The impact of unilateral pulmonary artery stenosis on right ventricular to pulmonary arterial coupling in patients with transposition of the great arteries

Renée S. Joosen, Michiel Voskuil, Gregor J. Krings, M. Louis Handoko, Michael G. Dickinson, Marielle C. van de Veerdonk, Johannes M.P.J. Breur*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

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Abstract

Background: Unilateral pulmonary artery (PA) stenosis is common in the transposition of the great arteries (TGA) after arterial switch operation (ASO) but the effects on the right ventricle (RV) remain unclear. Aims: To assess the effects of unilateral PA stenosis on RV afterload and function in pediatric patients with TGA-ASO. Methods: In this retrospective study, eight TGA patients with unilateral PA stenosis underwent heart catheterization and cardiac magnetic resonance (CMR) imaging. RV pressures, RV afterload (arterial elastance [Ea]), PA compliance, RV contractility (end-systolic elastance [Ees]), RV-to-PA (RV-PA) coupling (Ees/Ea), and RV diastolic stiffness (end-diastolic elastance [Eed]) were analyzed and compared to normal values from the literature. Results: In all TGA patients (mean age 12 ± 3 years), RV afterload (Ea) and RV pressures were increased whereas PA compliance was reduced. RV contractility (Ees) was decreased resulting in RV-PA uncoupling. RV diastolic stiffness (Eed) was increased. CMR-derived RV volumes, mass, and ejection fraction were preserved. Conclusion: Unilateral PA stenosis results in an increased RV afterload in TGA patients after ASO. RV remodeling and function remain within normal limits when analyzed by CMR but RV pressure–volume loop analysis shows impaired RV diastolic stiffness and RV contractility leading to RV-PA uncoupling.

Original languageEnglish
Pages (from-to)943-948
Number of pages6
JournalCatheterization and Cardiovascular Interventions
Volume103
Issue number6
DOIs
Publication statusPublished - 1 May 2024

Keywords

  • cardiac magnetic resonance imaging
  • pulmonary pressures
  • pulmonary stenosis
  • right ventricular dysfunction
  • transposition of the great arteries

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