Abstract
Aim: To evaluate the outcome of correction of Ebstein's anomaly in adult patients undergoing surgery as part of a program of care of adult patients with congenital heart defects. Group of patients/methods: A total of 15 patients with Ebstein's anomaly (age 21-63 years, median 45 years) were operated on from December 2005 through May 2008 at Na Homolce Hospital.
In terms of morphology, types A, B, and C were present in three, four, and eight patients, respectively. All patients had significant up to massive tricuspid insufficiency, combined with stenosis in two, eleven patients had arrhythmias (atrial fibrillation or flutter, AV nodal re-entrant tachycardia), with radiofrequency ablation (isthmus CT and modification of dual AV nodal pathway) undertaken preoperatively in two.
Three patients were severely cyanotic (resting saturation < 85%). In clinical terms, ten patients were significantly symptomatic (NYHA III-IV), three moderately (NYHA II-III), with two only mildly (NYHA I-II).
During surgical correction, the tricuspid valve was replaced by a biological valve prosthesis in seven patients, valvuloplasty was performed in another seven while no procedure was undertaken in one. In addition, bidirectional cavopulmonary anastomosis to relieve the dysfunctional right ventricle was performed in six, atrial defect closure in five, the MAZE procedure using cryoablation in ten, and plication of the atrialized portion of the right ventricle in six patients.
Results: Fourteen out of the 15 patients undergoing surgery are alive in a clinically improved condition at 4-32 months (median 13) post-surgery; one patient died on postoperative day 8 from intracerebral hemorrhage after being placed on ECMO (extracorporeal membrane oxygenation) for biventricular failure. All seven patients with tricuspid valve replacement show good function of the biological valve prosthesis free of stenosis or insufficiency; six of the seven patients undergoing tricuspid valvuloplasty show mild to moderate tricuspid valve insufficiency, and one significant tricuspid regurgitation.
Twelve patients are in sinus rhythm, while two are pacemaker dependent. Only one patient developed temporary atrial flutter (the patient did not have the MAZE procedure intraoperatively).
Statistical analysis demonstrated postoperative improvement of tricuspid valve function and patient self-assessed performance status without a change in right ventricular function. Conclusion: Correction of Ebstein's anomaly may improve the clinical status of patients with this serious congenital heart defect associated with acceptable mortality.
As the defect does not involve the tricuspid valve only, but the whole right heart, the surgical technique depends on the morphology of the defect and the patient's clinical state and age.