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

Echocardiographic Characteristics and Prognosis of the Fetuses with Ebstein Anomaly in Pregnancy | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Help Center Sign In Submit a Preprint Cite Share Download PDF Research Echocardiographic Characteristics and Prognosis of the Fetuses with Ebstein Anomaly in Pregnancy Yuduo Wu, Xiaoyan Gu, Yihua He, Hongjia Zhang This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-69914/v1 This work is licensed under a CC BY 4.0 License Status: Posted Version 1 posted You are reading this latest preprint version Abstract 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. Maternal & Fetal Medicine Ebstein anomaly fetal echocardiography prognosis Figures Figure 1 Figure 2 Figure 3 Figure 4 Figure 5 1 Introduction Ebstein anomaly (EA) is a congenital defect of the tricuspidvalve (TV), characterized by varying degrees of downward displacement of the TV leaflets into the cavity of the right ventricle 1 . EA accounts for less than 1% of all cases of congenital heart disease 2 . Current reports predominantly describe postnatal diagnosis of EA in neonates or infants. However, fetal presentation in EA is predicted to have a poor overall prognosis as the age at presentation is dependent on the severity of anatomic and hemodynamic derangements 3 . In this study, we evaluated the echocardiographic features and the prognosis of the fetuses with EA in pregnancy. Additionally, we analyzed the echocardiographic data that can be used as potential prognostic factors for fetuses with EA and tricuspid valve dysplasia to provide more valuable information on perinatal consultation. 2 Materials And Method 2.1 Study design and patient selection A total of 34,756 fetuses were examined from January 2011 to December 2018 in Beijing Anzhen Hospital, Capital Medical University, Beijing, China. The cohort of 53 fetuses (all singletons) diagnosed with EA were retrospectively identified in the perinatal databases of the tertiary centers according to the prenatal demographic, clinical, and echocardiographic data. All procedures involving human participants followed to the ethical standards of the institutional and/or national research committee, and the 1964 Helsinki declaration and its later amendments or comparable ethical standards. 2.2 Echocardiography All patients underwent ultrasound screening with high resolution ultrasound equipments (Voluson E8; General Electric). Ultrasound settings were standardized. In all cases, patients underwent detailed fetal echocardiographic examinations to evaluate the cardiac structures and function, which included fetal biometric measurements, and visualization of a normal 4-chamber, outflow tract, 3iewsow, 3iewsow l 4ion ofmea, and aortic and ductal arches. Color flow mapping and Pulsed Wave Doppler imaging were also performed. Moreover, extracardiac anomalies of the fetuses were also evaluated. Each study was documented by hardcopy images. The right atria (RA), left atria (LA), atrialized right ventricule (aRV), functional right ventricule (fRV), and left ventricule (LV) were measured separately. In 25 cases, the area ratio of (RA + aRV)/(LA + LV + fRV) was calculated (Fig. 1 ). According to these data, the tricuspid valve downward shift of the fetuses was grouped into four levels, in which the severity is associated with the prognosis of EA patients. The classification standard was set as follows: Grade I < 0.5, excellent prognosis; Grade II 0.5–0.99, good prognosis, survival rate is 92%; Grade III 1-1.49, poor prognosis, early mortality rate is 10%; Grade IV ≥ 1.5, extremely poor prognosis, and the mortality rate is 100%. 2.3 Statistical analyses Statistical analysis was performed using the SPSS software (SPSS version 22.0, SPSS, Inc., Chicago, IL, USA). Data including observational data were expressed as frequency histograms or absolute and percentage frequency values. Continuous data are expressed as mean SD and/or median and range, as appropriate. 3 Results 3.1 Data from prenatal ultrasound We evaluated the outcomes of 53 pregnant patients with EA. The pregnant women were aged from 17 to 40, with an average of 28. All the women enrolled had a single pregnancy. At the time of initial diagnosis, the gestational age ranged from 23 to 39 weeks, with an average of 28 + 6 weeks. Characteristics of the cases are summarized in Table 1 and Fig. 1 . Table 1 Characteristics of pregnant women and echocardiographic characteristics of fetuses with EA Item Pregnant age (years) 17–40 Gestational age at admission (weeks) 23–39 Fetuses with comorbidity n (%) Yes 37 (69.81%) No 16 (30.19%) The downward displacement of the TV Septal leaflets(mm) 6.86 ± 3.40 Posterior leaflets(mm) 9.11 ± 4.76 Changed in anterior leaflets n (%) 19(35.85%) TV regurgitation Mild n (%) 6(11.32%) Moderate n (%) 15(28.30%) Severe n (%) 32(56.60%) Severely n (%) 2(3.77%) Area ratio class at gestationn (%) I 1(4.00%) II 2(8.00%) III 20(80.00%) IV 2(8.00%) TV = tricuspidvalve; Area ratio=(RA + aRV)/(LA + LV + fRV). 3.2 Fetal new birthrate and induction rate In this study, 17 cases (%) of all enrolled fetuses were selected for continued pregnancy. Among the 53 fetuses, 16 cases showed simple EA but 37 cases showed EA-associated malformations (Fig. 2 ). In the pregnant women who continued with the pregnancy, only one of the fetuses was then delivered successfully. In this study, the birth rate of the fetus was 1.89% (1/53). The prenatal echocardiogram for this fetus is shown in Fig. 3 . On the second day after birth, the first postnatal echocardiogram was obtained, in which tricuspid septal displacement deformity, moderate tricuspid regurgitation, and a slightly dilated left atrium (LA) were presented (Fig. 4). The newborn had no other symptoms of discomfort and regular reexamination was conducted. The distribution histogram was generated according to the number of pregnant women with distinct fetal malformations (Fig. 5 ). The number of pregnant women who chose to terminate their pregnancy is relatively high, 40 in total (in red) including 10 without complications and 30 with complications, whereas the number of pregnant women who chose to continue their pregnancy is relatively low, 13 in total (in blue) including 6 without complications and 7 with complications . 