Clinical analysis of outcomes of different surgery methods for Ebstein anomaly in a single center / 中国胸心血管外科临床杂志

@#Objective    To investigate the indications and clinical effects of tricuspid cone reconstruction and tricuspid valve replacement in the treatment of downward displacement of tricuspid valve (Ebstein anomaly). Methods    The clinical data of 22 patients with Ebstein anomaly who underwent surgical treatment in our hospital from January 2013 to March 2020 were collected. There were 7 males and 15 females, aged 4-56 (33.68±17.78) years. The patients were divided into two groups according to different surgical methods: a tricuspid cone reconstruction group (tricuspid valvuloplasty group, n=12) and a tricuspid valve replacement group (n=10). The curative effect of the two operative methods were compared. Results    Twenty-two patients underwent surgical treatment under general anesthesia and cardiopulmonary bypass. One patient died of severe low cardiac output syndrome during operation. Downward displacement of tricuspid septum was found in 22 patients, and downward displacement of tricuspid septum and posterior septum in 21 patients, downward displacement of tricuspid in 8 patients. Twelve patients were treated with tricuspid  valvuloplasty, 10 patients with tricuspid valve replacement, and patients with other intracardiac structural malformations were treated at the same time. Postoperative cardiac doppler ultrasound indicated no tricuspid regurgitation in 9 patients, mild regurgitation in 8 patients, and moderate regurgitation in 4 patients. No perivalvular leakage occurred in all patients undergoing valve replacement. Four patients developed degree Ⅲ atrioventricular block after operation, among whom 3 patients recovered to sinus rhythm and 1 patient implanted the permanent pacemaker. Twenty-one patients were cured and discharged after successful operation, and were followed up for 3 to 78 months, with a follow-up rate of 100.0%. During the follow-up period, there was no severe tricuspid regurgitation in tricuspid valvuloplasty group, but mild or moderate regurgitation was found. After tricuspid valve replacement, only 1 patient had mild regurgitation, and the rest patients had no valve regurgitation. Conclusion    Tricuspid valvuloplasty and tricuspid valve replacement are effective in the treatment of Ebstein anomaly, and the tricuspid regurgitation is less severe after tricuspid valve replacement operation. The operation method should be selected according to the different anatomical characteristics and condition of tricuspid valve. The combined intracardiac malformation or arrhythmia can be dealt with simultaneously.

Left ventricular outflow tract obstruction secondary to cone reconstruction for Ebstein’s anomaly: A case report / 中国胸心血管外科临床杂志

@#Left ventricular outflow tract obstruction (LVOTO) in Ebstein's anomaly is a rare complication, and LVOTO related to surgery is rarer. We present a 46 years old female patient who was dignosed with Ebstein's anomaly, then suffered from cardiac arrest because of LVOTO secondary to cone reconstruction in ICU.

Reoperative strategy and mid- to long-term surgical outcomes of Ebstein anomaly / 中国胸心血管外科临床杂志

@#Objective    To analyze the Ebstein anomaly's reoperative strategy and mid- to long-term results. Methods    We retrospectively reviewed the clinical data of 23 patients who diagnosed with Ebstein anomaly and underwent reoperation for tricuspid valve insufficiency between July 2002 and July 2017 in Fuwai Hospital. There were 9 (39.1%) males and 14 (60.9%) females, with a median age of 28.0 (19.0, 45.0) years. Results    Among the 23 patients, 8 (34.8%) underwent tricuspid valvuloplasty and 15 (65.2%) underwent tricuspid valve replacement. The rate of valvuloplasty was 16.7% before 2012, and 54.5% after 2012 (P=0.089) as Cone reconstruction procedure was used. In the valvuloplasty cohort, 3 (37.5%) patients were treated with Danielson or Carpentier technique, and 5 (62.5%) patients were treated with Cone reconstruction procedure. There was no operation-related death. Early complications occurred in 3 (37.5%) patients. The median follow-up was 6.9 years (range, 3.0-15.1 years), and no adverse cardiac events occurred. In the patients with valve replacement, 7 (46.7%) received mechanical prosthesis and 8 (53.3%) received bio-prosthesis. There was no operation-related death. And early complications were observed in 3 (20.0%) patients. The median follow-up was 6.5 years (range, 2.5-15.3 years). One (6.3%) patient died and 4 (26.7%) had long-term complications during the follow-up period. Conclusion    The mid- to long-term outcomes are convincing in patients who undergo the second operation due to recurrent tricuspid regurgitation of Ebstein anomaly. A low incidence of reoperation is observed. Cone reconstruction procedure provides possibilities of second tricuspid valvuloplasty, and this technique can reduce the rate of tricuspid valve replacement in the second operation. Tricuspid valve replacement is still an alternative method for the treatment of recurrent tricuspid regurgitation in patients with Ebstein anomaly. The bioprosthetic prosthesis may be a better choice than mechanical prosthesis.

Perioperative risk factors for cone reconstruction of Ebstein's anomaly / 中华胸心血管外科杂志

Objective The study was designed to analyze the risk factors for perioperative adverse events in Ebstein's a-nomaly(EA) after cone construction.Methods A retrospective analysis was performed on EA cases undergoing cone con-struction in pediatric heart center of Beijing Anzhen Hospita from January 2010 to January 2017.Patients were divided into group A( no adverse events occurred) and group B( adverse events occurred) according to the presence or not of perioperative adverse events.Univariate and multivariate logistic regression analysis was performed with 9 preoperative and intraoperative var-iables as the predicted risk factors.Results The incidence of adverse events was 13.1％.Univariate logistic regression analy-sis showed that preoperative percutaneous oxygen saturation(SpO2)≤0.90(P=0.001, OR=10.578) and extracorporeal cir-culation time(P=0.034, OR=1.021) were risk factors for perioperative adverse events.Results of multivariate logistic re-gression analysis: preoperative SpO2≤0.90 was an independent risk factor for perioperative adverse events(P=0.002, OR=9.108).In 64 cases(64.6％) with atrial septum defect, there was no significant correlation between preoperative SpO2 and preoperative tricuspid regurgitation(P=0.838, r =0.026).Conclusion Preoperative SpO2≤0.90 is an independent risk factor for adverse events in the perioperative period of cone reconstruction .It is a simple and efficient method to evaluate the operation difficulty and determine the prognosis by SpO 2 .

