Spontaneous Coronary Artery Dissection in Fragile X Syndrome: A Novel IVUS-Guided PCI Approach

Spontaneous coronary artery dissection is an uncommon cause of acute coronary syndromes, with rare reports in fragile X syndrome.

A 44-year-old man with fragile X syndrome presented with ventricular fibrillation arrest and anterior ST-segment elevation. Angiography and intravascular ultrasound (IVUS) revealed extensive left anterior descending artery spontaneous coronary artery dissection with inadvertent false-lumen wiring. A dual-catheter technique was used, with live false-lumen IVUS-guided true-lumen wiring, hematoma fenestration, and ostial stenting, with postprocedural imaging confirming dissection sealing. Computed tomography angiography identified additional silent dissections of the celiac, superior mesenteric, and right femoral arteries.

This case suggests systemic vascular fragility in fragile X syndrome and describes a novel IVUS-guided bailout strategy using live false-lumen imaging to facilitate true-lumen wiring and ostial stent positioning.

Acute coronary syndrome in fragile X syndrome should prompt consideration of systemic arteriopathy and multivessel dissection. Live false-lumen IVUS may facilitate true-lumen wiring when conventional techniques fail.