Surgery for Ebstein's anomaly: The clinical and echocardiographic evaluation of a new technique

• Published in: Journal of the American College of Cardiology 1991-03, Vol.17 (3), p.722-728
• Main Authors: Quaegebeur, Jan M., Sreeram, Narayanswami, Fraser, Alan G., Bogers, Ad J.J.C., Stümper, Oliver F.W., Hess, John, Bos, Egbert, Sutherland, George R.
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Biological and medical sciences; Cardiology. Vascular system; Child; Child, Preschool; Congenital heart diseases. Malformations of the aorta, pulmonary vessels and vena cava; Ebstein Anomaly - physiopathology; Ebstein Anomaly - surgery; Echocardiography - methods; Electrocardiography, Ambulatory; Exercise Test; Follow-Up Studies; Heart; Humans; Medical sciences
• ISSN: 0735-1097; 1558-3597
• DOI: 10.1016/S0735-1097(10)80190-1

Ten consecutive patients (age range 4 to 44 years, mean 22) underwent surgical repair of Ebstein's anomaly by vertical plication of the right ventricle and reimplantation of the tricuspid valve leaflets. No patient died during or after operation. Intraoperative postbypass echocardiography documented a good result in nine patients but severe tricuspid regurgitation in one patient, who then underwent prosthetic valve replacement during a second period of cardiopulmonary bypass. Two of four patients who had had right ventricular papillary muscle dysfunction in the early postoperative period showed improved papillary muscle function with concomitant reduction of tricuspid regurgitation 6 months later. All patients were evaluated clinically and by echocardiography 2 to 23 months later. All patients showed clinical improvement, seven by one functional class and three by two classes. All were in sinus rhythm. The mean cardiothoracic ratio decreased by 6% (p < 0.05). On bicycle ergometry performed in six patients, peak oxygen consumption exceeded 20 ml/kg per min in five. Tricuspid regurgitation diminished in eight patients (by three grades in two patients, by two grades in five and by one grade in one patient); it remained unchanged in two. Comparison of preoperative and postoperative pulsed Doppler flow velocities across the pulmonary valve showed an increase in the peak velocity of flow across the valve (mean 83 ± 14 versus 97 ± 11 cm/s, p < 0.005) and a decrease in the time to peak velocity (mean 130 ± 16 versus 91 ± 23 ms, p < 0.05). The initial evaluation of patients treated by this new operation for Ebstein's anomaly demonstrates significant clinical improvement in all patients with a reduction in the severity of tricuspid regurgitation and improved forward flow after the creation of a functional right ventricle.