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.
