Backgrounds:
Systemic right ventricular circulation after Fontan procedures is known to have late hemodynamic complications. Although a number of studies have investigated the factors that may impact on survival, postoperative outcomes after palliations remain to be elucidated.
Objective:
The purpose of this study is to investigate the prognostic value of myocardial fibrosis identified by cardiac magnetic resonance imaging (cMRI) in patients with single ventricular physiology.
Methods:
Consecutive 23 patients undergoing Fontan procedures were prospectively scheduled to have cMRI study with late gadolinium enhancement (LGE) imaging and ventricle circumferential strain measurement before and 4 months after Fontan operation.
Results:
Of 23 patients (mean age 3.3±0.9 years), 7 were positive for LGE (LGE+) and median percent LGE was 3.0% (interquartile range 3.0% to 7.5%). Pre-Fontan examinations revealed that patients with LGE+ showed an increase in end-diastolic volume index (139.7±26.8 ml/BSA vs. 113.3±20.9 ml/BSA; P=0.02) and end-systolic volume index (ESVI: 99.9±32.2 ml/BSA vs. 70.8±20.0 ml/BSA; P=0.01) compared with those without LGE (LGE-). In contrast to LGE- group, LGE+ patients showed lower global circumferential strain (4.1±2.3% vs. 7.9±2.7%, P=0.006), decreased ejection fraction (EF: 29±9.1% vs. 38±8.7%; P=0.04), and reduced end-systolic elastance (1.1±0.3 mm Hg/ml/m2 vs. 1.7±0.5 mm Hg/ml/m2). In addition, LGE+ group had higher levels of BNP (91.0±72.4 pg/ml vs. 30.9±44.0 pg/ml, P=0.02) and New York University Pediatric Heart Failure Index (10.9±3.3 vs. 7.8±1.1, P=0.02) than LGE- group. This was validated by positive correlations between the area of LGE versus ESVI (r=0.85, P=0.01) and BNP levels (r=0.82, P=0.02), respectively. At 4 months after Fontan procedure, LGE- group showed higher EF (37.5±8.6% vs. 24.0±8.9%, P=0.02) compared with those in LGE+ patients, and increased global circumferential strain (6.5±2.0% to 7.4±2.7%, P=0.04).
Conclusion:
LGE identified by cMRI before operation may be associated with lower ventricular elastance that resulted in poorer functional recovery after staged palliation. This novel strategy may provide a prognostic value of latent myocardial dysfunction after Fontan procedure.