Unguarded tricuspid orifice‐‐‐a rare cause of fetal right atrial dilatation with characteristic color doppler sign: Case report with review of literature

Abstract

The anatomic causes for fetal right atrial dilatation with tricuspid regurgitation include Ebstein anomaly, tricuspid dysplasia, unguarded tricuspid orifice, and Uhl anomaly. Unguarded tricuspid orifice is characterized by complete or partial agenesis of the tricuspid valvular and subvalvular structures. It is commonly associated with pulmonary atresia. Its prenatal diagnosis is usually associated with unfavorable prognosis. We present a prenatally diagnosed case of fetal unguarded tricuspid orifice with description of its diagnostic workup, along with a review of literature, to enhance the understanding of this rarely reported entity. © 2016 Wiley Periodicals, Inc. J Clin Ultrasound 45:370-374, 2017.

Fetal Unguarded Mitral Valve Orifice, Aortic Atresia, and Severe Left Heart Enlargement

“…The finding of AA has been reported in all cases of unguarded MV in the setting of normal segmental cardiac anatomy ( 2 ). AA in the setting of an unguarded MV mirrors that of pulmonary atresia, which has been frequently reported in the setting of fetal unguarded tricuspid valve ( 9 ). We postulate that severe regurgitation of an unguarded AV valve results in ipsilateral arterial valvar atresia caused by the inefficient, and eventually absent, antegrade flow across the respective arterial valve.…”

Korumasız Triküspid Kapak Orifisinin İki Yeni Olgusu

“…Vikraman ve ark. sunduğu olguda 26 yaşında gravida 2 annenin, 2. trimesterde antenatal dönemde ultrasonografik incelemede rastlantısal fark edilen fetal sağ atriyumda genişleme görülmüş ve ileri inceleme için bir üst merkeze sevk edilen hastanın yapılan fetal ekokardiyografi ve renkli doppler incelemesinde korumasız triküspid kapak tanısı konulmuştur (14). Multidisipliner ekip yaklaşımı prenatal tanı ve postnatal hasta takip ve tedavisinde başarının artmasına önemli katkı sağlayacaktır.…”

Prenatal sonographic findings of prominent fetal tricuspid annulus: A case report

“…Dilatation and flattening of the tricuspid annulus compromises tricuspid valve coaptation, and may be associated with tricuspid regurgitation [ 4 , 5 ]. Overall, congenital structural anomalies of the fetal tricuspid valve are rare, and include Ebstein's anomaly, tricuspid atresia, partial absence, unguarded tricuspid orifice (absent leaflets) cleft, double orifice, bicuspid valve, and Uhl anomaly (absence of the right ventricular myocardium with an apposing endocardium and epicardium) [6] , [7] , [8] , [9] , [10] , [11] , [12] , [13] , [14] , [15] . We present an unusual case in which a prominent circular structure was noted in immediate proximity to, and slightly above the periphery of the fetal tricuspid valve at 31 weeks’ gestation.…”