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Earlier treatment better for Ebstein anomaly in adults

NEW YORK (Reuters Health) – Cardiac surgery for Ebstein anomaly (EA) in adults should be performed before the associated heart disease is too advanced, the authors of a new analysis advise.

EA occurs in about 1 to 5 of every 200,000 live births. Many patients survive into adulthood with minimal symptoms. “It’s the only anomaly that we have in congenital heart disease where the presentation can range from a newborn who needs urgent surgery to an incidental finding in a 70-year-old,” senior author Dr. Joseph A. Dearani of the Mayo Clinic in Rochester, Minnesota explained in an interview with Reuters Health.

Because symptoms may be relatively mild, physicians may not refer adult patients for surgery given the perception that the surgery can be high risk, Dr. Dearani noted. But by the time heart failure and shortness of breath appear, surgery is likely to only have palliative effects, he said.

In younger patients, the researcher said, EA usually can be treated effectively with valve repair, but older patients are more likely to require valve replacement.

In the June 5 issue of the Journal of the American College of Cardiology, Dr. Dearani and his colleagues review data on all patients with EA who had surgery at their center between 1980 and 2010, at or above the age of 50.

Altogether, 81 patients had 89 procedures. Their symptoms included palpitations, edema, and previous stroke/transient ischemic attack. Seventy-six patients were in New York Heart Association functional class III or IV.

Eighty-seven procedures (98%) involved tricuspid valve surgery, including replacement in 65 patients and repair in 22 patients. Three patients (4%), all treated before 1995, died early.

Dr. Dearani said the operative mortality rate would likely be higher elsewhere, because Mayo Clinic surgeons have performed more than 1,000 EA surgeries on patients of all ages. “These patients managed in the community would have a risk that would be greater than 4%,” he said. In EA younger patients, he added, surgery carries a 1% mortality risk.

Follow-up was available for 73 of the 78 survivors, with a median duration of 84 months. Sixty-three survivors had an improvement in functional class, and just eight (11%) were still in functional class III or IV.

Thirteen of the 78 patients who survived surgery died during follow-up. Actuarial 10-year survival was 71%, and 20-year survival was 65%, compared to an expected 88% and 74%, respectively, for age- and sex-matched controls. The strongest predictors of mortality were failure to improve postoperatively and older age at surgery.

The best approach to determining whether an adult with EA should undergo surgery, according to Dr. Dearani, is to refer that patient to doctors and surgeons with training in congenital heart disease, who can then determine if the patient needs to be referred on to cardiologists with specific expertise in EA.

When adults with congenital heart anomalies are treated by physicians with training in congenital disease rather than “garden variety adult cardiologists,” he added, “the outcome is better, the cost is lower, and the hospital stays are shorter….those are the three things that have been demonstrated in the literature in many studies.”


J Am Coll Cardiol 2012;59:2101-2106.