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Carpentier-Chauvaud-Cone Repair For Ebstein Anomaly: Three Decades Of Experience
Kevin M. Veen, Mostafa M. Mokhles, Jolien W. Roos-Hesselink, Bas R. Rebel, Johanna J.M. Takkenberg, Ad J.J.C. Bogers.
Erasmus MC, Rotterdam, Netherlands.

OBJECTIVE: Since 1988 we have used a vertical plication repair with de- and reattachment of the tricuspid valve (TV) for Ebstein anomaly. We describe the long term outcomes of these patients.
METHODS: All patients operated between January 1988 and November 2016 were retrospectively reviewed. Kaplan-Meier analyses were done for survival data and generalized least squares and generalized estimating equation models were used to analyze echocardiographic data.
RESULTS: There are 36 patients (mean age 25.415.9 years, 36% male) operated for Ebstein anomaly according to the techniques described by Carpentier and Chauvaud and, if possible, with application of the Cone technique. NYHA III/IV was present in 53% of patients and two patients had a prior ASD closure. One (3%) patient died 2 days after surgery and 2 patients (6%) had an early re-sternotomy due to tamponade. Median hospital stay was 11 days (IQR 8-13). Three (8%) patients had severe tricuspid regurgitation (TR) at intraoperative echocardiography post correction. One non-cardiac late death occurred during a median follow-up of 15.1 years (IQR 7.6-22.1) resulting in overall survival of 973% at 25 years. Six patients required a reoperation resulting in a freedom of reoperation of 915%, 808% and 808% at 1, 10 and 25 years. Freedom from NYHA III/IV was 818% at 25 years (patients were censored at reoperation). Longitudinal evolution of TV gradient and probability of severe TR are shown in Figure 1AB. Longer follow-up time, adjusted for sex, age and Carpentier class, was not a risk factor for a higher gradient or higher probability of severe TR in patients who are not re-operated.
CONCLUSIONS: The Carpentier-Chavaud-Cone technique for Ebstein anomaly has excellent results in terms of survival and functional class. Most reoperations are required within the first 5 years, and in patients without a reoperation, tricuspid stenosis and/or regurgitation do not appear to be progressive, which may indicate a durable repair.


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