A Case of Persistent Right Ventricular Dysfunction Post-Cone Repair (QF_TH_060)

Description of Clinical Presentation: Background
Ebstein's anomaly of the tricuspid valve occurs due to failure of delamination of the tricuspid valve, resulting in downward displacement of the tricuspid valve and atrialized right ventricle. Surgical techniques and decision-making are complex and vary depending on ventricular function, tricuspid valve regurgitation, and the potential need for 1.5 ventricle repair or transplantation(Yuan, 2017).

Case
A 16-year-old girl with Ebstein's anomaly of the tricuspid valve underwent cone repair for severe tricuspid regurgitation and effort intolerance at 6 years of age.
In 2021, an MRI revealed a reduced proper ventricular ejection fraction (RVEF) of 30%. Given the uncertainty of the benefit of CP shunt post-cone repair, we decided to continue to observe her and refer them for a cardiac transplant if her condition worsens.

She remained stable on bisoprolol and lisinopril for heart failure management. In a recent exercise test, her resting saturation dropped from 95% to 80%. Although she participates in PE in school, her effort tolerance remains limited.

Diagnostic Techniques and Their Most Important Findings: Her repeat pre-transition MRI  (2024)showed a moderately dilated RV (iEDV 121 ml/m2) with reduced systolic function (EF 32 %) and normal LV systolic function (EF 64 %). A comparison with data from 2021 showed no change in RV volume and function. RV segmentation validated with the short axis and transaxial stack demonstrated similar RV volumes and functions.

Learning Points from this Case:  
This case highlights the challenges of managing Ebstein's anomaly. Even with early intervention, ventricular function and effort tolerance remain suboptimal. Bidirectional Glenn could offload the right ventricle and could be considered in patients with severe right ventricular dysfunction, severe TR, and effort intolerance, though its efficacy post-cone repair remains uncertain. Early transplant assessment should be considered in these cases in the event of deterioration. For patients with persistent dysfunction, early referral for transplant evaluation should be considered to optimize long-term outcomes.

Yuan SM. Ebstein's Anomaly: Genetics, Clinical Manifestations, and Management. Pediatr Neonatol. 2017;58(3):211-215. doi:10.1016/j.pedneo.2016.08.004

Hetzer R, Hacke P, Javier M, et al. The long-term impact of various techniques for tricuspid repair in Ebstein's anomaly. J Thorac Cardiovasc Surg. 2015;150(5):1212-1219. doi:10.1016/j.jtcvs.2015.08.036