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Individualized Surgical Treatments for Children with Ebstein Anomaly
Zheng Jinghao1, Luo Kai1, Huang Yanhui1
1Department of Cardiothoracic Surgery, Shanghai Children's Medical Center, Heart Center, School of Medicine, Shanghai Jiaotong University, Shanghai, China.
Insights
Surgical treatment for Ebstein anomaly in children, including tricuspid valvuloplasty and palliative surgery, shows favorable short- and midterm outcomes. Individualized surgical approaches improve functional class and reduce tricuspid regurgitation, with a low mortality rate.
Area of Science:
- Cardiology
- Pediatric Surgery
- Congenital Heart Disease
Background:
- Ebstein anomaly is a rare congenital heart defect affecting the tricuspid valve.
- Current surgical options include tricuspid valvuloplasty, palliative procedures, and valve replacement.
- Outcomes of different surgical strategies in pediatric Ebstein anomaly patients require evaluation.
Purpose of the Study:
- To assess the short- and midterm results of various surgical treatments for Ebstein anomaly in children.
- To compare the effectiveness of tricuspid valvuloplasty, palliative surgery, and tricuspid valve replacement.
Main Methods:
- Retrospective analysis of 136 pediatric patients with Ebstein anomaly who underwent surgery between 2006 and 2015.
- Surgical interventions included tricuspid valvuloplasty (118 patients), palliative surgery (14 patients), and tricuspid valve replacement (4 patients).
- Follow-up included echocardiography, chest radiography, oxygen saturation, and cardiac function assessment over a mean of 35.8 months.
Main Results:
- Overall mortality rate was 2.2% (3/136), with no reoperations needed in survivors.
- Post-operative oxygen saturation improved significantly, especially after radical surgery (95-100%).
- Most patients improved in New York Heart Association functional class from III/IV to I/II, with reduced tricuspid regurgitation.
Conclusions:
- Individualized surgical strategies tailored to patient-specific factors (age, anatomy, function) optimize outcomes for Ebstein anomaly.
- Tricuspid valvuloplasty and palliative surgeries demonstrate effective short- and midterm results in pediatric populations.
- Personalized surgical decision-making is crucial for improving functional status and reducing regurgitation in Ebstein anomaly.
Abstract:
Objective Ebstein anomaly is a rare type of tricuspid malformation. The present surgical methods to resolve this anomaly include tricuspid valvuloplasty, palliative surgery, and tricuspid valve replacement. The purpose of this study was to evaluate the short- and midterm outcomes of different surgical treatments among children with Ebstein anomaly. Methods This was a retrospective study of 136 Ebstein anomaly patients undergoing surgery at our institution from January, 2006 to August, 2015. This cohort included 118 patients receiving tricuspid valvuloplasty, 14 patients receiving palliative surgery and 4 patients receiving tricuspid valve replacement. Results There were two in-hospital deaths and one delayed death 6 months after bidirectional cavopulmonary shunt during a second-stage operation; thus, the mortality rate was 2.2% (3/136). The follow-up variables included echocardiography, chest radiography, oxygen saturation, and cardiac function. After a mean follow-up duration of 35.8 ± 16.5 months (range: 6-98 months), all survivors had no indications for reoperation; the oxygen saturation following radical surgery and palliative surgery was 95 to 100% and 85 to 95%, respectively. Most of the patients exhibited an improved New York Heart Association (NYHA) functional class from III or IV preoperatively to I or II at follow-up; only two patients who underwent the Fontan procedure continued to exhibit NYHA functional class III. In most patients, tricuspid regurgitation (TR) degree decreased from moderate or severe preoperatively to mild or moderate at follow-up and only six patients continued to exhibit severe TR at follow-up. Conclusions Individualization of surgical methods based on different indications, including age, associated malformations, tricuspid anatomy, cardiac function, and intraoperative status, could effectively improve the outcomes of Ebstein anomaly patients.
