Ultrasonic diagnosis of fetal congenitally unguarded tricuspid valve orifice: analysis of two cases and literature review
10.3760/cma.j.cn113903-20230312-00127
- VernacularTitle:超声诊断胎儿三尖瓣口无挡畸形2例及文献复习
- Author:
Hailan LIU
1
;
Yan SONG
Author Information
1. 南昌市第一医院超声科，南昌 330006
- Keywords:
fetal congenitally unguarded tricuspid valve orifice;
Prenatal;
echocardiography
- From:
Chinese Journal of Perinatal Medicine
2023;26(9):741-745
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To review the imaging characteristics and evaluate the diagnostic value of echocardiography for fetal congenitally unguarded tricuspid valve orifice (CUTVO).Methods:Clinical data and ultrasonic images of two pregnant women diagnosed with fetal CUTVO through ultrasound and confirmed by pathology in Nanchang First Hospital between April 2019 and December 2021 were retrospectively analyzed. Relevant literature on confirmed CUTVO diagnosis cases with ultrasonic diagnosis results was searched in the Wanfang database, CNKI database, VIP database, Chinese Medical Journal database, PubMed, and Web of Science database. Descriptive statistical analysis was used to analyze the data.Results:The study involved 17 patients, 15 of whom were retrieved from the databases and two cases from our hospital. Among the 17 cases, 16 were singleton pregnancies, while one case involved a monochorionic monoamniotic twin pregnancy. The tricuspid valve was completely absent or hypoplastic in ten cases, and partial absence of the tricuspid leaflet was found in seven cases. Nine cases had pulmonary atresia with intact ventricular septum and two with ventricular septal defect, while complications were not described in the other six cases. Among the six misdiagnosed cases, four were prenatally misdiagnosed as fetal Ebstein malformation, two cases for Uhl's disease and suspected noncompaction of the ventricular myocardium. CUVTO were confirmed after pregnancy termination. Both cases of missed diagnosis were in the second trimester with no significant enlargement in the right heart, including one with twin transfusion syndrome and one with pulmonary valve stenosis. Nine cases terminated their pregnancies and seven of them had confirmed CUTVO by autopsy after an induced labor. Eight fetuses were delivered. Except for two deaths due to severe conditions, six neonates survived and underwent surgical treatment due to atrial septal defect and patent ductus arteriosus on postnatal echocardiography examination. Among them, two cases underwent tricuspid valve plasty and the tricuspid valve regurgitated moderately during follow-up within one year after surgery; two underwent tricuspid valve replacement with mechanical valves and postoperative less moderate regurgitation was also found; modified Fontan surgery was conducted in one case and the cardiac function turned normal; in one case, no surgical modality was described. Typical echocardiographic features of CUTVO were as follows: normal atrioventricular structure and the connection to arteries; total or partial absence of tricuspid leaflets and papillary muscles; the annulus of the tricuspid valve can be described as "empty" in the apical four-chamber view; Doppler evaluation shows to-and-fro flow across the tricuspid orifice with low velocity and a two-way spectrum.Conclusion:Typical echocardiography features of CUTVO were absence of tricuspid leaflets and moderate to massive regurgitation with a two-way spectrum. It is essential to make efforts to minimize missed and misdiagnoses through dynamic observation of multiple sections.
