Early and late results for operation for Ebstein anomaly

David Driscoll
Professor of Pediatrics, Mayo Clinic
Rochester, USA

【Introduction】Ebstein anomaly is spectrum of tricuspid valve (TV) and right ventricular (RV) dysplasia. The TV usually is insufficient but, also may be stenotic. We will assess the long-term outcome of operation for Ebstein anomaly. Specifically, we intend to define operative and long-term mortality, the determinants of operative and long-term mortality, the determinants of reoperation, and postoperative quality of life.
【Results】From April 1, 1972 to January 1, 2006, 539 patients with Ebstein anomaly had 604 cardiac operations at the Mayo Clinic. The range in age at the initial operation was 8 days to 79 years. At the time of the first operation at Mayo Clinic, 182 patients had tricuspid valve repair (TVrpr) and 337 had tricuspid valve replacement (TVrpl). The 30 day mortality was 5.9% for the entire cohort (2.7% after 2001). Late survival was 84.7% at 10 years and 71.2% at 20 years. In a multivariate analysis of overall mortality increased hematocrit, RV outflow tract obstruction, TVrpl, WPW, miscellaneous arrhythmia procedure, branch pulmonary artery enlargement, need for mechanical support postoperatively, emergent chest opening in the intensive care unit, and absence of sinus rhythm at dismissal were all predictive of mortality. When only preoperative characteristics were included, increased hematocrit, mitral valve regurgitation requiring surgical intervention, prior cardiac procedure, and moderate-severe to severe reduction in right ventricular (RV) systolic function were associated with mortality. Preoperative sinus rhythm and an accessory pathway were associated with survival
　Five patients had moderate-severe and 13 had severe LV dysfunction preoperatively. Among these 18 patients there were 2 early deaths (11%). For the 18 cases with greater than moderate preoperative LV dysfunction, dismissal or pre mortem echocardiography demonstrated postoperative LV function as normal (n = 9), mildly reduced (n = 2), moderately reduced (n = 4), moderate-severely reduced (n = 1), severely reduced (n = 1) and unknown (n = 1). LV dysfunction was associated with reduced survival and with lower survival free from reoperation (p < 0.05).
　Health status surveys were obtained from 285 surviving patients. Two hundred and thirty-seven (83%) of patients were in NYHA class I or II and 34% were taking no cardiac medication. Five patients (2%) reported having endocarditis, 1 patient ( < 1%) reported a stroke, and 103 patients (36%) had atrial fibrillation or flutter. There were a total of 202 pregnancies among 82 women. Recurrence of congenital heart disease was low. Only 9/232 (0.04%) of liveborn children had congenital heart disease.
【Conclusion】Ebstein anomaly can be surgically treated with low perioperative mortality. Both TVrpr and TVrpl are associated with good long term survival. Risk factors for poorer outcome included right and/or left ventricular systolic dysfunction, increased hemoglobin/hematocrit, male gender, RV outflow tract obstruction or hypoplastic pulmonary arteries.
　Although LV dysfunction is a risk factor for increased mortality and need for late reoperation in some patients with Ebstein anomaly, all but one patient had improved LV function after tricuspid valve repair/replacement. Decreasing LV function should be an indication to promptly restore tricuspid valve competence rather than a contraindication to tricuspid valve surgery.
　Patients have good long term survival and functional outcomes, after operation for Ebstein anomaly. Atrial arrhythmias are relatively common both before and after operation. Pregnancy appears to be well tolerated with low recurrence risk of congenital heart disease.