Work

Relationship Between Aortic Root Enlargement and Pulmonary Blood Flow in Pulmonary Atresia With Major Aortopulmonary Collateral Arteries

Year: 2025

Type: article

Abstract: Background Patients with pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) have markedly enlarged aortas compared with standard control subjects. Previous studies have suggested that aortic enlargement may be attributable to a combination of increased flow and intrinsic aortopathy. We hypothesized that aortic sizes in patients with PA/VSD/MAPCAs would correlate with the ratio of pulmonary to systemic blood flow (Q p /Q s ) values. Methods This was a retrospective review of 169 patients with PA/VSD/MAPCAs who underwent a surgical procedure. Echocardiographic studies were reviewed to assess aortic root size and z-scores. The Q p /Q s values were gleaned from preoperative cardiac catheterization. Patients were divided into 3 subgroups: single-stage complete repair, unifocalization and shunt, and aortopulmonary window. Results Median age at surgery was 6.1 months for the single-stage complete repair group, 5.9 months for the unifocalization and shunt group, and 1.9 months for the aortopulmonary window group. Patients who underwent a single-stage complete repair had higher Q p /Q s values (1.78 vs 0.94, 0.93; P < .001), higher absolute aortic root measurements (1.95 cm vs 1.66, 1.52; P < .05), and higher aortic root z-scores (5.81 vs 1.10, 2.92; P < .01) compared with patients who underwent unifocalization and shunt procedure and aortopulmonary window procedures. However, within each subgroup there was no correlation between Q p /Q s and either aortic root size or aortic root z-scores. Conclusions The data demonstrate that there were 3 distinct anatomic and physiologic patterns for the 3 subgroups. Within each subgroup, no correlation was found between Q p /Q s and either aortic root size or z-score. These results suggest that incremental increases in flow do not result in additional increases in aortic size.

Source: Annals of Thoracic Surgery Short Reports

DOI: 10.1016/j.atssr.2025.11.006

Authors: Richard D. Mainwaring, Nicole M. Jacobs, Raj Punn, Pooja Punn, Alisa Arunamata, Frank L. Hanley

Institutions: Lucile Packard Children's Hospital, Stanford Medicine, Stanford University

Language: English

Topic: Congenital Heart Disease Studies

Subfield: Epidemiology

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: gold

Estimated APC paid: $975