Publication Date




Download Full Text (335 KB)


Background Cardiac anomalies in PHACE syndrome (P: posterior fossa anomalies, H: hemangiomas, A: arterial lesions, C: cardiac abnormalities, E: abnormalities of the eye) often consists of complex aortic arch anomalies with double aortic arch (DAA) being an uncommon association. Atresia of the proximal segment of the left arch in DAA is extremely rare and can pose a diagnostic and management conundrum due to formation of a loose vascular ring and asymptomatic nature as seen in our case. Case Description A 5-year-old male, diagnosed with PHACE syndrome, at a different center was noted to have an incidental finding on head imaging of a right aortic arch (RAA) with an aberrant left subclavian artery. He was seen in our center for his follow up after relocation. Clinically, he was asymptomatic, growing well with no respiratory symptoms or dysphagia. His echocardiogram showed RAA with aberrant origin of the left subclavian artery along with the left common carotid from a distal trunk suspicious of a DAA. On cross sectional imaging, a DAA was appreciated with each arch giving rise to a common carotid artery and a subclavian artery with a vertebral artery (Figure 1A and 1B). The presumed portion of the left arch proximal to the common carotid artery did not have any discrete contrast enhancement but the proximity of the left and right carotid arteries suggested ligamentous continuity between the two arches completing the double arch and forming a vascular ring (Figure 2). A ductal dimple was appreciated on the undersurface of the left common carotid suggestive of a left sided ductus (Figure 2). There was no significant tracheal or left mainstem bronchus compression between the pulmonary trunk and distal left arch. Based on the asymptomatic presentation and discussion with family, our consensus was to not intervene surgically. Discussion: The diagnosis of this rare type of DAA with PHACE syndrome has not been previously described to our knowledge. An echocardiogram can assess common arch anomalies associated with this syndrome, however, cross-sectional imaging with CT angiography or MRI can help differentiate this rare diagnosis from its close mimics. Proximity of the right and left carotid arteries, symmetric four vessel sign at the thoracic inlet, posterior course of a patent segment of the atretic left arch and presence of the ductal dimple under the left common carotid are important diagnostic clues that can help differentiate this rare variant of DAA. Although surgical intervention has been performed in limited reported cases, diagnostic accuracy is paramount as patient may remain symptomatic if only the ductal ligament or atretic arch ligament is divided and the other left intact. Given the “loose vascular ring” and asymptomatic nature, patient specific discussion should be considered with the family to assess optimal management strategy. Conclusion: Aortic arch anomalies are common in PHACE syndrome, however, rare variants of DAA can be seen. Keeping a high index of suspicion and complementing cross sectional imaging with baseline echocardiography even in asymptomatic cases can be useful for diagnostic and management considerations as seen in our case.


Cardiology | Pediatrics


Presented at the 27th Annual Update on Pediatric and Congenital Cardiovascular Disease (Cardiology 2024); Scottsdale, AZ; Feb 14-18, 2024.

Double Aortic Arch with Atresia of the Left Aortic Arch Proximal to the Left Common Carotid Artery in a Patient with PHACE Syndrome – A Management Conundrum