Background: Various surgical techniques have been used for correction of Ebstein's anomaly. This paper reports our early and intermediate term results for repair of this disease using the technique initially employed by Carpentier.
Patients and methods: Since December 1987 through December 1991, 13 consecutive patients with Ebstein's anomaly underwent a Carpentier's type of repair. Their mean age was 16.3 years ranging from 6 to 51 years. The indications for operation were advanced NYHA functional class (III-IV) in 4 cases, cyanosis in 4, severe arrhythmia in 3 and cyanosis with arrhythmia in 2. Associated malformations were present in 9 patients. Eight had an atrial septal defect and four of them had, also, mitral prolapse whereas the remaining patient had a ventricular septal defect with subaortic stenosis. Tricuspid regurgitation was severe in 12 cases and moderate in one. According to the anatomo-functional classification described by Carpentier, seven patients had Ebstein's type C, whereas a type B was present in five patients and only one patient had a type A.
Results: There were three hospital deaths all of which occurred in patients with type C anomaly. Causes of death were respectively low output syndrome, sepsis and cerebral hemorrhage. Follow-up ranged from 49 to 105 months (median 58 months). There were no late deaths and all patients were in NYHA functional class I or II. Preoperative arrhythmia was improved or abolished in 4 out of 5 patients. Echocardiography showed that tricuspid regurgitation was absent in 2, mild in 4, moderate in 3 and severe in the remaining patient.
Conclusions: Repair of Ebstein's anomaly, using the technique reported by Carpentier, can be accomplished with an acceptable operative risk and satisfactory intermediate term results. Echocardiography has an important role in the diagnosis of Ebstein's anomaly as well as in the indications and timing for repair and it is particularly useful for intra- and postoperative monitoring and anatomofunctional evaluation.