Most patients who undergo surgery for Ebstein anomaly have good long-term survival and functional outcomes, according to a report in the August 5th issue of the Journal of the American College of Cardiology.
Many patients require operation for Ebstein anomaly, a spectrum of tricuspid valvular and right ventricular dysplasia, in order to improve their quality of life, the authors explain.
Dr. David J. Driscoll and colleagues from the Mayo Clinic and Foundation, Rochester, Minnesota assessed the long-term functional outcome and reproduction of 285 patients with Ebstein anomaly who underwent surgery at a mean age of 24.
At 10 and 20 years, 82% and 56% of patients, respectively, were alive without having had any reoperation, the authors report. Similarly, 68% (at 10 years) and 35% (at 20 years) of patients had been free of rehospitalization for cardiac causes.
Most patients were in NYHA functional class I (43%) or II (40%), with only a minority in class III (12%) or IV (4%), the researchers note, and half the subjects reported their exercise tolerance to be at least that of their peers.
Overall, outcomes were rated excellent in 7.4%, good in 72.3%, and poor in 20.4% of patients.
About 80% of female patients reported at least one pregnancy, and miscarriage rates were lower after surgery (19%) than before surgery (33%).
Of the 232 liveborn children, 9 were reported to have a heart defect (6 born to mothers and 3 born to fathers with Ebstein anomaly), the investigators report.
"Assessment of the late results of novel methods to repair the tricuspid valve is required," the authors note. "A more durable biological prosthesis is needed for patients whose valves are irreparable."
"The time appears to have arrived for systematic testing of the adjunctive surgical strategies of right ventricular volume reduction surgery, cavopulmonary connection, and prophylactic atrial arrhythmia surgery," writes Dr. Glen Van Arsdell, at the Hospital for Sick Children, University of Toronto, Ontario, Canada, in a related editorial. "They hold the potential for reducing late problems of atrial arrhythmia and right ventricular myopathy."
J Am Coll Cardiol 2008;52:460-469.
