Open Access Maced J Med Sci. 2021 Oct 04; 9(C):189-192. 189 Scientifc Foundation SPIROSKI, Skopje, Republic of Macedonia Open Access Macedonian Journal of Medical Sciences. 2021 Oct 04; 9(C):189-192. https://doi.org/10.3889/oamjms.2021.6012 eISSN: 1857-9655 Category: C - Case Reports Section: Case Report in Pediatrics Prenatal Diagnosis of Ebstein Anomaly in a Centre with Limited Resources: A Potential Pitfall Ramush Bejiqi 1,2,3 , Ragip Retkoceri 3 , Hana Bejiqi 4 , Aferdita Mustafa 3 , Arlinda Maloku 4 , Rinor Bejiqi 3 * 1 Texas Health Science Center, San Antonio, Texas, USA; 2 Medical School, University of Gjakova “Fehmi Agani”, Gjakova, Kosovo; 3 University Clinical Center of Kosovo, Prishtina, Kosovo; 4 Main Center of Family Medicine, Prishtina, Kosovo Abstract BACKGROUND: Ebstein anomaly is a rare congenital cardiac malformation characterized by varying degrees of downward displacement of the tricuspid valve leafets into the cavity of the right ventricle. The pathophysiology varies with the degree of valvular malfunction, the size, and contractility of the right ventricle, and the presence of obstruction within the right ventricular outfow tract. An early diagnosis makes worse the often prognosis. Fetal echocardiography is a useful tool for the diagnosis of congenital heart disease including the Ebstein anomaly which shows a wide spectrum of ultrasonographic manifestations and, sometimes it can be difcult to make a true prenatal diagnosis. CASE REPORT: Here, we describe a unique case of an Ebstein anomaly in 26 weeks of gestation fetus through prenatal echocardiographic evaluation. Fetus was suspected as a severe form of the Ebstein anomaly, with a severe form of right atrium enlargement, huge tricuspid insufciency, and hypoplastic pulmonary artery. The atrial septum, dimensions of cardiac structures, left and right cardiac output, and Doppler interrogation of cardiac fows were examined. CONCLUSION: This case study presents a well-documented case of Ebstein’s anomaly type D that was diagnosed prenatally using fetal echocardiography. Edited by: Igor Spiroski Citation: Bejiqi R, Retkoceri R, Bejiqi H, Mustafa A, Maloku A, Bejiqi R. Prenatal Diagnosis of Ebstein Anomaly in a Centre with Limited Resources: A Potential Pitfall. Open Access Maced J Med Sci. 2021 Oct 04; 9(C):189-192. https://doi.org/10.3889/oamjms.2021.6012 Keywords: Fetal echocardiography; Ebstein anomaly; Fetal heart; Tricuspid insufciency; Fetal hidrops *Correspondence: Rinor Bejiqi, University Clinical Center of Kosovo, Prishtina, Kosovo. E-mail: rinorbejiqi25@gmail.com Received: 22-Mar-2021 Revised: 18-Apr-2021 Accepted: 24-Sep-2021 Copyright: © 2021 Ramush Bejiqi, Ragip Retkoceri, Hana Bejiqi, Aferdita Mustafa, Arlinda Maloku, Rinor Bejiqi Funding: This research did not receive any fnancial support Competing Interests: The authors have declared that no competing interests exist Open Access: This is an open-access article distributed under the terms of the Creative Commons Attribution- NonCommercial 4.0 International License (CC BY-NC 4.0) Introduction Ebstein anomaly is an uncommon congenital abnormality with a prevalence of 0.2–0.5% of all cardiac malformations. It can appear at any age, from fetal life to adulthood, with a highly variable clinical course. In the fetus and neonate, a targeted echocardiogram has been proven to be reliable in diferentiating the variants of Ebstein anomaly [1]. However, because targeted fetal echocardiography cannot be used routinely for all obstetric patients, the ability to recognize Ebstein anomaly from a routine prenatal obstetric sonographic examination is important. The 4-chamber view of the heart, a component of the American Institute of Ultrasound in Medicine guidelines for fetal structural evaluations, is most helpful for the detection of many congenital heart defects, including Ebstein anomaly. The 4-chamber view, however, does have limitations in the diagnosis of certain cardiac malformations [2]. We here present a rare case of a fetus with severe form of Ebstein anomaly, diagnosed by fetal echocardiography after an initial routine obstetric sonogram, and discuss the diagnostic considerations in such patients. Case Report A 26-year-old gravida 1, para 0 woman was referred to our tertiary center at 20.4 weeks of gestation with a suspected cardiac anomaly. Her previous medical history was unremarkable, and she had no family history of congenital malformation. She had no known history of teratogen exposure at any time during pregnancy. The fetus had appropriate biometrical dimensions and appeared morphologically normal. Quad tests and other prenatal laboratory fndings were within normal ranges. A fetal echocardiography was performed and the four-chamber view of the heart revealed cardiomegaly with a right heart side dilatation and without defnite contractility. There were evidence of discernible tricuspid leafets (Figure 1a-c), indicating the downward displacement of a tricuspid valve structure. Color Doppler imaging showed forward and backward fows from the right atrium to the right ventricle with evidence of huge tricuspid regurgitation (Figures 1d and e and 2). Because the tricuspid valve structure was completely incompetent and also because there were communicating fows between the right atrium and right ventricle, the fetus was thought to have