Echocardiographic Assessment of Ebstein's Anomaly

Booker, Oscar Julian; Nanda, Navin C.

doi:10.1111/echo.12486

Cited by 32 publications

(20 citation statements)

References 42 publications

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“…Nonetheless, each patient has its own characteristics, mainly manifested in the differences among the tricuspid septal lobe, posterior lobe lesions, and occasionally anterior lobular abnormalities that shift down or block the right ventricular out ow tract. Additionally, the right ventricle looks abnormal, the right ventricle cavity below the tricuspid valve is signi cantly reduced, and the trabecular part also becomes smaller [6][7] . The funnel part can be narrowed by residual valve tissue or abnormal muscle bundles or ber bundles 8 .…”

Section: Discussionmentioning

confidence: 99%

Echocardiographic Characteristics and Prognosis of the Fetuses with Ebstein Anomaly in Pregnancy

et al. 2020

Preprint

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Objective: The aim of this study was to describe the fetal echocardiographic features of Ebstein anomaly (EA) and also to evaluate the prognosis of those fetuses.Methods: We retrospectively reviewed the clinical records of 53 fetuses with EA who were enrolled from January 2011 to December 2018 in Beijing Anzhen Hospital, and analyzed the ultrasonic features and prognosis of the fetuses. Results: In this single-institutional study, 53 fetuses were screened after 23~39 weeks of gestation with EA. In these fetuses, patients with complications accounted for a greater percentage (69.81%). Ultrasound imaging showed that the posterior lobe shifted down further than the septal leaflets, and the anterior lobe changed less than 50%. Severe tricuspid valve (TV) regurgitation showed the highest proportion in these fetuses (56.60%). Among the enrolled fetuses, 20 cases were classified into grade Ⅱ. 40 cases (75.47%) were inducted, and 13 cases (24.5%) were selected for continued pregnancy. However, only one of the fetuses developed till successful delivery. Conclusion: Echocardiography has become an important diagnostic tool in identifying the fetuses with EA, a rare and heterogeneous congenital heart disorder. The prognosis of the fetuses with EA is relatively poor. Therefore, understanding the echocardiographic characteristics and the prognosis of the fetuses with EA is of great value for pregnancy consultations during the perinatal period.

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Section: Discussionmentioning

confidence: 99%

Echocardiographic Characteristics and Prognosis of the Fetuses with Ebstein Anomaly in Pregnancy

et al. 2020

Preprint

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“…These echocardiographic and CT SIs were proportionate to the echocardiographically-defined Carpentier type in types A-C. Theoretically, the fRV area is subject to underestimation, because the majority of the fRV is out-of-plane on a four-chamber view. In fact, the fRV size appears generally larger on 3D imaging than it does on 2D imaging [26]. Therefore, there is a need for better severity assessment using the 3D approach to determine optimal surgical timing and the appropriate surgical procedure in Ebstein anomaly given its diverse regional patterns.…”

Section: Discussionmentioning

confidence: 99%

Volumetric Severity Assessment of Ebstein Anomaly Using Three-Dimensional Cardiac CT: A Feasibility Study

Goo

2019

Cardiovasc Imaging Asia

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Ebstein anomaly is a rare congenital heart disease that accounts for <1% of all congenital heart diseases. It is characterized by a congenital malformation of the tricuspid valve and the right ventricle (RV) [1]. Carpentier and Celermajer classifications, which are based on echocardiographic findings, have been used to predict the clinical outcomes in Ebstein anomaly [1] and to determine the surgical options according to its severity [2]. However, echocardiography cannot provide an accurate volumetric severity index (SI). Cardiac MRI has also been used to quantify ventricular volumes and function in Ebstein anomaly. However, cine MRI is limited by its ability to accurately segment the malformed tricuspid valve and RV endocardial borders because of its two-dimensional (2D) nature [3][4][5][6].Cardiac MRI is generally limited by its long examination time, which often requires conscious sedation or general anesthesia. In addition, the threshold-based segmentation results of threedimensional (3D) whole-heart MRI data are often influenced by inhomogeneous signal intensity and imaging artifacts [7]. In contrast, cardiac CT is increasingly used to evaluate congenital cc This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (https://creativecommons.org/licenses/bync/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.Objective: The purpose of this study was to demonstrate the feasibility of using cardiac CT to perform volumetric severity assessment of the Ebstein anomaly. Materials and Methods:Six children with Ebstein anomaly were included. We used cardiac CT to measure the delineated tricuspid valve, streak artifacts, and contrast enhancement heterogeneity. Cardiac CT data were used to quantify the volumes of all of the cardiac chambers, including the atrial and functional right ventricle (RV). In addition, the functional RV (fRV) fraction, fRV/left ventricle (LV) volume ratio, and total right/left-volume index were calculated. The volume-based CT severity index was compared using area-based echocardiographic and CT severity indices.Results: All of the patients had grade 3 or 4 extent of delineation of the malformed tricuspid valve and streak artifacts. In one case, the right atrium had image noise [126.7 Hounsfield units (HU)] that was >50 HU, while all others demonstrated image noise <50 HU. This image quality was appropriate to make all of the volumetric measurements. The RV volumes were enlarged in 83.3% (5/6) of the cases. The fRV fraction was 60.8-61.5%. The fRV/LV volume ratio and total right/left-volume index were 2.0±1.4 and 3.5±1.5, respectively. There was a smaller difference (0.2±0.1) between the area-based and volume-based CT severity indices than there was in other comparisons. These parameters also had a higher concordance rate (83.3%, 5/6) in the severity grades. Conclusion:Volumetric severity assessment using cardiac CT is feasible in Ebstein anomal...

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“…Tricuspid valve repair was previously not very successful but outcomes are now promising thanks to new operating techniques and better preoperative evaluation, including 3D echocardiography. Indications for surgical repair include severe TR and NYHA class higher than II or arrhythmias or deteriorating exercise capacity [1][2][3]. In selected EA cases, TV plasty and reduction of RV size is believed to be the best method of correction.…”

Section: Discussionmentioning

confidence: 99%

Ebstein’s anomaly treated surgically in an adult female

et al. 2016

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A 21-year-old woman with Ebstein's anomaly was admitted with syncope on exertion, fatigue and dyspnea (NYHA III). Physical examination revealed systolic apical murmur. Echocardiography showed normal LVEF, apical displacement of the septal tricuspid leaflet, severe tricuspid regurgitation, right ventricle dilatation and dysfunction. After detailed evaluation, surgery of the right atrioventricular ostium was proposed. Five months after surgery the patient reported an improvement in exercise tolerance (NYHA II). Echocardiography showed mild residual tricuspid regurgitation.

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Echocardiographic Assessment of Ebstein's Anomaly

Cited by 32 publications

References 42 publications

Echocardiographic Characteristics and Prognosis of the Fetuses with Ebstein Anomaly in Pregnancy

Echocardiographic Characteristics and Prognosis of the Fetuses with Ebstein Anomaly in Pregnancy

Volumetric Severity Assessment of Ebstein Anomaly Using Three-Dimensional Cardiac CT: A Feasibility Study

Ebstein’s anomaly treated surgically in an adult female

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