Comparison of the offset distance of the tricuspid septal leaflet in neonates with Ebstein’s anomaly and neonates with pulmonary atresia with intact ventricular septum

Abstract

An indexed offset distance of the tricuspid septal leaflet ⩾8 mm/m2 is a quantitative criterion for the diagnosis of Ebstein's anomaly. The purpose of this study was to investigate the validity of this criterion for the discrimination of Ebstein's anomaly from pulmonary atresia with intact ventricular septum in neonatal patients. A total of 122 neonatal patients, 56 with Ebstein's anomaly and 66 with pulmonary atresia with intact ventricular septum, were enrolled. Diagnosis of each anomaly was based on typical morphologic features. Echocardiographic variables, including the offset distance of the tricuspid septal leaflet, were measured via an offline analysis of images recorded before 1 month of age. The offset distance of the tricuspid septal leaflet was indexed by the body surface area, and the indexed offset distances in the Ebstein's anomaly and pulmonary atresia with intact ventricular septum groups were 34.2 mm/m2 (7.1-119.1 mm/m2) and 7.2 mm/m2 (0.0-25.6 mm/m2), respectively. The indexed offset distance was ⩾8 mm/m2 in 29 (43.9%) of the patients with pulmonary atresia with intact ventricular septum; clinical and echocardiographic characteristics were comparable between these 29 patients and the remaining 37 patients with pulmonary atresia with intact ventricular septum. When an indexed offset distance ⩾8 mm/m2 was applied as a cut-off for the diagnosis of Ebstein's anomaly, the sensitivity was 0.963 and the specificity was 0.561. In conclusion, indexed offset distance ⩾8 mm/m2 cannot be used as a cut-off for the diagnosis of complicated Ebstein's anomaly in neonatal patients with pulmonary atresia with intact ventricular septum.

A ‘holistic’ sonographic view on congenital heart disease – How automatic reconstruction using fetal intelligent navigation echocardiography (FINE) eases the unveiling of abnormal cardiac anatomy part I: Right heart anomalies

“…34 The value of the offset distance of the tricuspid septal leaflet compared to that of the mitral valve leaflet for estimation the severity of the disease has been questioned in the past. 35,36 Accordingly, this further highlights the need for an attitudinally appropriate assessment of the deformed and displaced valve. In EA, the aforementioned inlet component of the RV is functionally integrated into the right atrium (atrialized) and may become disproportionately dilated and dyskinetic, whereas the trabecular and outlet portions are situated downstream from the displaced orifice and show preserved contractility.…”

The Congenital Tricuspid Valve Spectrum: From Ebstein to Dysplasia

“…8,15 Other studies have already called into question the utility of this specific septal leaflet hinge point displacement measurement in the accurate diagnosis of Ebstein anomaly. 16,17…”

A ‘holistic’ sonographic view on congenital heart disease – how semiautomatic reconstruction eases the unveiling of abnormal cardiac anatomy part I: right heart anomalies

“…Morphologically, in contrast to isolated dysplasia, the apical displacement of the hinge points of the tricuspid valve in EA is towards the outflow tract with rotation of the valve apparatus along the axis of the aorta, constituting an "all-or-none" criterion for the diagnosis of EA 34 . The value of the offset distance of the tricuspid septal leaflet compared to that of the mitral valve leaflet for estimation the severity of the disease has been questioned in the past 35,36 . Accordingly, this further highlights the need for an attitudinally appropriate assessment of the deformed and displaced valve.…”