ABSTRACT
Objective:To improve the prenatal recognition and diagnostic accuracy of isolated tricuspid valve dysplasia (TVD) by analyzing fetal echocardiographic features.Methods:The fetal echocardiographic features of 7 cases with prenatal diagnosis of TVD were analyzed retrospectively in Pediatric Cardiovascular Center, Beijing Anzhen Hospital, Capital Medical University and Children′s Hospital of Shanxi from June 2012 to December 2020. The fetal echocardiographic characteristics of TVD were summarized.Results:Among the 7 fetuses with TVD, 6 cases showed functional pulmonary atresia with intact ventricular septum and different degrees of pulmonary valve regurgitation, and 1 case showed slightly narrowed pulmonary artery. Characteristic sonographical findings included: ①The axis of heart was exaggerated left-axis deviation and the ratio of cardiothoracic was obviously increased. The right heart was enlarged, in which the right atrium was significantly dilated (an important feature of TVD). ②The position of tricuspid valve ring was normal, the attachment point of each leaf was normal, some tricuspid valves were thickened and insufficiency. ③It was common to be complicated with functional pulmonary valve atresia with intact ventricular septum or severe pulmonary valve stenosis. ④Color Doppler imaging showed severe tricuspid regurgitation and the regurgitation velocity was less than 350 cm/s (an important feature of TVD). ⑤In fetus with functional pulmonary valve atresia with intact ventricular septum, color Doppler imaging showed retrograded ductal flow across the pulmonary artery and the different degrees regurgitation of pulmonary valve.Conclusions:Fetal TVD has typical echocardiographic features, mainly including large tricuspid regurgitation and significant enlargement of the right atrium. The prognosis of fetus with functional pulmonary atresia is poor. Early diagnosis plays a crucial role in prenatal consultation and prognosis evaluation.
ABSTRACT
<p><b>Background</b>The incidence of Ebstein's anomaly is extremely low, and except for the Mayo Clinic, no cardiac center has reported on a sufficient number of patients. The aim of our study was to report the outcomes of Ebstein's anomaly patients treated with tricuspid valvuloplasty (TVP) or tricuspid valve replacement (TVR).</p><p><b>Methods</b>TVP or TVR was performed in 245 patients from July 2006 to April 2016. We reviewed patients' records and contacted patients via outpatient service and over the telephone.</p><p><b>Results</b>The mean follow-up time was 43.6 ± 32.6 months, and 224 (91.4%) patients underwent follow-up. The mean operative age was 31.2 ± 15.7 years. TVR was performed in 23 patients, and TVP was performed in 201 patients. The 30-day mortality rate was 1.3%, and the overall survival rate was 97.9% at 5 and 10 years. The early mortality rate of the TVP group was lower than that of the TVR group (0.5% vs. 8.7%, P = 0.028), and the overall mortality rate of the TVP group was lower than that of the TVR group, without statistical significance (1.0% vs. 8.7%). After propensity score matching, the rates of mortality and New York Heart Association class ≥ III were lower in the TVP group than those in the TVR group without statistical significance. Seven patients with Type B Wolff-Parkinson-White (WPW) syndrome underwent one-stage surgery, and arrhythmias disappeared. Six patients suffered from episodes of left ventricular outflow tract obstruction (LVOTO) during surgery. Severe LVOTO could be treated with reoperation of the atrialized right ventricle.</p><p><b>Conclusions</b>Ebstein's anomaly patients treated with TVP or TVR can experience optimal outcomes with midterm follow-up. However, TVP should be the first-choice treatment. Optimal outcomes can be obtained from one-stage operation in patients with Type B WPW syndrome. Severe LVOTO during surgery might be related to improper operation of the atrialized right ventricle.</p>