4 Discussion Ebstein’s anomaly (EA) is a rare congenital heart disorder. Fetal diagnosis and presentations of the disease are typically the most severe and are associated with the highest mortality rates 4 – 5 . In the present study, we found that fetal echocardiography is a preferred tool to diagnose the lesion accurately. Two-dimensional echocardiograms were performed in an apical four-chamber view. The images mainly showed that (1) the tricuspid valve septum and posterior lobe can shiftdown, which may cause severe dysplasia, thus forming membrane-like remnants, and leading to abnormal development of leaflets, chordae, and papillary muscles; (2) although the anterior lobe is attached to the normal level of the annulus, it may be larger, and sail-shaped. The chordae space disappeared or adhered to the right ventricular wall to varying degrees and, in some cases, may be hypoplastic or down; (3) the annulus is enlarged; (4) the arterialized right ventricle and the right atrium is significantly expanded. 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 outflow tract. Additionally, the right ventricle looks abnormal, the right ventricle cavity below the tricuspid valve is significantly 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 fiber bundles 8 . In this study, 53 cases of EA were diagnosed by prenatal echocardiography, all of which had the above typical ultrasonographic manifestations of deformity. EA can be diagnosed prenatally granted the prenatal ultrasound examination reveals the above manifestations of the fetal heart.Since this complex congenital anomaly has a very variable anatomical and clinical spectrum,early diagnosis can provide patients with a timely and effective perinatal treatment plan. This is consistent with previous studies describing 9 – 11 the high detection rate of other congenital complex diseases with prenatal ultrasound, indicating that echocardiography is worth pursuing in clinical practice. Fetuses diagnosed with EA have a poor prognosis, and the total mortality rate is as high as 80% or more 12 . Mild EA had better prognosis compared to severe EA. However, worse prognosis is observed when EA is combined with other diseases, consistent with the conclusions in our study. Other studies also reported that factors, including fetal edema, cardiac hypertrophy, outflow obstruction, and cardiothoracic ratio > 0.55, are suggestive of poor prognosis for the fetuses 13 . Upon longer duration of TV malformation, the disease will be getting severe gradually; the right heart will be overloaded, thus inducing right heart failure, and increasing the burden of the right heart. In severe cases, the right heart can be twice size as the left heart, indicating that the prognosis of the fetus is extremely poor and requires timely treatment to ensure the safety of the child. Issues regarding the importance of the atrialized chamber have also been raised 14 – 15 . In this study, only one fetus was regularly followed up and examined by our center with an induction rate of 98.11% (1/53), which is much higher than the mortality rate of EA patients in previous studies. The main reason is that fetuses with severe or severely late tricuspid regurgitation as well as those with combined malformations account for a large proportion of cases. Our center is a cardiovascular hospital, and most of the patients are mothers with critically ill fetuses. Although the diagnosis can be made in time, the efforts seeking for treatment during the perinatal period cannot be exerted, which may contribute to a low birth rate of patients. The high proportion of grade Ⅲ and grade Ⅳ in the grading area associated with prognosis can also be explained by such a high rate of induction. On the other hand, it also shows that with the development of examination assistive technology, more congenital heart diseases can be identified in the fetal period. It is noteworthy that the high detection rate is also a factor contributing to the increase of the death rate, although this needs further verification. Although this is one of the largest cohorts of fetuses with EA, the sample size is still relatively small, which is therefore limited to provide a strong conclusion. Additionally this is a single-institutional retrospective study. Many patients were referred to us from other centers. As a result, there is potential patient selection bias which may have led to the data, and results presented here may not be directly applicable to other centers and populations. 