Publication Date

9-2025

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Abstract

We present a case of a newborn, born at 32 weeks, with prenatally diagnosed massive ascending aorta (AAo) dilatation and multiple congenital anomalies with unrevealing genetic testing. Postnatal echo revealed a bicuspid aortic valve and a dilated AAo (2.2cm, z-score 12). At 2 months of age, he had progressive hypoxic respiratory failure requiring intubation, with frequent positional hypoxia despite adequate ventilation. Repeat echocardiogram showed a 9mm increase in AAo diameter over 4 weeks. Surgical intervention was indicated, but the cause of hypoxia was unclear. The dilated AAo was found to compress the right atrium, causing right-to-left shunting at the atrial level, confirmed by agitated saline study. The patient underwent hemiarch and AAo replacement with a pulmonary homograft at 11 weeks of age. Imaging Findings: Initial echo showed a bicuspid aortic valve, massively dilated AAo (2.2cm, z-score 12), and PFO with left-to-right flow. CTA on day of life 5 confirmed these findings. Repeat echo 1 month later showed stable aortic measurements with no clear atrial-level shunt. At 2 months, a follow up echocardiogram and confirmatory CTA revealed further dilation of the AAo, extending to the arch (3.1cm, z-score 17), with mild anterior tracheal mass effect. No atrial defect was seen, so an echo with agitated saline was performed, which was rapidly positive, confirming right-to-left shunting. Role of Imaging in Patient Care: CTA did not show significant airway or pulmonary artery compression. The atrial septum was challenging to image on echo due to mass effect and distortion. The agitated bubble study quickly confirmed intracardiac right-to-left shunting. Discussion This patient presented a unique challenge in differentiating the etiology of his positional hypoxia (lung vs cardiac). Multimodality imaging and agitated saline study confirmed suspicion right atrial compression from his rapidly dilating aorta, a very unusual finding in the absence of a known genetic disorder, leading to right-to-left shunting across the PFO.

Disciplines

Cardiology | Pediatrics

Notes

Presented at the American Society of Echocardiography (ASE) 36th Annual Scientific Sessions; Nashville, TN; September 5-7, 2025.

Massively Dilated Aorta in a Neonate Leading to Symptomatic Right Heart Compression

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