Abstract： Ebstein anomaly is a relatively rare congenital heart malformation which can affect heart function significantly. It may cause right heart failure, even whole heart failure and eventually death. In recent years, the surgery has made much progress in dealing with the abnormal valve, improving the right ventricle function and pretreatment of its related complications. However, because of its complexity and diversity in pathological anatomy and clinical manifestations, the disease has not got an “almighty standard” to treat all pathological anatomy types of the deformity, or asurgery strategy to solve the practical problems encountered in all clinic situations. Furthermore, the therapeutic effect is also unsatisfactory. This article will review the advance of treatment of Ebstein anomaly and prevention of its related complications.
Objective To improve surgical results, the experience of surgical management of Ebstein anomaly in 36 cases is reported and reviewed. Methods Surgical procedures included tricuspid valve replacement (6 cases), Danielson’s operation (28 cases), Carpentier’s operation (2 cases), among them, there were 5 cases of ablation of right atrioventricular accessory pathway. Results Four patients died early after operation in the hospital, 3 from right heart failure (1 case of tricuspid valve replacement and 2 cas...
Objective We sought to review our experience of cone reconstruction for tricuspid valve repair and to evaluate this therapeutic approach for patients with Ebstein's anomaly, to report early clinical outcomes. Method We retrospectively analyzed the clinical data of 19 consecutive patients underwent cone reconstruction technique in our hospital between December 2011 and June 2014 year. There were 8 males and 11 females at mean age 24 years(ranged from 3 months to 53 years). Six patients were diagnosed by electrocardiography or electrophysiology examination, including 4 patients with B type Wolff-Parkinson-White syndrome and atrial fibrillation, 2 patients with paroxysmal supraventricular tachycardia. Results There was one patient of severe cyanosis hospital death for low cardiac output syndrome depending on cardiopulmonary bypass. The remaining patients were recovered smoothly. Atrial fibrillation associated with frequent ventricular premature, restoration of sinus rhythm with lidocaine and amiodarone occurred in one patient. Early postoperative echocardiograms showed good ventricular morphology and reduction in tricuspid regurgitation (TR) grade. Eleven patients were with mild regurgitation and 7 patients without or with trace amounts of regurgitation. All patients were followed up for 5-30 months. And postoperative cardiac function recovered to gradeⅠin 10 patients and gradeⅡ in 8 patients. None of patients needed re-operation. Conclusions The cone reconstruction technique shows low in-hospital mortality and complication rate, reducing TR, restoring right ventricular function, allowing reverse remodeling of the heart. Early postoperative follow-up shows improvement in patients' clinical outcome and low incidence of re-operation.
ObjectiveTo summarize the clinical characteristics and outcome of tricuspid valve replacement (TVR) in children aged no more than 14 years, and to discuss the selection of prosthesis.MethodsFrom September 2002 to August 2019, 14 patients aged no more than 14 years who received TVR were included in our study. There were 9 males and 5 females, with a mean age of 9.8±4.3 years.ResultsMechanical prosthesis was implanted in 8 patients, and bioprosthesis in 6 patients. The mean cardiopulmonary time and aortic-clamp time was 170.3±109.8 min and 95.1±63.1 min, respectively. The mortality within 30 days after surgery was 21.4% (3/14), and all 3 patients died of severe low cardiac output syndrome. Eleven patients were followed up for 34-199 (100.1±57.4) months. During the follow-up, mechanical prosthesis dysfunction occurred in 3 patients, 2 of whom received secondary TVR. One patient died during the follow-up.ConclusionThe bioprosthesis is the first choice for TVR in children. Some long-term complications may occur after TVR, and close follow-up and timely intervention are needed.
ObjectiveTo analyze the Ebstein anomaly's reoperative strategy and mid- to long-term results.MethodsWe 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.ResultsAmong 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.ConclusionThe 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.
ObjectiveTo 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).MethodsThe 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.ResultsTwenty-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.ConclusionTricuspid 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.