5 Conclusions Echocardiography has become an important tool in identifying the fetuses with EA, a rare and heterogeneous congenital heart disorder. The prognosis of EA patients during the fetal period is relatively poor. Therefore, understanding the echocardiographic characteristics and the prognosis of fetal EA is of great value for pregnancy consultations during the perinatal period. Abbreviations EA Ebstein anomaly; TV:Tricuspid valve; RA:Right atria; LA:Left atria; aRV:Atrialized right ventricule; fRV:functional right ventricule; LV:Left ventricule. Declarations Acknowledgements Not applicable. Authors’ contributions Yuduo Wu was responsible for data collection, data analysis, and manuscript preparation; Xiaoyan Gu was responsible for data interpretation; Yihua He was responsible for manuscript revision and finalization. Funding was secured by Hongjia Zhang. Founding This study was supported by National Science Funding (No.81301241), Beijing Key Laboratory of Maternal-Fetal Medicine and Fetal Heart Disease (No. BZ0308), and Beijing Municipal Administration of Hospitals’ Ascent Plan (No. DFL20180602). Availability of data and materials The datasets analyzed for the current study are available from the correspond‑ ing author on reasonable request. Ethics approval and consent to participate The human study were approved by the ethics committee of Beijing Anzhen Hospital. Written informed consent was obtained from all subjects. Consent for publication Not applicable. Competing interests The authors declare that they have no competing interests. Author details 1 Department of Cardiac Surgery, Beijing Anzhen Hospital, Capital Medical University, Beijing, China; 2 Department of Echocardiography, Beijing Key Laboratory of Maternal‐Fetal Medicine in Fetal Heart Disease, Beijing Anzhen Hospital, Capital Medical University, Beijing, China; Address for co-correspondence: Yihua He, Department of Echocardiography, Beijing Key Laboratory of Maternal‐fetal Medicine in Fetal Heart Disease, Beijing Anzhen Hospital, Capital Medical University, No.2, Anzhen Road, Chaoyang District, Beijing 100029, China. Email: [email protected] ; fax number: +86 10 84006368, telephone：18910778673. Hongjia Zhang, Department of Cardiac Surgery, Beijing Anzhen Hospital, Capital Medical University, No.2, Anzhen Road, Chaoyang District, Beijing 100029, China. E-mail: [email protected] . fax numbers: +86 10 85231211, telephone：13311516256. References I G, L G, R S, et al. Ebstein's Anomaly of the Tricuspid Valve in the Fetus- A Multicenter Experience.[J]. Ultraschall in der Medizin (Stuttgart, Germany: 1980), 2017,38(4):427–436. Shetty RK, Vivek G, Nayak K, et al. Fetal Ebstein's anomaly[J]. BMJ Case Rep, 2012,2012. LR F, MC E, BT K, et al. Outcomes and Predictors of Perinatal Mortality in Fetuses With Ebstein Anomaly or Tricuspid Valve Dysplasia in the Current Era: A Multicenter Study.[J]. Circulation. 2015;132(6):481–9. Freud LR, Escobar-Diaz MC, Kalish BT, et al. Outcomes and Predictors of Perinatal Mortality in Fetuses With Ebstein Anomaly or Tricuspid Valve Dysplasia in the Current Era: A Multicenter Study[J]. Circulation. 2015;132(6):481–9. Gottschalk I, Gottschalk L, Stressig R, et al. Ebstein's Anomaly of the Tricuspid Valve in the Fetus - A Multicenter Experience[J]. Ultraschall Med. 2017;38(4):427–36. Prota C, Di Salvo G, et al. "Prognostic value of echocardiographic parameters in pediatric patients with Ebstein's anomaly.". Int J Cardiol. 2019;278:76–83. Booker OJ, Nanda NC. Echocardiographic assessment of Ebstein'sanomaly[J]. Echocardiography, 2015,32Suppl 2:S177-S188. Gottschalk I, Gottschalk L, Stressig R, et al. Ebstein's Anomaly of the Tricuspid Valve in the Fetus-AMulticenter Experience[J]. Ultraschall Med. 2017;38(4):427–36. Kaya B, Ekiz A, Acar DK, et al. Prenatal diagnosis of aortopulmonary window by foetal echocardiography: 'U or reversed U sign'[J]. J ObstetGynaecol. 2019;39(5):712–3. Yu S, Han J, Gao S, et al. The prenatal diagnosis of aortopulmonary window by fetal echocardiography[J]. Echocardiography. 2018;35(11):1835–40. Garcia C, Alvarez T, Bravo C, et al. Prenatal Diagnosis of an Aortopulmonary Window With an Interrupted Aortic Arch[J]. J Ultrasound Med. 2016;35(10):2087–93. Pornprasertchai V, Vijarnsorn C, Kanjanauthai S, et al. Contemporary outcomes and mortality risks of Ebstein anomaly: A single-center experience in Thailand[J]. Congenit Heart Dis, 2019. Zimmer EZ, Blazer S, Lorber A, et al. Fetal Ebstein's anomaly: early and late appearance[J]. PrenatDiagn. 2012;32(3):228–33. RS L, LG A, MA A, et al. Diagnosis and prognosis of fetal ebstein anomaly.[J]. Ginecologia y obstetricia de Mexico. 2014;82(2):93–104. Selamet TE, McElhinney DB, Freud LR, et al. Assessment of Progressive Pathophysiology After Early Prenatal Diagnosis of the Ebstein Anomaly or Tricuspid Valve Dysplasia[J]. Am J Cardiol. 2017;119(1):106–11. 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Also discoverable on Platform About Our Team In Review Editorial Policies Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-69914","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Research","associatedPublications":[],"authors":[{"id":1994589,"identity":"154adb41-405f-42ad-a255-7c48cdd929a3","order_by":0,"name":"Yuduo Wu","email":"","orcid":"","institution":"Capital Medical University Affiliated Anzhen Hospital","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Yuduo","middleName":"","lastName":"Wu","suffix":""},{"id":1994590,"identity":"b3044062-13bf-495a-b3fb-1e910e9c858d","order_by":1,"name":"Xiaoyan Gu","email":"","orcid":"","institution":"Capital Medical University Affiliated Anzhen Hospital","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Xiaoyan","middleName":"","lastName":"Gu","suffix":""},{"id":1994591,"identity":"95a1b4d0-c50a-4ef1-b05e-2db30dc3d60d","order_by":2,"name":"Yihua He","email":"","orcid":"","institution":"Capital Medical University Affiliated Anzhen Hospital","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Yihua","middleName":"","lastName":"He","suffix":""},{"id":1994592,"identity":"9eb2fbcc-6f1f-4c92-b213-589ead2af1d7","order_by":3,"name":"Hongjia Zhang","email":"data:image/png;base64,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","orcid":"https://orcid.org/0000-0002-5737-4084","institution":"Beijing An Zhen Hospital: Capital Medical University Affiliated Anzhen Hospital","correspondingAuthor":true,"submittingAuthor":false,"prefix":"","firstName":"Hongjia","middleName":"","lastName":"Zhang","suffix":""}],"badges":[],"createdAt":"2020-09-01 10:23:25","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-69914/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-69914/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":2257986,"identity":"1b16595b-71d6-48b5-bfdd-871ecfbcde92","added_by":"auto","created_at":"2020-09-04 21:51:23","extension":"jpg","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":30551,"visible":true,"origin":"","legend":"Measurements were done in a four-chamber section of the heart at the end of diastole. Area ratio =(RA+ aRV)/( fRV+LA+LV). 