Left Atrial Dysfunction in Children with Repaired Pulmonary Artery Atresia with Ventricular Septal Defect: A

Yanyan Ma1, Liwei Hu1, Qian Wang1

  • 1Department of Radiology, Shanghai Children's Medical Center, School of Medicine, Shanghai Jiao Tong University, Shanghai 200120, China.

Insights

Cardiac MRI reveals altered left atrial (LA) function in repaired pulmonary artery atresia with ventricular septal defect (rPA/VSD) patients, even with preserved biventricular ejection fraction. This dysfunction may precede LA enlargement and indicate early diastolic dysfunction.

Area of Science:

  • Cardiology
  • Medical Imaging
  • Pediatric Cardiology

Background:

  • The left atrium (LA) plays a crucial role beyond left ventricular filling.
  • Pulmonary artery atresia with ventricular septal defect (PA/VSD) is a complex congenital heart defect requiring surgical repair.
  • Assessing residual cardiac function post-repair is vital for long-term patient management.

Purpose of the Study:

  • To evaluate left atrial (LA) volume and function in pediatric patients with repaired PA/VSD using cardiac magnetic resonance (CMR).
  • To identify potential early indicators of diastolic dysfunction in this patient population.

Main Methods:

  • Prospective recruitment of 31 pediatric patients with repaired PA/VSD and 30 healthy controls.
  • Utilized CMR for comprehensive assessment of LA volume and function, including ejection fraction (EF), strain, and strain rate during reservoir, conduit, and pump phases.
  • Employed cardiac function analysis software for detailed measurements.

Main Results:

  • Patients with repaired PA/VSD exhibited significantly decreased maximal LA volume index compared to controls.
  • Significantly lower LA reservoir strain and strain rate, conduit strain and strain rate, booster pump strain rate, total EF, and passive EF were observed in the PA/VSD group.
  • Even in patients with preserved but lower right ventricular EF (<50%), reduced LA reservoir strain, reservoir strain rate, and pump strain rate were noted compared to those with higher RVEF (≥50%).

Conclusions:

  • Repaired PA/VSD is associated with altered LA function, detectable even when biventricular EF is preserved.
  • These functional changes may serve as an early marker for left ventricular diastolic dysfunction.
  • CMR is effective in detecting early-stage LA dysfunction, potentially before anatomical changes like LA enlargement become apparent.