Frontiers in Medicine · Published 2026-08-05 · DOI 10.3389/fmed.2026.1912034
Qian Qian, Yalan Zhou, Jia Shi, Qinlan Chen
BackgroundInterrupted aortic arch (IAA) is a rare congenital cardiac anomaly, almost invariably lethal in the neonatal period without surgical intervention, with adult survival exceedingly rare. The hemodynamic consequences of uncorrected Celoria–Patton type B IAA with a ventricular septal defect (VSD) and a patent ductus arteriosus (PDA) have not been previously characterized by 4D cardiac computed tomography (4D-CCT)-based computational fluid dynamics (CFD).Case presentationAn 18-year-old woman with known congenital heart disease since childhood but without complete anatomic characterization presented with exertional dyspnea (NYHA class II), a grade 6/6 holosystolic murmur, and a 22 mmHg arm–leg blood pressure gradient. Echocardiography identified a large perimembranous VSD (3.33 cm) with bidirectional flow, a 0.60 cm ductus arteriosus, severe tricuspid regurgitation (PGmax 203 mmHg), and an estimated pulmonary artery systolic pressure of 168 mmHg. 4D-CCT confirmed Celoria–Patton type B IAA with the descending thoracic aorta arising as a ductal continuation from the right pulmonary artery and supplying the left subclavian artery distally. Right heart catheterization documented severe predominantly pre-capillary pulmonary hypertension (mPAP 82 mmHg; PAWP 9 mmHg; PVRi 9.6 Wood units·m2; Qp 9.7 L/min, Qs 3.2 L/min, Qp/Qs 3.02). 4D-CCT-based CFD showed bidirectional VSD shunting and a main pulmonary artery time-averaged wall shear stress (WSS) of 2.18 Pa — higher than the modelled ascending aortic WSS (1.22 Pa) — with a mildly dilated main pulmonary artery (z-score +2.1). Inhaled nitric oxide reduced mPAP from 82 to 64 mmHg, a partial vasodilator response.ConclusionIn adults with unrepaired type B IAA, 4D-CCT-based CFD characterizes bidirectional shunting and provides hypothesis-generating biomechanical information concordant with invasive hemodynamics; it may be useful for longitudinal surveillance of pulmonary artery geometry.
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Qian, Q., Zhou, Y., Shi, J., et al. (2026). Case Report: Higher modelled pulmonary wall shear stress in an adult with Celoria–Patton type B aortic interruption, ventricular septal defect, and patent ductus arteriosus. Frontiers in Medicine. https://doi.org/10.3389/fmed.2026.1912034