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      The Surgical Management of Ebstein Anomaly

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          Abstract

          Among all congenital heart disease, Ebstein anomaly is the lesion with the widest spectrum of presentation. It can present as early as the neonatal period where immediate intervention is often needed to the adult with no discernable symptoms. It is also one where overall outcome greatly relates to age at presentation. Generally, presentation in the neonatal period is associated with poor outcomes while presentation beyond the neonatal period has excellent outcomes with low operative mortality. It is thus apparent that understanding every aspect of the anatomy and physiology of Ebstein anomaly and how it manifests clinically is paramount to successful treatment. The purpose of this review is to outline the current concepts and strategies in the surgical management of Ebstein anomaly, and to discuss the current expert consensus on how best to tackle this congenital heart disease at different ages of diagnosis.

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          Most cited references 18

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          A reevaluation of risk of in utero exposure to lithium.

          To reevaluate the risk associated with in utero exposure to lithium. Data were obtained from all published studies, in multiple languages, referenced in MEDLINE, Toxline, and the Lithium Information Center databases. Unpublished studies were not included. The search terms were lithium, pregnancy, teratogen, abnormalities (drug induced), Ebstein's anomaly, and adverse effects. In the 1970s a very strong association was suggested between maternal lithium treatment during pregnancy and Ebstein's anomaly of the heart in the offspring. The relative risk for Ebstein's anomaly among such children was estimated to be 400 on the basis of data collected from a registry of voluntarily submitted cases. More recent controlled epidemiologic studies have consistently shown a lower risk. No women who took lithium during pregnancy were found among four case-control studies of Ebstein's anomaly involving 25, 34, 59, and 89 affected children, respectively. In two cohort studies, risk ratios of 3.0 (95% confidence interval [CI], 1.2 to 7.7) and 1.5 (95% CI, 0.4 to 6.8) for all congenital anomalies have been observed. The risk ratios for cardiac malformations in these studies were 7.7 (95% CI, 1.5 to 41.2) and 1.2 (95% CI, 0.1 to 18.3), respectively. While initial information regarding the teratogenic risk of lithium treatment was derived from biased retrospective reports, more recent epidemiologic data indicate that the teratogenic risk of first-trimester lithium exposure is lower than previously suggested. The clinical management of women with bipolar disorder who have childbearing potential should be modified with this revised risk estimate.
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            A new reconstructive operation for Ebstein's anomaly of the tricuspid valve.

            Ebstein's anomaly is a complex malformation involving the tricuspid valve and the right ventricle. Various surgical techniques, either repair or replacement of the abnormal tricuspid valve, have been used with variable results. In an attempt to enlarge the indications of conservative procedures, we developed a technique of repair that comprises the reconstruction of a normally shaped right ventricle and the repositioning of the tricuspid valve at the normal level. In a series of 14 patients operated on between January 1980 and December 1986 in our institution, only one patient with an associated atrioventricular septal defect required a tricuspid valve replacement. All of the other patients, regardless of the complexity of the tricuspid malformation, were able to benefit from this conservative technique. There were two hospital deaths and no late deaths in this series. All surviving patients displayed a marked improvement over their preoperative status with regard to functional class (92% are in class I or II) and rhythm disturbances. Echocardiographic and Doppler studies demonstrated a normal shape of the right ventricle and good tricuspid valve function in all the patients but one.
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              The outcomes of operations for 539 patients with Ebstein anomaly

              Our objective was to review the long-term outcomes of patients with Ebstein anomaly who underwent cardiac surgery at our institution.
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                Author and article information

                Journal
                CVIA
                Cardiovascular Innovations and Applications
                CVIA
                Compuscript (Ireland )
                2009-8782
                2009-8618
                May 2018
                May 2018
                : 3
                : 1
                : 13-20
                Affiliations
                1University of Florida, Congenital Heart Center, Congenital Heart Surgery, 1600 Southwest Archer Road, Gainesville, FL 32608, USA
                2University of Florida, Department of Surgery, Division of Thoracic and Cardiovascular Surgery, 1600 Southwest Archer Road, Gainesville, FL 32608, USA
                aBoth authors equally contributed to this work.
                Author notes
                Correspondence: Karl M. Reyes, MD, Associate Professor, Congenital Heart Center, Departments of Surgery and Pediatrics, Congenital Heart Surgery, University of Florida – Shands Children’s Hospital, 1600 Archer Road – HD 308, Gainesville, FL 32608, USA, Tel.: +(352) 273-9569, Fax: +(352) 273-5927, E-mail: karl.reyes@ 123456peds.ufl.edu
                Article
                cvia20170052
                10.15212/CVIA.2017.0052
                Copyright © 2018 Cardiovascular Innovations and Applications

                This is an open-access article distributed under the terms of the Creative Commons Attribution 4.0 Unported License (CC BY-NC 4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. See https://creativecommons.org/licenses/by-nc/4.0/.

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