Spontaneous Coronary Artery Dissection in Loeys-Dietz Syndrome: Role of Optical Coherence Tomography in Diagnosis and Management.
Coronary artery aneurysm with dissection is an uncommon presentation of Loeys-Dietz syndrome (LDS). Diagnosis and management of patients with LDS who present with unstable clinical syndromes merit special attention given unique pathophysiology derangements associated with this disease entity. Clinical experience in treating such patients is limited to a few case reports, and an optimal catheter-based treatment approach is less well defined. Intravascular imaging techniques, particularly optical coherence tomography (OCT), are able to provide further insight about the morphology of lesions and guide-catheter based intervention. In this case report, we describe one such patient and highlight the key morphological characteristics of coronary artery disease in patients with LDS as well as the crucial role of OCT in its management.
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Anoop
A
Agrawal
Shamsa
S
Baaj
Jeffrey
J
Schwartz
John J
JJ
Lopez
D003323:Coronary Aneurysm / Q000150:complications / Q000209:etiology; D003330:Coronary Vessel Anomalies / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology / Q000601:surgery*; D003937:Diagnosis, Differential; D019468:Disease Management; D006801:Humans; D047908:Intracellular Signaling Peptides and Proteins / Q000235:genetics*; D055947:Loeys-Dietz Syndrome / Q000150:complications / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology / Q000601:surgery; D008297:Male; D008875:Middle Aged; D009154:Mutation; D062645:Percutaneous Coronary Intervention / Q000379:methods*; D041623:Tomography, Optical Coherence / Q000379:methods*; D014652:Vascular Diseases / Q000151:congenital* / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology / Q000601:surgery
D047908:Intracellular Signaling Peptides and Proteins; C111893:TGFBRAP1 protein, human