CTICasesPulmonary Artery Aneurysm in Beckwith-Wiedemann Syndrome
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Imaging findings
The patient presents with a large pulmonary artery aneurysm involving the main pulmonary artery and its proximal branches, while the distal pulmonary arteries appear normal. The right heart is not dilated, there is no hypertrophy, and the atriums and left atrial pressure are normal, with no evidence of pulmonary regurgitation.
Key takeaways
Pulmonary artery aneurysm can be an uncommon, isolated complication of Beckwith-Wiedemann syndrome, a congenital overgrowth syndrome. These are believed to be true aneurysms, not pseudoaneurysms, and can occur in the absence of pulmonary hypertension or significant valvular disease. The underlying mechanism is thought to involve abnormalities in smooth muscle or connective tissue. Other causes of pulmonary artery aneurysms include congenital etiologies, valve disease, and vasculitides like Behçet disease.
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