1=RA+ aRV, 2=fRV, 3=LA, 4=LV. Among the enrolled fetuses, twenty-five cases were graded according to the EA severity. One case in grade Ⅰ, twenty cases in grade Ⅱ, two cases in grade Ⅲ, and two cases in grade Ⅳ. RA: right atria; LA: left atria; aRV: atrialized right ventricule; fRV: functional right ventricule; LV: left ventricule. ","description":"","filename":"1.JPG","url":"https://assets-eu.researchsquare.com/files/rs-69914/v1/1.JPG"},{"id":2257987,"identity":"e0b10cc4-0d94-445c-8627-c95b0bda4c4e","added_by":"auto","created_at":"2020-09-04 21:51:23","extension":"jpg","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":30095,"visible":true,"origin":"","legend":"A schema of echocardiographic analysis for the enrolled fetuses with EA.","description":"","filename":"2.JPG","url":"https://assets-eu.researchsquare.com/files/rs-69914/v1/2.JPG"},{"id":2257988,"identity":"1136ecfb-435d-4529-a0f6-7489da2d243f","added_by":"auto","created_at":"2020-09-04 21:51:23","extension":"jpg","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":33730,"visible":true,"origin":"","legend":"Prenatal ultrasound examinations for the developing fetus in a four-chamber view. (A) Two-dimensional ultrasound. Two-way arrows indicate the downward shift of septum tricuspid at 28 wks of gestation. (B) Color flow mapping. Color Doppler flow imaging indicates a moderate tricuspid regurgitation before birth (the arrow mark). tRA: right atrium, aRV: Atrialized right ventricle, fRV: Functional right ventricle, LA: left atrium; LV: left ventricle.","description":"","filename":"3.JPG","url":"https://assets-eu.researchsquare.com/files/rs-69914/v1/3.JPG"},{"id":2257989,"identity":"e509bbb9-f1c9-4816-8206-d911506bcdd5","added_by":"auto","created_at":"2020-09-04 21:51:23","extension":"jpg","order_by":4,"title":"Figure 4","display":"","copyAsset":false,"role":"figure","size":27009,"visible":true,"origin":"","legend":"The postnatal ultrasound\t examination from the developing fetus in the four-chamber view. (A) Two-dimensional ultrasound. Two-way arrows indicate the TV septal descending. (B) Color flow mapping. Color Doppler indicates the tricuspid regurgitation. TV=tricuspid valve. ","description":"","filename":"4.JPG","url":"https://assets-eu.researchsquare.com/files/rs-69914/v1/4.JPG"},{"id":2257990,"identity":"2283cde2-0b5d-41f5-8ac0-78a3161d15eb","added_by":"auto","created_at":"2020-09-04 21:51:23","extension":"jpg","order_by":5,"title":"Figure 5","display":"","copyAsset":false,"role":"figure","size":38510,"visible":true,"origin":"","legend":"The distribution of patients was presented under each relevant indicator. P=0 termination of pregnancy (red), P=1 continuation of pregnancy (blue).","description":"","filename":"5.JPG","url":"https://assets-eu.researchsquare.com/files/rs-69914/v1/5.JPG"},{"id":13588115,"identity":"f56aaf59-f925-4863-9b18-dfbf8fbb3295","added_by":"auto","created_at":"2021-09-17 04:54:09","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":431236,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-69914/v1/487099c9-796f-43c4-b578-1a7ab9214467.pdf"}],"financialInterests":"","formattedTitle":"\u003cp\u003eEchocardiographic Characteristics and Prognosis of the Fetuses with Ebstein Anomaly in Pregnancy\u003c/p\u003e","fulltext":[{"header":"1 Introduction","content":" \u003cp\u003eEbstein anomaly (EA) is a congenital defect of the tricuspidvalve (TV), characterized by varying degrees of downward displacement of the TV leaflets into the cavity of the right ventricle\u003csup\u003e\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e\u003c/sup\u003e. EA accounts for less than 1% of all cases of congenital heart disease\u003csup\u003e\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e\u003c/sup\u003e. Current reports predominantly describe postnatal diagnosis of EA in neonates or infants. However, fetal presentation in EA is predicted to have a poor overall prognosis as the age at presentation is dependent on the severity of anatomic and hemodynamic derangements\u003csup\u003e\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e\u003c/sup\u003e. In this study, we evaluated the echocardiographic features and the prognosis of the fetuses with EA in pregnancy. Additionally, we analyzed the echocardiographic data that can be used as potential prognostic factors for fetuses with EA and tricuspid valve dysplasia to provide more valuable information on perinatal consultation.\u003c/p\u003e "},{"header":"2 Materials And Method","content":"\u003cp\u003e\u003cb\u003e2.1\u0026emsp;Study design and patient selection\u003c/b\u003e \u003c/p\u003e \u003cp\u003eA total of 34,756 fetuses were examined from January 2011 to December 2018 in Beijing Anzhen Hospital, Capital Medical University, Beijing, China. The cohort of 53 fetuses (all singletons) diagnosed with EA were retrospectively identified in the perinatal databases of the tertiary centers according to the prenatal demographic, clinical, and echocardiographic data. All procedures involving human participants followed to the ethical standards of the institutional and/or national research committee, and the 1964 Helsinki declaration and its later amendments or comparable ethical standards.