Change in Right Ventricular Strain after Cone Reconstruction of Ebstein's Anomaly: A CMR Feature Tracking Study

Background: Ebstein's anomaly (EA) is a rare congenital heart disease defined by apical displacement of the tricuspid valve. It is, however, deemed a disorder of development involving the entire right ventricular myocardium. The latest surgical approach for EA has been Da Silva's cone repair. Identifying a parameter that truly reflects myocardial function before and after cone repair remains challenging. Cardiovascular magnetic resonance feature tracking (CMR-FT) is a relatively new method to quantify myocardial contraction, which has demonstrated diagnostic and prognostic value in many diseases. We therefore aimed to assess the value of CMR-FT of the right ventricle (RV) in pre- and postoperative management of EA patients undergoing cone repair.

Method: Nine EA patients submitted to cone repair with a complete CMR evaluation before and after surgery were selected retrospectively. CMR-FT analysis was performed in each patient pre- and postoperatively by manually tracing the endo- and epicardial borders of the functional RV on steady-state free precession cine images. Global radial (GRS) and global circumferential strain (GCS) were obtained from short-axis cine images in basal, mid and apical planes. Global longitudinal strain (GLS) was derived from a four-chamber cine image.

Results: We evaluated 9 patients (56% female), median age of 26 years (range: 11–40 years) at first CMR. Median postoperative CMR scans were performed at median 12 months (range: 10–18 months) after Cone-repair. RV ejection fraction decreased from median 55% (range: 28−61%) to 36% (range: 22−42%; p = 0.0097) after repair. GLS deteriorated in all but one patient, without reaching a significant result from −19.95% (range: −30.01 (−12.8) %) to −15.20% (range −18.39 (−8.6) %; p = 0.0907). Median GRS and GCS did not differ (15.43%, range: 8.49–24.37% vs. 17.83%, range: 9.28–23.73%; p = 0.4225 and −9.97%, range: −14.99 (−4.04) % vs. −12.41%, range −15.74 (−7.62) %; p = 0.2864), with a slight trend toward improvement.

Conclusion: RV-FT parameters did not show any remarkable impact after cone repair. Slight impaired GLS might indicate, similar to RVEF, dependence on different loading conditions and reduced afterload due to tricuspid regurgitation that might lead to “false” normal GLS preoperatively. GRS and GCS did not change, indicating that short-axis strain might be less confounded by altered load dependent hemodynamics.

Publication History

Article published online:
28 January 2023