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Operation for Ebstein anomaly in patients with moderate to severe left ventricular dysfunction effective, study shows

Thursday, December 18 2008 | Comments

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What's This? In patients with Ebstein anomaly, left ventricular (LV) systolic dysfunction occurs infrequently, but operative intervention for those with significant LV dysfunction appears to have favorable results over the long term, according to recently published data.

The initial study cohort included 539 patients who underwent an operation for Ebstein anomaly at Mayo Clinic from April 1972 to January 2006. Findings from preoperative echocardiographic assessment of LV function were availble for 495 subjects. Of these, 50 had moderate to severe LV systolic dysfunction.

The authors of the study noted their primary goal was to examine outcomes, such as mortality, reoperation, and LV function, following operative intervention for individuals with Ebstein anomaly and significant LV dysfunction.

In the 50 patients with LV dysfunction, 36 had tricuspid valve (TV) replacement and 12 had TV repair. One patient who had severe right ventricular (RV) dysfunction in addition to moderate LV dysfunction underwent a 1.5 ventricle repair with TV replacement and bidirectional Glenn shunt. Another subject underwent orthotopic cardiac transplantation.

Five early deaths (<30 days) occurred in the 50 patients with moderate to severe LV dysfunction (10%). Because of the small number of deaths, the analysis was limited in its ability to identify variables associated with early mortality, although the investigators did report that for the entire cohort of 539 patients, RV dysfunction was predictive of early death, but LV dysfunction was not. Only 4 patients' LV function did not improve after surgery, and no patients' LV function worsened.

The survival rates for the entire cohort at 1, 5, and 10 years were 86%, 77%, and 67%, respectively. Univariate analysis showed that only absence of sinus rhythm at discharge was an independent predictor of higher overall mortality (P=.003). For patients with moderate to severe LV dysfunction, survival was worse as compared with those with normal or only mild LV dysfunction. In the overall cohort of 539 patients, the hazard ratio for moderate to severe LV dysfunction as a predictor of late mortality was 3.76 (P<.001).

The survival-free-of-operation rates were 89% at 1 year, 73% at 5 years, and 59% at 10 years. After a mean follow-up of 6.9 years, 86% of patients were in New York Heart Association class I or II and 14% were in class III or IV.

"Although early mortality is greater in patients with LV dysfunction, the late results are favorable," the authors concluded. "Decreasing LV function should be an indication to promptly restore TV competence rather than a contraindication to TV operation." (Brown ML, et al. Am J Cardiol 2008;102:1724-1727.)

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