Endocardial Fibroelastosis Recurrence: Comparing Single Ventricle Palliation versus Biventricular Repair

Background: The presence of endocardial fibroelastosis (EFE) has been reported in several cardiac diseases and can potentially restrict ventricular growth and function. Ventricular recruitment surgery with the goal of biventricular repair (BiV) involves resection of EFE to alleviate ventricular restriction. Even after successful resection, recurrence is often observed and patients undergo repeated resections. This study aims to determine the outcome of primary EFE resection (PR) in patients with small ventricles at Boston Children's Hospital (BCH).

Method: In a retrospective review of medical records, imaging data, and surgical pathology, we identified all EFE resections at BCH in patients with small ventricles from January 2010 to March 2021. We determined EFE recurrence, defined by repeat resection and/or progression on imaging, and compared single ventricle palliation (SV) versus BiV outcomes in terms of recurrence and survival.

Results: PR was performed in 59 patients (mean age, years ± SD; 1.8 ± 2.7) with small left (sLV, n = 49) and small right (sRV, n = 10) ventricles. In sLV, the risk of recurrence was significantly higher than in sRV (76.1 ± 7.4 vs. 14.4 ± 13.2%, p = 0.007) and mean time to recurrence significantly shorter (mean age, years, IQR: 4.1 [2.7–5.4] vs. 9.2 [7.1–11.2], p = 0.007). The overall survival rate was 79.6 ± 6.4%, with a mean survival time of 9.3 years [IQR: 8.3–10.4] after PR, and did not differ between sLV and sRV. A BiV repair was conducted in 79.7% of cases, with a 10.6% BiV failure rate. The disease-free interval after PR in patients with successful BiV repair was significantly longer compared with patients with SV physiology, despite an attempt for ventricular rehabilitation (mean age, years, IQR: 5.6 [4.1–7.1] vs. 2.0 [0.6–3.4], p = 0.015). The risk of overall EFE recurrence in BiV was significantly lower (BiV, 61.6 ± 9.4 vs. SV, 89.0 ± 10.3%, p = 0.015) and overall survival rates were significantly higher (BiV, 86.3 vs. SV, 59.1 ± 14.2%, p = 0.023).

Conclusion: EFE resection results in successful BiV repair in over 70% of patients with small ventricles and EFE at birth. In the remaining patients, EFE recurrence occurred faster, likely indicating more aggressive disease progression, and resulted in SV palliation. Furthermore, despite successful BiV repair, the probability of EFE recurrence remained greater than 60% in a 10-year observation period. Our data demonstrate the need to identify the mechanisms behind EFE disease progression to develop treatment options for EFE beyond surgical resection.

Publication History

Article published online:
12 February 2022