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Right Heart Remodeling After Pulmonary Valve Replacement in Patients With Pulmonary Atresia or Critical Stenosis With

Margaret Irwin1,2, Lindsey Reynolds1,2, Geoffrey Binney1,2

  • 1Harvard Medical School Boston MA.

Journal of the American Heart Association
|November 6, 2023
PubMed

Insights

Patients with pulmonary atresia/intact ventricular septum (PA/IVS) show similar right ventricular remodeling after pulmonary valve replacement (PVR) compared to tetralogy of Fallot (TOF). PA/IVS patients had less RV mass reduction and higher RV ejection fraction post-PVR.

Area of Science:

  • Cardiology
  • Pediatric Cardiac Surgery
  • Cardiac Imaging

Background:

  • Pulmonary valve replacement (PVR) is crucial for patients with pulmonary atresia or critical pulmonary stenosis with intact ventricular septum (PA/IVS).
  • Right ventricular (RV) remodeling post-PVR is well-documented in tetralogy of Fallot (TOF).
  • Understanding RV changes in PA/IVS post-PVR is essential for optimizing patient outcomes.

Purpose of the Study:

  • To investigate and compare right ventricular (RV) remodeling in patients with PA/IVS versus TOF after pulmonary valve replacement (PVR).
  • To analyze cardiac magnetic resonance imaging (CMR) data to assess RV changes, including volume, ejection fraction, and mass.

Main Methods:

  • Retrospective cohort study of PA/IVS patients undergoing PVR (1995-2021) at Boston Children's Hospital, matched 1:3 with TOF patients by age at PVR.
  • Cardiac magnetic resonance imaging (CMR) was used to evaluate RV indexed end-diastolic volume, ejection fraction, and mass before and after PVR.
  • Median regression modeling was applied to compare RV remodeling between the PA/IVS and TOF groups.

Main Results:

  • A total of 20 PA/IVS patients (cases) and 60 TOF patients (controls) were analyzed, with a median age of 14 years at PVR.
  • Post-PVR, both groups showed similar reductions in indexed RV end-diastolic volume.
  • PA/IVS patients exhibited a higher RV ejection fraction and less reduction in RV mass compared to TOF patients post-PVR.

Conclusions:

  • Patients with PA/IVS demonstrate comparable RV remodeling to TOF patients following PVR.
  • PA/IVS patients experience a lower reduction in RV mass and maintain a higher RV ejection fraction post-PVR.
  • Further research into diastolic parameters is warranted to fully understand RV function post-PVR in PA/IVS.

Keywords:
congenital heart diseasepulmonary atresiastrain imagingvalve replacement

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