Heghine Khachatryan: When Vascular Fragility Defines Risk – Rethinking Hemostasis in LDS
Heghine Khachatryan, Editor-in-Chief of Hemostasis Today, Head of Hemophilia and Thrombosis Center at Yeolyan Hematology and Oncology Center, shared a post on LinkedIn:
“When Vascular Fragility Defines Risk: Rethinking Hemostasis in Loeys – Dietz Syndrome
Loeys – Dietz syndrome (LDS) is widely recognized as a connective tissue disorder with aggressive vascular involvement, driven by mutations in the TGF-β signaling pathway.
Yet, beyond aneurysms and dissections, an important question remains insufficiently addressed:
Do we fully understand the hemostatic and perioperative risks in these patients?
Clinical Reality
Patients with LDS often present with:
- Marked arterial tortuosity and aneurysmal disease
- Extreme vascular fragility
- Need for repeated surgical or endovascular interventions
However, intraoperative and postoperative courses may be complicated by:
- Unexpected bleeding
- Tissue friability
- Challenging hemostasis despite ‘normal’ routine labs
The Hidden Hemostasis Dimension
While LDS is not classically categorized as a coagulation disorder, several mechanisms may be clinically relevant:
- Structural vessel wall defects – mechanical bleeding tendency
- Altered extracellular matrix – impaired vascular integrity
- Possible endothelial dysfunction
- Limited evidence regarding platelet – vessel interaction abnormalities
Key Questions for Experts
- Should LDS be considered a functional bleeding-risk condition, even with normal PT/aPTT?
- Is there value in extended hemostasis testing (platelet function, viscoelastic assays)?
- How should we optimize perioperative hemostatic strategies?
- Should antifibrinolytics (e.g., tranexamic acid) be used more proactively?
- Do current surgical protocols sufficiently reflect vascular fragility biology?”
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