 Good evening everyone, I am Dr. Zeyden Amdar and today I will be presenting a case report on the prenatal diagnosis of Epstein's Anomaly at 2nd trimester ultrasound scan. To begin with Epstein's Anomaly is a rare complex cyanotic congenital heart disorder which primarily involves the tricuspid valve apparatus and the right side of the heart. The pathophysiology implicated here is the failure of delamination of tricuspid valve leaflets which gives an appearance of apical displacement of the valve along with a small functional right ventricle. Ecocardiography is the investigation of choice for diagnosis, however with careful sonographic evaluation detection of this anomaly during routine, 2nd trimester ultrasound screening is also possible. The affected neonates will present at birth with cyanosis and congestive cardiac failure which may prove to be rapidly fatal without prompt surgical intervention. The aim of this case report is to emphasise the importance of screening for cardiac anomalies during routine obstetric ultrasound in the 2nd trimester and to demonstrate USG findings in fetus with Epstein's Anomaly to facilitate the antinatal diagnosis of the same. I present a case of a 31-year-old primigravida with 23 weeks of gestation who presented to our OPD for routine 2nd trimester obstetric ultrasound scan also known as the level 2 anomaly scan. Patient was vitally stable at the time of examination. A 2D ultrasound examination was performed using a 3.5 MHz transabdominal curvilinear probe on the Philips Clearview 550 ultrasound equipment at our institution. The ultrasound showed a single live intrauterine fetus of gestational age 23 weeks and 2 days with adequate liquor, however the cardiac screening ultrasound revealed the following malformations. Cardiomegaly with an enlarged right atrium, apical displacement of the tricuspid valve, decreased volume of the functional right ventricle with dilated and thinned-out part of right ventricle lying above the annulus which is also known as the itrialization of the right ventricle and tricuspid regurgitation was detected on color Doppler. Based on the above findings, a provisional diagnosis of fetal Epstein's Anomaly was made which was further confirmed by targeted fetal echocardiography. Now I would like to show some ultrasound images to enhance our understanding of the topic. Coming to figure 1 and 2, this is the 4 chamber view of the heart which shows a grossly dilated right atrium as we can see in these both figures. The figure 3 and 4 shows a magnified view of the 4 chambered heart and here the mitral valve as well as the tricuspid valve has been denoted with the help of arrows. Note the apical displacement of the tricuspid valve in relation to the mitral valve and the dilated thinned-out part of the right ventricle which lies above the annulus and is known as the itrialization of right ventricle which is characteristic of the Epstein's Anomaly. In this figure, we see color Doppler image which shows aliasing of color at the tricuspid valve region and denotes tricuspid regurgitation. Coming to the case discussion, Epstein's Anomaly is a cyanotic congenital cardiac anomaly which was first described by Wilhelm Epstein as a rare type of insufficiency of the tricuspid valve. It has a reported incidence of 1 in 20,000 live births and accounts for less than 1% of all congenital cardiac anomalies. The primary pathology here involves the abnormal attachment of tricuspid valve leaflets. Incomplete delamination of the proximal portion causes tethering of the valve leaflets to the right ventricular free valve and interventricular septum. This gives rise to characteristic morphological features in the anomaly like right atrial enlargement, apical displacement of the tricuspid valve, functionally small right ventricle. The thinned out and dilated portion of the right ventricle seen above the tricuspid valve annulus is known as the atrialization of the right ventricle. Due to tethering and redundancy of the valve leaflets, there is dilatation of the tricuspid valve annulus which gives rise to tricuspid regurgitation. The Epstein anomaly is a spectrum of malformations which may range from minimal apical displacement of the valve leaflet to a muscular shelf between the inlet and trapecular zone of the right ventricle. This depends on where the process of delamination has been arrested. Carpentier's classification is widely used in Epstein's anomaly. It is based on the volume of functional right ventricle and degree of anterior leaflet motion of the tricuspid valve. The disease has been divided into 4 types from A to D in increasing order of severity. The image in the left is a pictorial depiction of the failure of delamination which is the pathophysiology behind the Epstein's anomaly. The valve leaflets are derived from the delamination of inner portion of the right ventricle and failure of this process causes the Epstein's anomaly. Therefore, it is a spectrum which might have infinite variability. To the right side, we see Carpentier's classification. In type A, the volume of true right ventricle is adequate and as we go downwards to type D, almost near complete atrialization of the right ventricle is seen. Coming to the etiology, most cases of the Epstein's anomaly are sporadic. However, certain genetic and environmental factors have been implicated in its causation. New studies have revealed the MYH7 gene as well as cardiac transcription factor NKX2.5 mutations may be a causative factor in the Epstein's anomaly. The environmental factors include maternal benzodiazepine use and lithium ingestion in the first trimester, which is used in profile axis and treatment of bipolar disorders. Epstein's anomaly may be associated with other cardiac malformations, which most commonly includes the atrial septal defect or persistent foramine ovale. Others may include ventricular septal defect, right ventricular outflow tract obstruction, pulmonary stenosis and atrizia. Fetal echocardiography is the investigation of choice for antenatal detection of Epstein's anomaly. The four-chambered view of the heart serves as an important tool for diagnosis. Tricuspid regurgitation can be demonstrated using color Doppler and spectral waveforms. The presence of cyanosis, severe tricuspid regurgitation and right-sided heart failure in units necessitates surgical intervention. Most units with symptomatic Epstein's anomaly at birth cannot survive for long without surgical treatment. The surgical treatment includes tricuspid valve replacement or repair and may also involve correction of the associated malformations like surgical rejection of the atrialized right ventricle and thinned-out right atrium. Complete repair of the anomaly correlates with a good survival outcome in the symptomatic units. Thus, prenatal USG allows for appropriate management planning of delivery at a tertiary hospital with adequate facilities for early neonatal intervention, which overall increases the survival chances. To conclude, Epstein's anomaly is a rare type of cyanotic congenital heart disease and is characterized by malformation of the tricuspid valve and right side of the heart. Echocardiography is the investigation of choice for diagnosis of this condition. However, it can also be detected with careful sonographic evaluation during the routine second trimester ultrasound scan. The apical displacement of the tricuspid valve, right atrial enlargement, functionally small right ventricle and associated atrialization of the right ventricle are key features for its diagnosis. Symptomatic units present at birth with rapidly worsening features like cyanosis and right heart failure and mortality is imminent without prompt surgical treatment. This underlines the importance of early diagnosis of this anomaly in prenatal USG in order to facilitate the antinatal and perinatal management of the affected babies and intervention in early neonatal period and this improves the overall prognosis. That's all from my side for today. Thank you.