Abstract

Background: Infants with shunt-dependent pulmonary blood flow are at risk for developing proximal pulmonary artery (PA) stenosis, which may result in morbidity and mortality. Transthoracic echocardiography (TTE) is the primary means of surveillance for PA narrowing but has significant limitations and has not been compared to computed tomographic angiography (CTA)–derived measurements of the proximal PA anatomy in this population. Methods: A retrospective chart review identified infants with shunt-dependent pulmonary blood flow who had both TTE and CTA performed <14 days apart during a five-year period. Proximal right pulmonary artery (RPA) and left pulmonary artery (LPA) diameters were measured by TTE and CTA. Pulmonary artery z-score, linear and intraclass correlation (ICC) coefficients, and Bland-Altman plots were computed. Results: Seventeen pairs of studies were analyzed. The TTE and CTA proximal PA diameters had moderate linear correlation and agreement ( R = 0.67, P ≤ .0001, ICC = 0.65); the RPA showed stronger correlation and agreement ( R = 0.76, P = .0004, ICC = 0.72) than the LPA (R = 0.59, P = .01, ICC = 0.59). Computed tomographic angiography detected missed PA stenosis ( Z score < −2) in five (14.7%) cases, four of which were on the LPA. Conclusion: In this study of infants with shunt-dependent pulmonary blood flow, TTE and CTA proximal PA diameters had only moderate correlation and agreement overall, which was worse when comparing LPA measurements. This resulted in missed PA stenosis by TTE. Computed tomographic angiography may be warranted in patients with poorly visualized PAs by TTE or suspicion for deficient pulmonary blood flow.

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