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Direct Re-implantation of Left Coronary Artery into the Aorta in Adults with Anomalous Origin of Left Coronary Artery from the Pulmonary Artery (ALCAPA)
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Relationship Between Aortic Root Enlargement and Pulmonary Blood Flow in Pulmonary Atresia With Major Aortopulmonary

Richard D Mainwaring1,2, Nicole M Jacobs1,2, Raj Punn1,2

  • 1Division of Pediatric Cardiac Surgery, Stanford University School of Medicine, Lucile Packard Children's Hospital Stanford, Stanford, California.

Annals of Thoracic Surgery Short Reports
|June 10, 2026
PubMed

Insights

In patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries (PA/VSD/MAPCAs), aortic size did not correlate with blood flow ratios within surgical subgroups. This suggests increased flow alone does not enlarge aortas in these complex congenital heart conditions.

Area of Science:

  • Cardiology
  • Pediatric Cardiology
  • Congenital Heart Disease

Background:

  • Patients with PA/VSD/MAPCAs exhibit enlarged aortas, potentially due to increased blood flow and intrinsic aortopathy.
  • Previous research indicates a link between aortic enlargement and hemodynamic factors in this population.

Purpose of the Study:

  • To investigate the correlation between aortic root size and the ratio of pulmonary to systemic blood flow (Qp/Qs) in patients with PA/VSD/MAPCAs.
  • To determine if increased blood flow directly contributes to aortic enlargement within specific surgical management subgroups.

Main Methods:

  • Retrospective review of 169 patients with PA/VSD/MAPCAs who underwent surgery.
  • Analysis of echocardiographic data for aortic root size and z-scores.
  • Correlation of Qp/Qs values from preoperative cardiac catheterization with aortic dimensions across three surgical subgroups: single-stage complete repair, unifocalization and shunt, and aortopulmonary window.

Main Results:

  • Patients undergoing single-stage complete repair had significantly higher Qp/Qs values and aortic root z-scores compared to the other two groups.
  • Despite differences in Qp/Qs and aortic size between groups, no correlation was found between Qp/Qs and aortic root size or z-score within any of the three subgroups.
  • The study identified distinct anatomic and physiologic patterns among the three surgical subgroups.

Conclusions:

  • Aortic size in PA/VSD/MAPCAs is not directly correlated with the ratio of pulmonary to systemic blood flow within specific surgical management strategies.
  • These findings suggest that factors beyond incremental increases in blood flow, such as intrinsic aortopathy, may play a more significant role in aortic enlargement in this patient population.
Abstract

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