Outflow tract geometries are associated with adverse outcome indicators in repaired tetralogy of Fallot

J Thorac Cardiovasc Surg. 2021 Jul;162(1):196-205. doi: 10.1016/j.jtcvs.2020.09.072. Epub 2020 Sep 22.

Abstract

Objectives: A wide variety of right ventricular outflow tract (RVOT) and pulmonary artery (PA) geometries has been reported in patients with repaired tetralogy of Fallot (rTOF). We aimed to investigate the associations between RVOT/PA geometries and outcome indicators in a large rTOF cohort receiving non-conduit repair.

Methods: Three-dimensional magnetic resonance angiographic images of 206 patients with rTOF who had a pulmonary regurgitation (PR) fraction ≥20% were reviewed. Patients' RVOT geometry was quantitatively classified into 4 distinct shapes (tubular, hourglass, pyramid, and inverted trapezoid). Bilateral PA size discrepancy was defined as the diameter of the smaller side being less than 70% of that of the bigger side.

Results: Based on lateral projection of the 3-dimensional images, patients with an inverted trapezoid-shaped RVOT had the smallest RV end-diastolic volume index (EDVi) (108.7 ± 24.3 mL/m2) and pulmonary valve annulus diameter, and shortest QRS duration, whereas those with a pyramid-shaped RVOT had the largest RV EDVi (161.0 ± 44.6 mL/m2) and pulmonary valve annulus diameter. Similar trends of differences were also observed if such classifications were based on the frontal projections. Multivariable analysis revealed that RVOT shapes, subvalvular diameter, PR fraction, QRS duration, and the presence of bilateral PA size discrepancy were independent determinants of RV EDVi. Furthermore, having bilateral PA size discrepancy (25.2%) was independently associated with lower peak oxygen consumption (P = .041).

Conclusions: Distinct RVOT morphologies and branch PA size discrepancy are associated with variations in RV remodeling and exercise capacity in patients with rTOF. These findings may aid decision-making regarding reintervention for PR and branch PA size discrepancy.

Keywords: branch pulmonary artery size discrepancy; cardiovascular magnetic resonance; exercise capacity; right ventricular outflow tract morphology; tetralogy of Fallot.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Exercise Tolerance / physiology
  • Female
  • Heart Ventricles* / diagnostic imaging
  • Heart Ventricles* / pathology
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Pulmonary Artery* / diagnostic imaging
  • Pulmonary Artery* / pathology
  • Pulmonary Valve Insufficiency / etiology
  • Pulmonary Valve Insufficiency / physiopathology
  • Pulmonary Valve Insufficiency / surgery
  • Tetralogy of Fallot / complications
  • Tetralogy of Fallot / physiopathology
  • Tetralogy of Fallot / surgery*
  • Young Adult