title: Mid-term outcomes in adults with ebstein anomaly and cavopulmonary shunts.
abstract: 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.
authors: Toronto Congenital Cardiac Centre for Adults, University Health Network, University of Toronto, Toronto, Canada.
authors: Khalid S
authors: KS
authors: Al-Najashi
authors: Olga H
authors: OH
authors: Balint
authors: Erwin
authors: E
authors: Oechslin
authors: William G
authors: WG
authors: Williams
authors: Candice K
authors: CK
authors: Silversides
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D006348:Cardiac Surgical Procedures / Q000009:adverse effects / Q000379:methods*; D015331:Cohort Studies; D004437:Ebstein Anomaly / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*; D004502:Education, Medical, Continuing; D005260:Female; D005500:Follow-Up Studies; D018610:Heart Bypass, Right / Q000379:methods*; D019918:Heart Valve Prosthesis Implantation / Q000379:methods; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D011336:Probability; D018570:Risk Assessment; D018709:Statistics, Nonparametric; D016019:Survival Analysis; D013997:Time Factors; D016896:Treatment Outcome; D014261:Tricuspid Valve / Q000601:surgery*; D018497:Ventricular Dysfunction, Right / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*; D055815:Young Adult