Key result
4D flow CMR quantifies severe tricuspid regurgitation in Ebstein anomaly, showing ~82% regurgitant fraction.
Why the study?
Direct quantification of tricuspid regurgitation in patients with Ebstein’s anomaly using 4D flow cardiovascular magnetic resonance has not been demonstrated.
Case Report (n=1)
Answers are grounded in this paper and cite their sources.
4D flow CMR is feasible in Ebstein’s anomaly; hypothesis-generating for congenital valve assessment pending validation studies.
A patient with Ebstein’s anomaly, a rare congenital heart disease in which the septal and posterior leaflets of the tricuspid valve are located more apically (Panel A, stylized representation of the tricuspid valve leaflets with the septal leaflet indicated by an arrow, RV: right ventricle, LV: left ventricle), in sinus rhythm, underwent cardiovascular magnetic resonance (CMR) imaging including state-of-the-art whole-heart 4D flow CMR. Validated 4D flow CMR with retrospective valve tracking was used to directly quantify tricuspid regurgitation (TR). Streamline visualization was used to visualize flow patterns and the dynamic regurgitation jet. A measurement plane was placed on the level of maximum velocity in every timeframe and adjusted perpendicular to the flow direction (Panel B, see Supplementary data online, Video S1). The resulting flow curve (Panel C) shows a regurgitation fraction of 82%, illustrated by the ratio between the negative versus positive area-under-the-curve. Retrospective valve tracking was done over all valves in a single acquisition (acquisition time 8 min, post-processing time 20 min). The small differences between the net flow volumes through the four valves of maximally 3 mL indicate conservation of mass (Panel D). This proof-of-principle case demonstrates the feasibility of direct TR-quantification by 4D flow CMR with retrospective valve tracking in a patient with Ebstein’s anomaly and can be a starting point for prospective evaluation of clinical utility of direct TR-quantification in Ebstein patients with various degrees of TR. 4D Flow CMR-derived direct quantification of TR may prove to be helpful in the follow-up of patients with Ebstein’s anomaly and may have implications for the timing of an intervention. Supplementary data are available at European Heart Journal – Cardiovascular Imaging online. Funding V.P.K. is financially supported by a grant from the Dutch Heart Foundation (Grant Number 2013T091). J.J.M.W. is financially supported by a grant of ZonMw (project number 104003001).
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Kamphuis et al. (2018) conducted a case report in Ebstein's anomaly (n=1). 4D flow CMR with retrospective valve tracking was evaluated on Regurgitation fraction. 4D flow CMR with retrospective valve tracking successfully quantified tricuspid regurgitation in a patient with Ebstein's anomaly, demonstrating a regurgitation fraction of 82%.
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