Cardiac Remodeling Trajectory and Renal Stage-Specific Risk Stratification in East Asian Fabry Disease Patients

Area of Science:

• Cardiology
• Genetics
• Nephrology

Background:

• Static assessments fail to capture Fabry cardiomyopathy's dynamic nature.
• Clinical outcomes in Fabry disease are highly variable.

Purpose of the Study:

• Evaluate prognostic value of cardiac remodeling trajectories.
• Assess renal stage-specific structural thresholds for risk stratification.

Main Methods:

• Longitudinal remodeling velocity quantified by anatomical cardiac raw score (aCRS) slope in 105 Fabry patients.
• Primary outcome: composite of heart failure hospitalization or cardiovascular death.
• Firth's penalized Cox regression used for prognostic associations.

Main Results:

• Higher baseline aCRS and steeper aCRS slope independently predicted adverse outcomes.
• Enzyme replacement therapy showed significant protective effect.
• Renal stage-specific thresholds identified for risk stratification.

Conclusions:

• Cardiac remodeling velocity predicts adverse outcomes in Fabry disease.
• Integrating dynamic structural data and renal stage improves risk stratification.
• Personalized management strategies can be developed based on these findings.