\u003c/p\u003e \u003cp\u003e \u003cb\u003e2.2\u0026emsp;Echocardiography\u003c/b\u003e \u003c/p\u003e \u003cp\u003eAll patients underwent ultrasound screening with high resolution ultrasound equipments (Voluson E8; General Electric). Ultrasound settings were standardized. In all cases, patients underwent detailed fetal echocardiographic examinations to evaluate the cardiac structures and function, which included fetal biometric measurements, and visualization of a normal 4-chamber, outflow tract, 3iewsow, 3iewsow l 4ion ofmea, and aortic and ductal arches.\u003c/p\u003e \u003cp\u003eColor flow mapping and Pulsed Wave Doppler imaging were also performed. Moreover, extracardiac anomalies of the fetuses were also evaluated. Each study was documented by hardcopy images. The right atria (RA), left atria (LA), atrialized right ventricule (aRV), functional right ventricule (fRV), and left ventricule (LV) were measured separately. In 25 cases, the area ratio of (RA\u0026thinsp;+\u0026thinsp;aRV)/(LA\u0026thinsp;+\u0026thinsp;LV\u0026thinsp;+\u0026thinsp;fRV) was calculated (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003e). According to these data, the tricuspid valve downward shift of the fetuses was grouped into four levels, in which the severity is associated with the prognosis of EA patients. The classification standard was set as follows: Grade I\u0026thinsp;\u0026lt;\u0026thinsp;0.5, excellent prognosis; Grade II 0.5\u0026ndash;0.99, good prognosis, survival rate is 92%; Grade III 1-1.49, poor prognosis, early mortality rate is 10%; Grade IV\u0026thinsp;\u0026ge;\u0026thinsp;1.5, extremely poor prognosis, and the mortality rate is 100%.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003e \u003cb\u003e2.3\u0026emsp;Statistical analyses\u003c/b\u003e \u003c/p\u003e \u003cp\u003eStatistical analysis was performed using the SPSS software (SPSS version 22.0, SPSS, Inc., Chicago, IL, USA). Data including observational data were expressed as frequency histograms or absolute and percentage frequency values. Continuous data are expressed as mean SD and/or median and range, as appropriate.\u003c/p\u003e "},{"header":"3 Results","content":"\u003cp\u003e \u003cb\u003e3.1\u0026emsp;Data from prenatal ultrasound\u003c/b\u003e \u003c/p\u003e \u003cp\u003eWe evaluated the outcomes of 53 pregnant patients with EA. The pregnant women were aged from 17 to 40, with an average of 28. All the women enrolled had a single pregnancy. At the time of initial diagnosis, the gestational age ranged from 23 to 39 weeks, with an average of 28\u003csup\u003e+\u0026thinsp;6\u003c/sup\u003e weeks. Characteristics of the cases are summarized in Table\u0026nbsp;\u003cspan refid=\"Tab1\" class=\"InternalRef\"\u003e1\u003c/span\u003e and Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003e.\u003c/p\u003e \u003cp\u003e \u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab1\" border=\"1\"\u003e \u003ccaption language=\"En\"\u003e \u003cdiv class=\"CaptionNumber\"\u003eTable 1\u003c/div\u003e \u003cdiv class=\"CaptionContent\"\u003e \u003cp\u003eCharacteristics of pregnant women and echocardiographic characteristics of fetuses with EA\u003c/p\u003e \u003c/div\u003e \u003c/caption\u003e \u003ccolgroup cols=\"2\"\u003e \u003cthead\u003e \u003ctr\u003e \u003cth align=\"left\" colname=\"c1\"\u003e \u003cp\u003eItem\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/th\u003e \u003c/tr\u003e \u003c/thead\u003e \u003ctbody\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003ePregnant age (years)\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e17\u0026ndash;40\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003eGestational age at admission (weeks)\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e23\u0026ndash;39\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003eFetuses with comorbidity n (%)\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eYes\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e37 (69.81%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eNo\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e16 (30.19%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003eThe downward displacement of the TV\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSeptal leaflets(mm)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e6.86\u0026thinsp;\u0026plusmn;\u0026thinsp;3.40\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003ePosterior leaflets(mm)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e9.11\u0026thinsp;\u0026plusmn;\u0026thinsp;4.76\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003eChanged in anterior leaflets n (%)\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e19(35.85%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003eTV regurgitation\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eMild n (%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e6(11.32%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eModerate n (%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e15(28.30%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSevere n (%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e32(56.60%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSeverely n (%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e2(3.77%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003eArea ratio class at gestationn (%)\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eI\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e1(4.00%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eII\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e2(8.00%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eIII\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e20(80.00%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eIV\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e2(8.00%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003c/tbody\u003e \u003c/colgroup\u003e \u003ctfoot\u003e \u003ctr\u003e\u003ctd colspan=\"2\"\u003eTV\u0026thinsp;=\u0026thinsp;tricuspidvalve; Area ratio=(RA\u0026thinsp;+\u0026thinsp;aRV)/(LA\u0026thinsp;+\u0026thinsp;LV\u0026thinsp;+\u0026thinsp;fRV).