Purpose or Case Report: Aortic calcifications are an uncommon finding in the pediatric population and when present are often reflective of underlying congenital heart or metabolic disease. Intraluminal polypoid calcified lesions (coral reef aorta) causing flow limiting stenosis are exceedingly rare in even the congenital heart disease population.
A 15-year-old patient with a history of hypoplastic left heart syndrome (HLHS) with mitral and aortic atresia. She was found to have significant flow limiting stenosis of the augmented neo-aorta under catheter hemodynamic assessment. CT angiography was performed showing a 4.4 cm partially calcified mass in the ascending aorta extending into the proximal arch causing an 80% narrowing of the aortic lumen. The intrinsic multi-energy capabilities of the first-generation photon counting CT (PCCT) scanner used permitted retrospective material decomposition and thus more definitive characterization of the mass. The patient underwent open ascending aorta replacement and arch repair where densely calcified ascending aorta and base of the arch were removed en bloc along with a large intraluminal mass component without complication. Pathologic assessment revealed PTFE conduit material, thrombus, and extensive calcification with fibrous incorporation of graft and arterial wall.
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Conclusions: The coral reef aorta was originally described in 1984 with sporadic cases described in the literature predominantly affecting the abdominal aorta with lower limb ischemia as the presenting symptom. This case represents the first reported case in a pediatric patient as well as the first associated with graft material. This case also highlights the retrospective spectral nature of PCCT imaging which incorporates the problem-solving capabilities of multi-energy spectral acquisition into every CT scan.