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

BACKGROUND: Spontaneous coronary artery dissection is an uncommon cause of acute coronary syndromes, with rare reports in fragile X syndrome. CASE SUMMARY: 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. DISCUSSION: 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. TAKE-HOME MESSAGES: 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.