Cone reconstruction for Ebstein anomaly: Late biventricular function and possible remodeling

To evaluate late-term tricuspid valve competence and biventricular function following cone reconstruction for Ebstein anomaly, and to explore biventricular remodeling.

Consecutive adult and pediatric patients who underwent cone reconstruction from 2009 to 2019 were reviewed for inclusion in this retrospective cardiac magnetic resonance imaging study. Tricuspid valve competence was assessed with tricuspid regurgitation fraction. Biventricular systolic function was assessed by ejection fraction, cardiac index, indexed stroke volume, and indexed aortic and pulmonary artery beat volume. Biventricular remodeling was assessed by planimetered areas (right atrium, functional right ventricle, left heart), and indexed end-diastolic and end-systolic ventricular volumes. Paired t tests or Wilcoxon signed-rank tests were used for analyses.

Of 58 included patients, 50 underwent cardiac magnetic resonance imaging. Twelve patients had both preoperative and late postoperative cardiac magnetic resonance imaging with a median follow-up of 5.11 years (interquartile range, 3.12-6.07 years). Focusing on these, tricuspid regurgitation fraction decreased (from 69% to 10%; P = .014), right ventricle ejection fraction remained stable, and antegrade pulmonary artery beat volume increased (from 26.7 to 41.6 mL/beat/m²; P = .037). The left ventricle stroke volume (from 30.4 to 44.1 mL/m²; P = .015) and antegrade aortic beat volume (from 28.5 to 41.1 mL/beat/m²; P = .014) also increased, and the left ventricle stroke volume improved progressively with time since surgery (P = .048). Whereas the right atrium area decreased (P = .004), the functional right ventricle and left heart area increased (cm², P = .021 and P = .004). Right ventricle volumes showed a tendency to normalize and left ventricle indexed end-diastolic volume increased (from 50 to 69 mL/m²; P = .03) over time.

Cone valve integrity was sustained. Biventricular function improved progressively during follow-up, and there are positive signs of biventricular remodeling late after cone reconstruction.