythmias in 8TR was moderate or severe in 50 patients (87.7%).,Approaches to the Tricuspid Valve,1 The detrimental effects of a very large tricuspid annulus,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.,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, 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.,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.,Concomitant Procedures Performed at Initial Ebsteins Anomaly RepairProcedures 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,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.,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).,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).,我院情況,1996年10月2005年10月 151例，死亡7例心胸比0.7,26例,術(shù)前TV均為大量返流 5月-60歲，8.3-58kg 死亡5例，2例ECMO（均存活）,手術(shù)方法,單純Glenn （常溫1例） 3例GlennDanielson成形 1例 GlennCarpentier成形 1例 GlennDevega環(huán)縮 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環(huán)縮 3例,2例ECMO,術(shù)后早期TV返流，右心功能嚴(yán)重衰竭，左心受累右心EF:2025%、1015 ECMO支持時(shí)間8天、5天右心明顯縮小：60 39mm左心功能明顯好轉(zhuǎn)，EF：45 70,外科策略和原則-總結(jié)和得到的啟發(fā),RV功能好盡可能雙心室矯治（ASD開窗）新生兒期后可增加Glenn手術(shù)保證右心有效減壓(一個(gè)半心室）急診新生兒Starnes或改良矯治是另一種選擇（降低死亡率）雙心室和一個(gè)半心室矯治，RVOTS和PS必須解除對(duì)于嚴(yán)重右心衰竭影響左心的患者，可短期ECMO支持，待左心功能恢復(fù)后可逐漸脫離晚期患者可考慮心臟移植,謝謝,