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 2017-11-18  Case of the week # 461  © Lusine Karapetyan
Case of the week # 461
January 4, 2018 - January 18, 2018

Lusine Karapetyan, MD

Case report

Following  images and videos show a case of tricuspid atresia associated with ventricular septal defect(VSD) and complete transposition of the great arteries(TGA) detected at 15 and 21 weeks of otherwise non-complicated pregnancy.


Tricuspid atresia (TA) is a rare form of congenital heart disease. The Baltimore-Washington Infant Study showed a prevalence at live birth of 1/25 000(1). In a prenatal screening cohort Tegnander et al. reported a comparable low incidence of 1/15 000 or 0.47% of all cardiac defects detected(2). 


TA is defined as an absent or, rarely, an imperforate right atrioventricular connection(3). In affected cases, gray-scale imaging in the four-chamber view demonstrates no patent tricuspid valve, a small right ventricle and, in most cases, a ventricular septal defect . Color flow mapping demonstrates no flow from right atrium to right ventricle and a marked right to left shunt at the atrial septum.   The vessel arising from the small right ventricle tends to be hypoplastic. Therefore, pulmonary stenosis or atresia are frequent findings in concordant ventriculoarterial connection, especially when the ventricular septal defect is small.   In ventriculoarterial discordance( which demonstrated in our case), which occurs in a significant proportion of TA cases, hypoplasia of the aortic arch   or even aortic atresia may occur, depending on the size of the ventricular septal defect. Less frequently associated cardiac defects include double outlet ventricle, anomalous pulmonary venous return and common arterial trunk .

Images 1 and 2, videos 1-3 (15 weeks): show  atretic and echogenic tricuspid valve , no flow through the tricuspid valve, hypoplastic aorta arising from the hypoplastic right ventricle , pulmonary artery arising from the left ventricle and VSD.


Images 3-6, videos 4-8 (21 weeks): 
2D and color Doppler images show VSD connecting right and left ventricles, parallel course of the great arteries, pulmonary artery arising from the left ventricle, aorta arising from the hypoplastic right ventricle and no flow through the tricuspid valve. 




1Ferencz CRubin JDMcCarter RJBrenner JINeill CAPerry LWHepner SIDowning JWCongenital heart disease: prevalence at livebirth. The Baltimore-Washington Infant StudyAm J Epidemiol 19851213136.

2Tegnander E
Williams WJohansen OJBlaas HGEik-Nes SHPrenatal detection of heart defects in a non-selected population of 30149 fetuses: detection rates and outcomeUltrasound Obstet Gynecol 200627252265.

3Freedom RM
Yoo SJTricuspid atresia. In The Natural and Modified History of Congenital Heart DiseaseFreedomRMYooSJMikailianHWilliamsWG (eds). Blackwell Publishing: New York2004381386.

4C.Berg,R.Lachmann,C.Kaiser,P.Kozlowski,R.Stressig,M.Schneider,B.Asfour,U.Herberg,J.Breuer,U.Gembruch,A.Geipel Prenatal diagnosis of tricuspid atresia: Intrauterine course and outcome 2010 Ultrasound in obstetrics and gynecology
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