Objectives of the study: to analyse retrospectively the characteristics and outcome of cases with tricuspid valve anomaly diagnosed at fetal echocardiography.
Material: Sixteen fetuses with Ebstein (Ebst) and 16 with non Ebstein anomaly (NEbst) of the tricuspid valve (TV), one with associated mitral dysplasia) diagnosed at 21-36 (median 30) week’s gestation (wg), between 1986-2008, were followed up in utero and postnatally for 6 months-21 years, median 5 years.
Results: Echocardiographic features: Moderate-severe tricuspid regurgitation (TR) was present in 11/16 Ebst fetuses (with severe displacement of TV) and in 10/16 NEbst; 4 cases had associated pulmonary stenosis and 4 functional-organic pulmonary atresia. Six fetuses (4 Ebst, 2Nebst) had fetal hydrops (FH). Outcome: Two cases opted for termination of pregnancy, 3 Ebst died in utero at 31 wg, (2 with FH, 1 in supraventricular tachycardia). Five cases died spontanouesly at 1-7 days. Three infants Ebst and 3 NEbst were operated, 2 surviving; the case with associated mitral dyplasia improved the TR but required a mitral valve plasty and is alive at 8 yrs. One Ebst died at 3 years due to arrhythmia and severe worsening of TR. Total mortality was 13/29 ( 44.8%) cases that continued pregnancy, 4/6 with FH,. 9/16 Ebst (56%), 4 /16 NEbst 25%. Sixteen cases are alive at 6m-21 years, stable or improved.
Conclusions: Our data confirm a variable spectrum of tricuspid valve anomalies presenting in utero. Negative prognostic factors for outcome were heart failure and arrhythmias, milder forms of both variants stabilized after birth.