Results of surgery for Ebstein anomaly: A multicenter study from the European Congenital Heart Surgeons Association

Abstract

Most patients coming to surgery presented in childhood and were significantly symptomatic. More than half underwent valve replacement or repair, but a considerable proportion had severe disease necessitating 1(1/2) ventricle repair or palliative procedures. Operative mortality did not differ significantly among repair, replacement, and 1(1/2) ventricle repair but was associated with palliative procedures for severe disease early in life, young age being the only independent predictor of operative death.

A técnica do cone para correção da anomalia de Ebstein: resultados imediatos e em longo prazo

“…The experience of the Carpentier group, representing the second largest series among the ones published, had a mortality rate of 9% 17 . The publication by Sarris et al 18 , reporting the collective results of 13 institutions associated with the European Congenital Heart Surgeons Association, showed an in-hospital mortality of 13.3%, although it included newborns, which are a higher-risk group.…”

Pathophysiology of Congenital Heart Disease in the Adult

“…This allows the blood returning from the upper body to drain directly to the lungs and back to the LV, whereas the small RV is unloaded and needs to handle only return from the inferior vena cava. 8 As a rule, most patients with Ebstein's anomaly and significant symptoms due to hypoplasia of the RV will have undergone prior surgery as children, receiving either restorative valve surgery or single-ventricle repairs.…”

“…However, embryologically, the defect appears to be a single developmental error 8 that involves the terminal portion of the spiral septum, which divides the primitive truncus arteriosus from the pulmonary artery. In tetralogy of Fallot, this muscle bundle is displaced rightward and anteriorly, which precludes correct fusion with the growing muscular ventricular septum, narrowing the pathway from RV to pulmonary artery and enlarging the aortic root such that it extends over the RV outflow tract.…”

“…8 In some of these newborns, the ductus arteriosus may be the only source of pulmonary blood flow (blood flows from the high-resistance aorta to the lower-resistance pulmonary arteries). In the first few days of life, as the ductus begins to close, these patients will become profoundly cyanotic and will require medical intervention (prostaglandin E1) to maintain ductal patency, followed by a surgical reconstruction of the RV outflow tract.…”

Anomalia de Ebstein

“…The cone technique has been under development since 1989 by da Silva et al 1 , and adds to other tricuspid valve plastic surgeries for correction of Ebstein's anomaly in a more favorable manner due to lower mortality (3.8%) compared to Danielson's (5.8%), Carpentier's (9%), and 13 institutions of the European Congenital Heart Surgery (13.3%) under various techniques 2 , delivering even better results, including in the long-term with respect to the need for reintervention, and good functionality of the tricuspid valve and right ventricle 1 .…”