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Heghine Khachatryan: When Vascular Fragility Defines Risk – Rethinking Hemostasis in LDS

Heghine Khachatryan/LinkedIn

Apr 10, 2026, 01:19

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?”

Heghine Khachatryan