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Type A Dissection at Subthreshold Dimensions in Loeys-Dietz Syndrome: When Guideline-Directed Surveillance Is Not
Zachary Bokhari1, Hirohisa Ikegami2, Sabahat Bokhari3
1Rutgers Robert Wood Johnson Medical School, New Brunswick, New Jersey, USA.
Background:
Loeys-Dietz syndrome (LDS) is a heritable connective tissue disorder associated with aggressive aortic disease.
Case Summary:
A 45-year-old woman with a family history of aortic dissection and a TGFB2 variant associated with LDS underwent surveillance demonstrating an aortic root diameter of 3.7 to 4.0 cm. Five months later, she developed an acute Stanford type A aortic dissection requiring emergent Bentall aortic root and hemiarch replacement. Her postoperative course was complicated by graft-adjacent infection, giant graft-adjacent pseudoaneurysms requiring redo aortic root replacement with Cabrol coronary reconstruction, and inferior ST-segment elevation myocardial infarction caused by thrombotic occlusion of the right coronary graft.
Discussion:
This case illustrates catastrophic vascular progression despite guideline-directed surveillance and highlights the challenges of long-term management after aortic root reconstruction.
Take-Home Messages:
Patients with LDS may experience life-threatening aortic complications despite subthreshold aortic dimensions. Improved individualized risk stratification beyond maximal aortic diameter is needed to optimize prophylactic intervention.