Heghine Khachatryan: Marfan vs LDS – Not All Aortopathies Are the Same
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:
“Marfan vs Loeys – Dietz: Not All Aortopathies Are the Same
Marfan syndrome and Loeys – Dietz syndrome (v) share phenotypic overlap, yet their biological behavior and clinical risk profiles differ fundamentally.
While Marfan syndrome is primarily a structural connective tissue disorder (FBN1-related) with progressive aortic root dilation,
LDS represents a TGF-β – driven vasculopathy characterized by:
- Aggressive and early-onset aneurysms
- Widespread arterial involvement (beyond the aorta)
- Marked vascular tortuosity
- Encreased risk of dissection at smaller diameters
Importantly, LDS should be approached not merely as an aortopathy, but as a systemic vascular fragility condition, with implications for:
- Earlier surgical thresholds
- Meticulous perioperative planning
- Individualized risk assessment
Normal coagulation tests do not exclude bleeding risk in LDS.
Clinical Insight
- Marfan – structural aortopathy
- Loeys – Dietz – aggressive systemic vasculopathy
Recognizing this distinction is not academic – it is life – saving.”
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