HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Full Text Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): William G. Williams Permission Requests Google Scholar Articles by Al-Najashi, K. S. Articles by Silversides, C. K. PubMed Articles by Al-Najashi, K. S. Articles by Silversides, C. K. Related Collections Congenital - cyanotic

---

Original Articles: Pediatric Cardiac

Mid-Term Outcomes in Adults With Ebstein Anomaly and Cavopulmonary Shunts

Toronto Congenital Cardiac Centre for Adults, University Health Network, University of Toronto, Toronto, Canada

Accepted for publication March 17, 2009.

^_* Address correspondence to Dr Silversides, University Health Network, Toronto General Hospital, 585 University Ave, 5N-521 North Wing, Toronto, Ontario, M5G 2N2, Canada (Email: candice.silversides{at}uhn.on.ca).

Background: In patients with Ebstein anomaly and poorly functioning right ventricles, a cavopulmonary shunt (CPS) can be created to reduce the preload on the right ventricle. The purpose of this study was to examine the early and mid-term outcomes in adults with Ebstein anomaly who have undergone tricuspid valve repair or replacement with or without a concomitant CPS.

Methods: We examined the outcomes of 40 consecutive patients seen at our center with Ebstein anomaly who had undergone tricuspid valve repair or replacement with (n = 23) or without (n = 17) concomitant CPS. Follow-up data were obtained by either chart review or contacting the referring cardiologist. Mid-term survival was examined using Kaplan-Meier curves.

Results: The mean age at surgery was similar in patients with and without CPS (42 ± 12 versus 39 ± 19 years; p = 0.63). There were 2 early postoperative deaths owing to refractory right-sided heart failure. Mid-term follow-up data were available in 95% of patients. The mean follow-up time was 6.7 ± 4.8 years. Patients who received a CPS more commonly had preoperative heart failure or cyanosis (p = 0.04) and had worse preoperative functional status (p = 0.09). In both groups, arrhythmias were the most common late complication. There were 5 late deaths, 3 of which occurred in patients with CPS. Five-year survival with or without CPS was comparable (83% ± 9% versus 86% ± 10%; p = 0.85).

Conclusions: Adolescent and adult patients with Ebstein anomaly undergoing tricuspid valve replacement or repair and concomitant CPS are at risk for early and mid-term complications. However, Ebstein surgery along with CPS appears to be a reasonable surgical strategy in patients not thought to be suitable for tricuspid valve surgery alone.