Ebstein's anomaly


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Ebstein's anomaly

 [eb´shtīnz]
a malformation of the tricuspid valve, the septal and posterior leaflets being adherent to the wall of the right ventricle to a varying degree, producing tricuspid deficiency, and the anterior leaflet being normally attached to the annulus fibrosus; usually associated with an atrial septal defect.
Ebstein's anomaly with tricuspid valve significantly displaced downward in the right ventricle; leakage occurs through the tricuspid valve back to the right atrium, and unoxygenated blood is shunted across the atrial-septal defect into the left atrium. From Betz et al., 1994.

Ebstein's anomaly

An uncommon (0.5% of all congenital heart defects) cardiac malformation, which is characterised by apical displacement of the septal and posterior tricuspid valve leaflets, with atrialisation of the right ventricle and displacement of the anterior leaflet, leading to tricuspid regurgitation of varying severity and backflow of blood into the right atrium from the right ventricle.
References in periodicals archive ?
Surgical management of Ebstein's anomaly in the adult.
The following are the hemodynamic goals for the management of TOF and Ebstein's anomaly: avoid a drop in systemic vascular resistance; maintain intravascular volume and venous return; prevent hypoxemia, hypercapnea and acidosis, as well as myocardial depression during anesthesia (12).
Echocardiogram also showed severe right heart enlargement with atrialization of the right ventricle and apical displacement of the tricuspid valve consistent with Ebstein's anomaly, which can be seen in Figures 1 and 4.
Suarez et al reported a case of graded epidural anaesthesia being used for caesarean section in a patient with Ebstein's anomaly in whom hypotension was managed successfully with crystalloids and phenylephrine.
Mothers with cyanotic congenital heart disease have higher incidence of fetal losses, preterm births and low weight neonates; however in Ebstein's anomaly it is considered that even without cyanosis, fetal morbidity is high with an increased incidence of congenital problems (8).
Chest X-ray showed enlarged right atrium with cardiomegaly, while 2D echocardiogram showed the presence of moderately enlarged right atrium and ventricle, tricuspid regurgitation with downwardly displaced tricuspid valves, confirming diagnosis of Ebstein's anomaly. There was no atrial septal defect or pulmonary hypertension.
Many of us learned in medical school that lithium is a known teratogen and should not be used in pregnancy, but we now know that its teratogenicity is relatively modest: The risk of Ebstein's anomaly is about 0.05% among babies exposed to lithium in the first trimester.
Sue suffered from Ebstein's Anomaly, a rare congenital heart condition.
[1] There is an association between MVP and other congenital heart diseases such as ostium secundum atrial septal defect, hypertrophic cardiomyopathy, Marfan's syndrome, Ebstein's anomaly and Wolf Parkinson White (WPW) syndrome, although these are rare occurrences.