ObjectiveTo analyze clinical features and surgical strategies of Ebstein's anomaly (EA) in adults. MethodsSeventy-eight adult patients with EA underwent surgical treatment in Fu Wai Hospital from January 2008 to December 2011. There were 24 males and 54 females with their age of 18-54 (33.0±9.5) years. Preoperatively, 72 patients were in NYHA class Ⅰ or Ⅱ, and 6 patients were in NYHA class Ⅲ or Ⅳ. Clinical presentations mainly included exercise capacity deterioration and exertional dyspnea. Preoperative echocardiography showed downward displacement of the septal leaflet (SL) of the tricuspid valve (TV) of 34.8±12.7 (20-60) mm. Three patients had severe dysplasia or agenesis of tricuspid SL. Downward displacement of the posterior leaflet (PL) of TV was 46.8±11.6 (20-70) mm, and 1 patient had agenesis of tricuspid PL. Average TV annulus was significantly enlarged with 60±10 (37-70) mm. Mean atrialized portion of the right ventricle was about 40%. There were 18 patients with moderate tricuspid regurgitation (TR) and 60 patients with moderate-to-severe TR. Seventy-five patients received tricuspid valvuloplasty (TVP). Fifty-six patients received plication of the atrialized right ventricle (ARV), 20 patients received ARV resection, and 2 patients didn't receive any specific management of ARV. Thirty-two patients received TVP with a prosthetic ring. Three patients underwent tricuspid valve replacement. ResultsTwo patients died posto-peratively, and in-hospital mortality was 2.5%. Postoperative recovery of the survival patients was good. There was no severe atrioventricular block or other complication. Echocardiography before discharge showed good function of TV without moderate or more severe TR. Mean follow-up was 26 months. None of the patients needed re-operation. ConclusionThe incidence of acute heart failure in EA adults is low. TVP is the main surgical procedure to achieve main goals of surgical treatment including improvement in heart function, exercise capacity and quality of life.
Objective To summarize the experience and prognosis of Cone reconstruction used in Ebstein's anomaly. Methods We retrospectively analyzed the clinical data of 10 consecutive patients with Ebstein's anomaly, who underwent Cone reconstruction in our hospital from January 1, 2012 through February 1, 2015. According to Carpentier's classification, there were 2 patients of type A, 4 of type B, 2 of type B-C and 2 of type C. Results There was no death. One patient had arrhythmia after operation. The mean regurgitation area was 1.4±1.8 cm2 after operation, 6.4±6.9 cm2 at the time of discharge, respectively. Both of them were lower than preoperative regurgitation area (18.7±11.4 cm2) with statistical differences (95%CI –25.154 to –9.573,P=0.001; 95%CI 6.567 to 18.113,P=0.001). Three months after operation, the regurgitation was improved significantly compared to the preoperative regurgitation (95%CI 4.523 to 12.052,P=0.004). While it was higher in the regurgitation area when the patients were discharged. However, there was no statistical difference (95%CI –5.783 to 1.039,P=0.126). The incidence of severe regurgitation was about 85.7% at end of 3 months after operation, while decreased to 32.1%, 13 months later. The size of right atrium was much smaller than preoperative size (95%CI 1.033 to 31.480,P=0.039) when the patients were discharged. Conclusion Cone technique with ringed annuoplasty permits a good leaflet-to-leaflet coaptation. Tricuspid competence after operation could be sustained for a long time. However, postoperative short term is the crisis period to severe regurgitation. Further investigation for more appropriate surgical strategy should be carried on.
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.
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.