Early and midterm results of Ebstein′s anomaly with the treatment:the cone reconstruction for the tricuspid valve / 实用医学杂志

Objective To evaluate the early and midterm effect by retrospective analysis of the cone reconstruction in Ebstein′s anomaly. Methods From January 2012 through June 2016,16 patients with Ebstein′s anomaly underwent the cone reconstruction in our hospital. There were 7 males and 9 females at mean age(28.21 ± 11.88)years,including 12 patients of Carpentier B type,4 patients of Carpentier C type. There were 9 patients in New York Heart Association(NYHA)functional class Ⅲ,5 patients in NYHA functional class Ⅳand the others in NYHA functional class Ⅱ. All patients had Tricuspid insufficiency ,including 2 patients with Grade 2 ,10 patients with Grade 3 and 4 patients with Grade 4. All patients were followed up over a period of 6 to 51 months (mean:24.36 ± 10.71). By comparing the details of pre-operation with post-operation and post-operation with current stage,we analyze the outcomes of early and midterm effects. Results All patients are well survived. There were one patient recovering from the low cardiac output syndrome with adding the strength of the positive inotropic drug in the first day after operation ,and one patient recovering from bigeminy of ventricular prematurebeat with the aid of amiodarone and lidocaine the third day after operation. The remaining patients recovered smoothly. Early post-operative echocardiograms showed good right ventricular morphology and reduction in tricuspid regurgitation grade from(3.13 ± 0.62)to(1.75 ± 0.45)(P<0.001)with the functional class improved from(3.19 ± 0.66)to(2.00 ± 0.52)(P<0.001). After mean follow-up of(24.36 ± 10.71)months,the situation is improving furtherly with the tricuspid regurgitation grade reducing to(1.31 ± 0.48)(P=0.016)and the functional class rising to(1.50 ± 0.52) (P = 0.031). Conclusions This surgical technique for Ebstein′s anomaly can improve the function of tricuspid valve and the right ventricular morphology. It showed a satisfactory performance in the early and midterm results by enhancing the Cardiac function and life quality. But indications should be strictly grasped and more experiences need to be accumulated. Long-term effect need to be furtherly explored.

Modified cone reconstruction in the treatment of Ebstein’s anomaly in 18 patients / 中国胸心血管外科临床杂志

@#Objective    To investigate the early and mid-term clinical outcomes of the modified cone reconstruction in the treatment of Ebstein’s anomaly (EA). Methods    Clinical data of 18 consecutive patients with EA in our hospital between May 2008 and August 2015 were retrospectively analyzed. All patients were diagnosed by echocardiography. There were 8 males and 10 females with an average age of 20.3 years ranging from 5 to 41 years. According to New York Heart Association classification, 12 patients were classified into grade Ⅱ and 6 grade Ⅲ. One patient had acute arterial embolism and amputation of left lower extremity caused by paradoxical embolism of combined secundum atrial septal defect, and another one was combined with double-orifice technique due to postoperative poor closure of tricuspid valve. The modified cone reconstruction was used to correct the EA, to make leaflets coapted well and form central blood flow. For those patients whose anterior leaflet developed poor and smaller, valve leaflet was widened by using autologous pericardial. For all patients, tricuspid annulus was reinforced by autologous pericardial. Results    Two patients suffered arrhythmia, and returned to normal after medication. The rest patients recovered well without death. Echocardiography found 1 patient with moderate regurgitation and the rest of patients’ leaflets coapted well and had no tricuspid stenosis. They were followed up 9 to 38 months postoperatively, and cardiac function of gradeⅠin 14 patients and gradeⅡin 4 patients. Conclusion    The early and mid-term clinical outcomes of the modified cone reconstruction in the treatment of EA are affirmative which can make leaflets coapt completely and have a strong anti-regurgitation ability, reducing the incidence of re-operation, valve replacement and postoperative mortality.

A Case of Cone Reconstruction at Primary Biventricular Repair for Neonatal Ebstein Anomaly / 日本心臓血管外科学会雑誌

<p>There are few reports of cone reconstruction for neonatal Epstein's anomaly. We report 14-day-old girl with symptomatic Ebstein's anomaly with prenatal diagnosis prenatally and who was born by caesarean section. Continuous infusion of prostaglandin was started immediately and she was transferred to our institution. The cardiothoracic ratio (CTR) was 74% on chest roentgenograph. The echocardiograph showed severe tricuspid valve regurgitation (TR) and functional pulmonary atresia. Despite aggressive medical therapy, congestive heart failure had advanced and early surgical intervention was required. The anterior leaflet was severely plastered to free the wall, like the Carpentier type C. After cone reconstruction, the intraoperative echocardiography showed mild to moderate TR, antegrade flow to the pulmonary arteries and good function of both ventricles. The patient was extubated on the 6th postoperative day and stayed in the intensive care unit for another 2 days. The CTR was 58%, and the echocardiography showed a well-functioning RV with mild to moderate TR and a right to left transatrial shunt flow with an arterial saturation level of over 90%. Cone reconstruction might even be effective in such border line cases of biventricular repair with dysplastic anterior leaflet of tricuspid valve.</p>