Comprehensive evaluation of right ventricular function in children with different anatomical subtypes of hypoplastic

Colin Petko1, Peter Möller, Ulrike Hoffmann

  • 1Children's Heart Centre, University Hospital of Schleswig-Holstein, Campus Kiel, Schwanenweg 20, 24105 Kiel, Germany. petko@pedcard.uni-kiel.de

Insights

In hypoplastic left heart syndrome (HLHS) survivors, right ventricular (RV) function after Fontan surgery was similar between those with mitral/aortic atresia (MA/AA) and other HLHS subgroups. This finding suggests RV function is not impacted by LV size differences post-surgery.

Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Disease
  • Cardiac Surgery

Background:

  • Hypoplastic left heart syndrome (HLHS) survivors with larger left ventricular (LV) cavities may face poorer long-term outcomes.
  • A diminished LV cavity, as seen in mitral and aortic atresia (MA/AA), is hypothesized to potentially preserve right ventricular (RV) function.

Purpose of the Study:

  • To compare RV function in HLHS patients post-Fontan surgery.
  • To investigate if the presence of MA/AA and a negligible LV impacts RV function compared to other HLHS anatomical variations.

Main Methods:

  • Retrospective echocardiographic evaluation of RV function in 42 HLHS children post-Fontan surgery.
  • Utilized 2D, Doppler, and color tissue Doppler imaging.
  • Compared patients with MA/AA (group 1, n=20) to other HLHS subgroups (group 2, n=22).

Main Results:

  • The MA/AA group exhibited a smaller ascending aorta diameter and LV area at birth.
  • The MA/AA group had a larger RV end-diastolic and end-systolic area post-Fontan.
  • No significant differences in RV function parameters (MPI, TAPSE, RV FAC, myocardial velocities) were observed between the groups.

Conclusions:

  • RV function in HLHS patients following Fontan surgery was comparable between the MA/AA subgroup and other HLHS anatomical variations.
  • The anatomical differences in LV size did not translate to measurable differences in RV function in this cohort.
Abstract

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