\u003c/td\u003e\u003c/tr\u003e \u003c/tfoot\u003e \u003c/table\u003e\u003c/div\u003e \u003c/p\u003e \u003cp\u003e \u003cb\u003e3.2\u0026emsp;Fetal new birthrate and induction rate\u003c/b\u003e \u003c/p\u003e \u003cp\u003eIn this study, 17 cases (%) of all enrolled fetuses were selected for continued pregnancy. Among the 53 fetuses, 16 cases showed simple EA but 37 cases showed EA-associated malformations (Fig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003e). In the pregnant women who continued with the pregnancy, only one of the fetuses was then delivered successfully. In this study, the birth rate of the fetus was 1.89% (1/53). The prenatal echocardiogram for this fetus is shown in Fig.\u0026nbsp;\u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e3\u003c/span\u003e. On the second day after birth, the first postnatal echocardiogram was obtained, in which tricuspid septal displacement deformity, moderate tricuspid regurgitation, and a slightly dilated left atrium (LA) were presented (Fig.\u0026nbsp;4). The newborn had no other symptoms of discomfort and regular reexamination was conducted. The distribution histogram was generated according to the number of pregnant women with distinct fetal malformations (Fig.\u0026nbsp;\u003cspan refid=\"Fig4\" class=\"InternalRef\"\u003e5\u003c/span\u003e). The number of pregnant women who chose to terminate their pregnancy is relatively high, 40 in total (in red) including 10 without complications and 30 with complications, whereas the number of pregnant women who chose to continue their pregnancy is relatively low, 13 in total (in blue) including 6 without complications and 7 with complications .\u003c/p\u003e"},{"header":"4 Discussion","content":"\u003cp\u003eEbstein\u0026rsquo;s anomaly (EA) is a rare congenital heart disorder. Fetal diagnosis and presentations of the disease are typically the most severe and are associated with the highest mortality rates \u003csup\u003e\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e\u003c/sup\u003e. In the present study, we found that fetal echocardiography is a preferred tool to diagnose the lesion accurately. Two-dimensional echocardiograms were performed in an apical four-chamber view. The images mainly showed that (1) the tricuspid valve septum and posterior lobe can shiftdown, which may cause severe dysplasia, thus forming membrane-like remnants, and leading to abnormal development of leaflets, chordae, and papillary muscles; (2) although the anterior lobe is attached to the normal level of the annulus, it may be larger, and sail-shaped. The chordae space disappeared or adhered to the right ventricular wall to varying degrees and, in some cases, may be hypoplastic or down; (3) the annulus is enlarged; (4) the arterialized right ventricle and the right atrium is significantly expanded. 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 outflow tract. Additionally, the right ventricle looks abnormal, the right ventricle cavity below the tricuspid valve is significantly reduced, and the trabecular part also becomes smaller\u003csup\u003e\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e\u003c/sup\u003e. The funnel part can be narrowed by residual valve tissue or abnormal muscle bundles or fiber bundles\u003csup\u003e\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e\u003c/sup\u003e. In this study, 53 cases of EA were diagnosed by prenatal echocardiography, all of which had the above typical ultrasonographic manifestations of deformity. EA can be diagnosed prenatally granted the prenatal ultrasound examination reveals the above manifestations of the fetal heart.Since this complex congenital anomaly has a very variable anatomical and clinical spectrum,early diagnosis can provide patients with a timely and effective perinatal treatment plan. This is consistent with previous studies describing\u003csup\u003e\u003cspan additionalcitationids=\"CR10\" citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e\u003c/sup\u003e the high detection rate of other congenital complex diseases with prenatal ultrasound, indicating that echocardiography is worth pursuing in clinical practice.