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Ebstein's malformation is usually treated by replacing the tricuspid valve, albeit with controversial results. The “Carpentier technique,” introduced in 1980, offers both a new classification and new surgical methods for treatment of this lesion. The classification is based on the severity of the displacement of the septal and posteroinferior leaflets, with four grades of increasing severity. The surgical techniques involve mobilization of the anterosuperior leaflet, reduction of the diameter of the annulus, and longitudinal plication of the atrialized part of the right ventricle. Following this approach, 64 patients underwent consecutive surgical evaluation, with only one valvar replacement. The mean age was 26±15 years. The hospital mortality was nine percent. The mean follow-up is now 5.18±3.25 years. The actuarial survival rate was 87% at one year and 82% at five and 10 years. Of the patients, 90% were in postoperative functional class I or II. Control echocardiography revealed either no insufficiency, or minimal regurgitation in 80%. A second operation was needed in six patients because of right ventricular failure (two patients) or recurrence of valvar insufficiency (four patients). Sinus rhythm was present in 44 (88%) of the surviving patients. We conclude that repair of the malformed tricuspid valve, associated with longitudinal plication of the right ventricle, is safe and efficient. The prognosis depends on right ventricular contractility, which should be evaluated carefully before and after the operative procedure.

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Lesions producing congenital mitral valvar stenosis are complex and often multiple, From 1970 to 1994, 58 patients under 12 years of age (mean 5 ± 3.2 y) underwent surgical treatment for such malformations in our department. In order to provide a clear understanding of the pathology, we used a classification based on anatomy and function. Echocardiography was a major tool of the preoperative investigations. The lesions were classified as follows:Normal papillary muscles: commissural fusion in 19, excess leaflet tissue in 2, valvar ring in 12, and annular hypolasia in 2.Abnormal papillary muscles: paachute arrangement in 10 and hammock mitral valve in 13.Associated malformations were found in 63%, the most common being multiple stenosis in the left heart. Conservative treatment was possible in 54 patients (95%) using the techniques developed by Carpentier. Hospital mortality was 22%, with 95% confident limits between 13 and 36% (13/58). Of the 13 patients with a hammock valve, 6 died in hospital. The overall mean period of follow up was 7.8 + 5 years. No thromboembolic events occured after conservative surgery. Late death occured in 3%. The actuarial survival at 5 years was 64 + 15%. Reoperation was needed for residual mitral valvar stenosis in three patients or insufficiency in one.In spite of effective techniques to relieve mitral obstruction, 12% of our patients need early or delayed replacement of the valve. The hospital mortality remains high due to the complexity of the lesions, associated with the associated malformations. Echocardiography proved to be extremly helpful for the functional analysis. In order to improve operative results, simple and early valvar repair, even if considered palliative, seems to be appropriate.

There are many congenital malformations of the mitral valve which produce valvar insufficiency. From a surgical point of view, systems based exclusively on anatomic analysis are not always entirely appropriate for assessment of these lesions. With this in mind, Carpentier proposed a functional approach for analysis based upon the motion of the valvar leaflets. From 1969 to 1994, 135 children under the age of 12 (mean age: 5.8 + 3.15 Y, 0.6–12Y) underwent surgery in our department, basing treatment on such analysis. Since motion of the leaflets during the operation is compromised by cardioplegia, and sometimes exposure can be however difficult, preoperative echocardiography was a mandatory part of the diagnostic cascade.Normal motion of the leaflets was present in 41 patients, with deformation of the annulus in 14, a cleft in 21, and partial agenesis in 6. Prolapse of leaflets was present in 42 patients. Leaflet motion was restricted in 28 patients. These were divided in two groups, one with normal papillary muscles and commissural fusion or short cords. The other with abnormal papillary muscles producing a parachute arrangement in 6 and a hammock valve in 9. Associated lesions were present in 47% of the patients. Conservative surgical procedures following the precepts developed by Carpentier were used in 127 patients. Valvar replacement was necessary in 8 patients. Operative mortality was 4%. Mean follow up was 8.4 ± 5.3 years (1–23Y). Actuarial survival at 5 years was 90 ± 6% and, at this time, was stable. No thromboembolic events occurred after conservative surgery. The reoperation rate was 5% for those undergoing repair (6 patients). We conclude that the functional classification developed by Carpentier is a reliable and robust approach to these complex lesions. Conservative surgery is feasible in most of the cases presenting with congenital mitral valvar insufficiency. Results are stable and reliable. Surgery should be undertaken before the onset of left ventricular deterioration.