Spontaneous Coronary Artery Dissection in Fragile X Syndrome
- 1Department of Cardiology, Royal Papworth Hospital, Cambridge, United Kingdom
Abstract
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.
Related
- Double-Vessel Involvement: A Case Report of Simultaneous Left Anterior Descending Artery (LAD) and Right Coronary Artery (RCA) Spontaneous Coronary Artery Dissection Causing Acute Coronary Syndrome
- Reply: Spontaneous coronary artery dissection in France
- Spontaneous coronary artery dissection revealed by an iatrogenic spasm provocation test.
- Spontaneous coronary artery dissection presenting as myocardial infarction in a postmenopausal woman
- Recurrent Spontaneous Coronary Artery Dissection.
BibTeX
@article{anwar2026spontaneous,
title = {{Spontaneous Coronary Artery Dissection in Fragile X Syndrome}},
author = {Majid Anwar and Harsh Chawla and Thomas Kaier and Paul Bambrough},
journal = {JACC Case Reports},
year = {2026},
month = sep,
doi = {10.1016/j.jaccas.2026.110387},
url = {https://doi.org/10.1016/j.jaccas.2026.110387},
note = {CC BY 4.0},
}
RIS
TY - JOUR
TI - Spontaneous Coronary Artery Dissection in Fragile X Syndrome
AU - Majid Anwar
AU - Harsh Chawla
AU - Thomas Kaier
AU - Paul Bambrough
PY - 2026
DA - 2026/09/23
JO - JACC Case Reports
DO - 10.1016/j.jaccas.2026.110387
UR - https://doi.org/10.1016/j.jaccas.2026.110387
AB - 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.
SN - 2666-0849
ER -