\u003c/p\u003e \u003cp\u003eFetuses diagnosed with EA have a poor prognosis, and the total mortality rate is as high as 80% or more\u003csup\u003e\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e\u003c/sup\u003e. Mild EA had better prognosis compared to severe EA. However, worse prognosis is observed when EA is combined with other diseases, consistent with the conclusions in our study. Other studies also reported that factors, including fetal edema, cardiac hypertrophy, outflow obstruction, and cardiothoracic ratio\u0026thinsp;\u0026gt;\u0026thinsp;0.55, are suggestive of poor prognosis for the fetuses\u003csup\u003e\u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e\u003c/sup\u003e. Upon longer duration of TV malformation, the disease will be getting severe gradually; the right heart will be overloaded, thus inducing right heart failure, and increasing the burden of the right heart. In severe cases, the right heart can be twice size as the left heart, indicating that the prognosis of the fetus is extremely poor and requires timely treatment to ensure the safety of the child. Issues regarding the importance of the atrialized chamber have also been raised\u003csup\u003e\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR15\" class=\"CitationRef\"\u003e15\u003c/span\u003e\u003c/sup\u003e. In this study, only one fetus was regularly followed up and examined by our center with an induction rate of 98.11% (1/53), which is much higher than the mortality rate of EA patients in previous studies. The main reason is that fetuses with severe or severely late tricuspid regurgitation as well as those with combined malformations account for a large proportion of cases. Our center is a cardiovascular hospital, and most of the patients are mothers with critically ill fetuses. Although the diagnosis can be made in time, the efforts seeking for treatment during the perinatal period cannot be exerted, which may contribute to a low birth rate of patients. The high proportion of grade Ⅲ and grade Ⅳ in the grading area associated with prognosis can also be explained by such a high rate of induction. On the other hand, it also shows that with the development of examination assistive technology, more congenital heart diseases can be identified in the fetal period. It is noteworthy that the high detection rate is also a factor contributing to the increase of the death rate, although this needs further verification.\u003c/p\u003e \u003cp\u003eAlthough this is one of the largest cohorts of fetuses with EA, the sample size is still relatively small, which is therefore limited to provide a strong conclusion. Additionally this is a single-institutional retrospective study. Many patients were referred to us from other centers. As a result, there is potential patient selection bias which may have led to the data, and results presented here may not be directly applicable to other centers and populations.\u003c/p\u003e "},{"header":"5 Conclusions","content":" \u003cp\u003eEchocardiography has become an important tool in identifying the fetuses with EA, a rare and heterogeneous congenital heart disorder. The prognosis of EA patients during the fetal period is relatively poor. Therefore, understanding the echocardiographic characteristics and the prognosis of fetal EA is of great value for pregnancy consultations during the perinatal period.\u003c/p\u003e "},{"header":"Abbreviations","content":" \u003cdiv class=\"DefinitionList\"\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eEA\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eEbstein anomaly; TV:Tricuspid valve; RA:Right atria; LA:Left atria; aRV:Atrialized right ventricule; fRV:functional right ventricule; LV:Left ventricule.\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003c/div\u003e "},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eAcknowledgements \u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNot applicable.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAuthors\u0026rsquo; contributions \u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eYuduo Wu was responsible for data collection, data analysis, and manuscript preparation; Xiaoyan Gu was responsible for data interpretation; Yihua He was responsible for manuscript revision and finalization. Funding was secured by Hongjia Zhang.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eFounding\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThis study was supported by National Science Funding (No.81301241), Beijing Key Laboratory of Maternal-Fetal Medicine and Fetal Heart Disease (No. BZ0308), and Beijing Municipal Administration of Hospitals\u0026rsquo; Ascent Plan (No. DFL20180602).\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAvailability of data and materials \u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe datasets analyzed for the current study are available from the correspond‑ ing author on reasonable request.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eEthics approval and consent to participate \u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe human study were approved by the ethics committee of Beijing Anzhen Hospital. Written informed consent was obtained from all subjects.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConsent for publication\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003e\u0026nbsp;Not applicable.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCompeting interests\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe authors declare that they have no competing interests.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAuthor details\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003e\u003csup\u003e1\u003c/sup\u003eDepartment of Cardiac Surgery, Beijing Anzhen Hospital, Capital Medical University, Beijing, China;\u003c/p\u003e\n\u003cp\u003e\u003csup\u003e2\u003c/sup\u003eDepartment of Echocardiography, Beijing Key Laboratory of Maternal‐Fetal Medicine in Fetal Heart Disease, Beijing Anzhen Hospital, Capital Medical University, Beijing, China;\u003c/p\u003e\n\u003cp\u003eAddress for co-correspondence:\u003c/p\u003e\n\u003cp\u003eYihua He, Department of Echocardiography, Beijing Key Laboratory of Maternal‐fetal Medicine in Fetal Heart Disease, Beijing Anzhen Hospital, Capital Medical University, No.2, Anzhen Road, Chaoyang District, Beijing 100029, China. Email: [email protected]; fax number: +86 10 84006368, telephone：18910778673.