Surgical treatment of Ebstein’s deformity in adults

Ebstein’s malformation (tricuspid valve subluxation malformation) is a rare and complex cardiac malformation, and there are few clinical reports on the surgical treatment of Ebstein’s malformation in adults. In order to further explore the indications, surgical methods and prognosis of Ebstein malformation in adults, we summarize our experience in surgical treatment of this malformation. 1. Subjects: From April 2002 to August 2006, 19 patients (10 males and 9 females) with adult Eb2stein malformation were admitted to our hospital; their ages ranged from 20 to 45 (average 32) years. All cases were diagnosed by echocardiography with tricuspid septal and posterior valve subluxation, anterior valve redundancy, and severe regurgitation; 10 cases were complicated by atrial septal defect and 6 cases were complicated by patent foramen ovale. The preoperative cardiac function was grade I in 10 cases, grade II in 6 cases, and grade III in 3 cases. 2 Surgical methods: conventional tracheal intubation and general anesthesia. All cases were performed under hypothermic extracorporeal circulation, using conventional aortic and inferior and superior vena cava cannulation methods; myocardial protection was performed by perfusion of cold stop solution containing blood through the aortic root. In all 19 patients, tricuspid valvuloplasty was performed using a 6 × 14 double-ended needle with shimmed mattress suture through the inferiorly displaced root of the tricuspid valve, through the atrialized ventricular portion, to fix the inferiorly displaced tricuspid valve in the normal annular position, with the suture continuing through the tricuspid valvuloplasty ring made of folded polyester patch, and with additional annular ring reduction for significantly enlarged tricuspid valves. In three patients, the posterior leaflet was attached directly to the ventricular wall, and the posterior leaflet was carefully removed with a circular knife and re-grafted to a normal annular position. The extracorporeal circulation time of this group ranged from 50 to 118 (72 ± 21) min, and the aortic block time ranged from 28 to 64 (43 ± 18) min. 3. Results: Postoperative cardiac ultrasound showed no tricuspid regurgitation in 12 patients (63%), mild regurgitation in 6 patients (32%), and moderate regurgitation in 1 patient (5%). There was no surgical death in the whole group of patients, and 2 cases of secondary open-heart hemostasis. After discharge, the patients’ symptoms improved significantly, and their activity tolerance returned to normal. At the average follow-up of 46 months, the cardiac ultrasound showed that the patients had no tricuspid regurgitation in 10 cases (53%), mild regurgitation in 6 cases (32%), and moderate regurgitation in 3 cases (16%); the recovery of cardiac function was grade I in 17 cases and grade II in 2 cases. 4, Discussion: There are different opinions on the selection of surgical indications and timing of surgery for Ebstein’s malformation. dannielson et al [ 1 ] considered surgery for those with progressive cyanosis, progressive cardiac function grade III-IV, and weakness complicated by other malformations. In contrast, Wu Qingyu et al [ 2 ] suggested that surgery can be performed as soon as the diagnosis of tricuspid valve subluxation is confirmed and obvious clinical symptoms appear. Summarizing our own clinical experience, we also believe that surgery should be considered if the diagnosis of tricuspid valve subluxation is confirmed and obvious clinical symptoms, such as cyanosis, panic, weakness, and obvious enlargement of the heart, are present. The right atrium and atrialized ventricle may enlarge further with age, and right heart failure may occur once the heart loses compensation. In many centers, valve replacement is used more often in the treatment of adult patients with Ebstein anomalies to avoid the need for reoperation after valvuloplasty to avoid worsening regurgitation, and Chen et al [3] showed that tricuspid valvuloplasty is more effective in pediatric patients than in adult patients, who have a higher rate of reoperation after valvuloplasty. However, patients who undergo prosthetic valve replacement face problems of prosthetic valve dysfunction, infective endocarditis, and thromboembolism, especially in tricuspid valve replacement, where the incidence of thromboembolism is much higher than in other locations of the valve. Tricuspid valvuloplasty can avoid these problems of valve replacement. In our group, tricuspid valvuloplasty was used in 19 patients with good surgical results. Therefore, tricuspid valvuloplasty should be used as much as possible in appropriate patients when technical conditions permit. In the event of worsening regurgitation or deterioration of cardiac function, valve replacement is indicated. In the surgical correction of Ebstein’s malformation in adults, several issues require special attention: 1, adult patients have a rich collateral circulation due to perennial ischemia and hypoxia, and bleeding is often difficult to control after diversion. The perioperative blood protection is especially important, such as shortening the time of diversion, applying peptidase for blood protection, postoperative platelet transfusion, cold precipitation, and fresh plasma can effectively reduce blood leakage. 2, adult patients with varying degrees of cardiac function (especially right heart function), intraoperative special attention to myocardial protection to prevent postoperative hypocardial row. In conclusion, surgical correction should be considered for adult patients with Ebstein malformation as long as clinical symptoms and heart enlargement occur, and tricuspid valvuloplasty should be the preferred surgical approach.