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.
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.
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.
ObjectiveTo summarize the early and mid-term surgical outcomes of cone reconstruction for Ebstein’s anomaly. MethodsPatients with Ebstein’s anomaly who underwent cone reconstruction at Guangdong Provincial People’s Hospital from 2015 to 2024 were retrospectively enrolled. Baseline characteristics, echocardiographic parameters before and after surgery, and follow-up results were collected and analyzed. ResultsA total of 115 patients were included, comprising 28 males and 87 females, with a mean age of (33.29±15.72) years. There were 94 patients in the adult group (>14 years old), and 21 patients in the pediatric group (≤14 years old). Severe or greater tricuspid regurgitation was present in 98.3% of patients preoperatively, and 89.6% were in New York Heart Association (NYHA) functional class Ⅱ. All patients successfully underwent cone reconstruction. The in-hospital reoperation rate for tricuspid valve disease was 1.7%, and no in-hospital death occurred. In the pediatric group, tricuspid regurgitation area and right atrial longitudinal dimension were significantly smaller at 5 days, 6 months, and 1 year postoperatively than preoperative values (P<0.05). In the adult group (n=92, excluding 2 patients who underwent in-hospital reoperation for tricuspid valve disease), tricuspid regurgitation area, pulmonary artery systolic pressure, right atrial longitudinal dimension and anteroposterior diameter of the right ventricular outflow tract were significantly decreased at 5 days, 6 months, and 1 year after surgery (P<0.05), while the right ventricular longitudinal dimension was significantly increased (P<0.001) compared to preoperative levels. The left ventricular end-diastolic and end-systolic diameters were significantly increased at 6 months and 1 year postoperatively (P<0.001). Right heart functional parameters (right ventricular fractional area change, tricuspid annular plane systolic excursion, and tricuspid annular myocardial systolic velocity) demonstrated a trend of gradual recovery after an initial decline in the early postoperative period. The median follow-up duration was 1.8 years, with a follow-up rate of 95.7%. During the follow-up, 3 patients underwent repeated tricuspid valve surgery and 1 patient died. ConclusionCone reconstruction is safe and effective for Ebstein’s anomaly, and can significantly alleviate tricuspid regurgitation and reverse right heart structural remodeling. Both pediatric and adult patients achieve marked improvements in tricuspid regurgitation and right heart morphology, additionally, adult patients also gain obvious benefits in left heart function. Postoperative right heart function shows a gradual recovery trend, with favorable mid-term follow-up outcomes.