\u003c/p\u003e\n\u003cp\u003eHongjia Zhang, Department of Cardiac Surgery, Beijing Anzhen Hospital, Capital Medical University, No.2, Anzhen Road, Chaoyang District, Beijing 100029, China. E-mail:[email protected]. fax numbers: +86 10 85231211, telephone：13311516256.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\u003cli\u003e \u003cspan\u003eI G, L G, R S, et al. Ebstein's Anomaly of the Tricuspid Valve in the Fetus- A Multicenter Experience.[J]. Ultraschall in der Medizin (Stuttgart, Germany: 1980), 2017,38(4):427\u0026ndash;436.\u003c/span\u003e \u003c/li\u003e \u003cli\u003e \u003cspan\u003eShetty RK, Vivek G, Nayak K, et al. Fetal Ebstein's anomaly[J]. BMJ Case Rep, 2012,2012.\u003c/span\u003e \u003c/li\u003e \u003cli\u003e \u003cspan\u003eLR F, MC E, BT K, et al. Outcomes and Predictors of Perinatal Mortality in Fetuses With Ebstein Anomaly or Tricuspid Valve Dysplasia in the Current Era: A Multicenter Study.[J]. Circulation. 2015;132(6):481\u0026ndash;9.\u003c/span\u003e \u003c/li\u003e \u003cli\u003e \u003cspan\u003eFreud LR, Escobar-Diaz MC, Kalish BT, et al. Outcomes and Predictors of Perinatal Mortality in Fetuses With Ebstein Anomaly or Tricuspid Valve Dysplasia in the Current Era: A Multicenter Study[J]. Circulation. 2015;132(6):481\u0026ndash;9.\u003c/span\u003e \u003c/li\u003e \u003cli\u003e \u003cspan\u003eGottschalk I, Gottschalk L, Stressig R, et al. Ebstein's Anomaly of the Tricuspid Valve in the Fetus - A Multicenter Experience[J]. Ultraschall Med. 2017;38(4):427\u0026ndash;36.\u003c/span\u003e \u003c/li\u003e \u003cli\u003e \u003cspan\u003eProta C, Di Salvo G, et al. \"Prognostic value of echocardiographic parameters in pediatric patients with Ebstein's anomaly.\". Int J Cardiol. 2019;278:76\u0026ndash;83.\u003c/span\u003e \u003c/li\u003e \u003cli\u003e \u003cspan\u003eBooker OJ, Nanda NC. Echocardiographic assessment of Ebstein'sanomaly[J]. Echocardiography, 2015,32Suppl 2:S177-S188.\u003c/span\u003e \u003c/li\u003e \u003cli\u003e \u003cspan\u003eGottschalk I, Gottschalk L, Stressig R, et al. Ebstein's Anomaly of the Tricuspid Valve in the Fetus-AMulticenter Experience[J]. Ultraschall Med. 2017;38(4):427\u0026ndash;36.\u003c/span\u003e \u003c/li\u003e \u003cli\u003e \u003cspan\u003eKaya B, Ekiz A, Acar DK, et al. Prenatal diagnosis of aortopulmonary window by foetal echocardiography: 'U or reversed U sign'[J]. J ObstetGynaecol. 2019;39(5):712\u0026ndash;3.\u003c/span\u003e \u003c/li\u003e \u003cli\u003e \u003cspan\u003eYu S, Han J, Gao S, et al. The prenatal diagnosis of aortopulmonary window by fetal echocardiography[J]. Echocardiography. 2018;35(11):1835\u0026ndash;40.\u003c/span\u003e \u003c/li\u003e \u003cli\u003e \u003cspan\u003eGarcia C, Alvarez T, Bravo C, et al. Prenatal Diagnosis of an Aortopulmonary Window With an Interrupted Aortic Arch[J]. J Ultrasound Med. 2016;35(10):2087\u0026ndash;93.\u003c/span\u003e \u003c/li\u003e \u003cli\u003e \u003cspan\u003ePornprasertchai V, Vijarnsorn C, Kanjanauthai S, et al. Contemporary outcomes and mortality risks of Ebstein anomaly: A single-center experience in Thailand[J]. Congenit Heart Dis, 2019.\u003c/span\u003e \u003c/li\u003e \u003cli\u003e \u003cspan\u003eZimmer EZ, Blazer S, Lorber A, et al. Fetal Ebstein's anomaly: early and late appearance[J]. PrenatDiagn. 2012;32(3):228\u0026ndash;33.\u003c/span\u003e \u003c/li\u003e \u003cli\u003e \u003cspan\u003eRS L, LG A, MA A, et al. Diagnosis and prognosis of fetal ebstein anomaly.[J]. Ginecologia y obstetricia de Mexico. 2014;82(2):93\u0026ndash;104.\u003c/span\u003e \u003c/li\u003e \u003cli\u003e \u003cspan\u003eSelamet TE, McElhinney DB, Freud LR, et al. Assessment of Progressive Pathophysiology After Early Prenatal Diagnosis of the Ebstein Anomaly or Tricuspid Valve Dysplasia[J]. Am J Cardiol. 2017;119(1):106\u0026ndash;11.\u003c/span\u003e \u003c/li\u003e\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":true,"highlight":"","institution":"","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true},"keywords":"Ebstein anomaly, fetal, echocardiography, prognosis","lastPublishedDoi":"10.21203/rs.3.rs-69914/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-69914/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003e\u003cstrong\u003eObjective: \u003c/strong\u003eThe aim of this study was to describe the fetal echocardiographic features of Ebstein anomaly (EA) and also to evaluate the prognosis of those fetuses.\u003c/p\u003e\u003cp\u003e\u003cstrong\u003eMethods: \u003c/strong\u003eWe 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. \u003c/p\u003e\u003cp\u003e\u003cstrong\u003eResults: \u003c/strong\u003eIn 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. \u003c/p\u003e\u003cp\u003e\u003cstrong\u003eConclusion: \u003c/strong\u003eEchocardiography 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.\u003c/p\u003e","manuscriptTitle":"Echocardiographic Characteristics and Prognosis of the Fetuses with Ebstein Anomaly in Pregnancy","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2020-09-04 21:51:21","doi":"10.21203/rs.3.rs-69914/v1","editorialEvents":[{"type":"communityComments","content":0}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"dd138107-c3af-47b8-877b-8c522645d6cb","owner":[],"postedDate":"September 4th, 2020","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[{"id":434759,"name":"Maternal \u0026 Fetal Medicine"}],"tags":[],"updatedAt":"2020-10-16T15:04:38+00:00","versionOfRecord":[],"versionCreatedAt":"2020-09-04 21:51:21","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-69914","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-69914","identity":"rs-69914","version":["v1"]},"buildId":"wLkW0s4AflPzk-lpfg-fK","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}