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Dilated cardiomyopathy, or DCM, is a progressive myocardial disorder characterized by ventricular chamber dilation and contractile dysfunction.EtiologyVarious factors can cause DCM, including hypertension and heavy alcohol intake, which contribute to the weakening and enlargement of the heart muscle. Viral infections, such as Coxsackievirus B, adenoviruses, and influenza, can lead to DCM by causing inflammation and damage to heart tissue. Certain chemotherapeutic agents, including daunorubicin,...
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Does left ventricular function predict cardiac outcome in Anderson-Fabry disease?

Letizia Spinelli1, Giuseppe Giugliano2, Antonio Pisani3

  • 1Department of Advanced Biomedical Sciences, Federico II University of Naples, Via Pansini, 5, 80131, Naples, Italy. letspine@unina.it.

The International Journal of Cardiovascular Imaging
|November 19, 2020
PubMed
Summary

Left ventricular (LV) function impairment predicts adverse cardiac events in Anderson-Fabry disease (AFD). Myocardial work (MW) did not add prognostic value beyond clinical factors and LV function.

Keywords:
Adverse cardiac eventAnderson–Fabry diseaseLV longitudinal strainMyocardial work

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Area of Science:

  • Cardiology
  • Genetics
  • Echocardiography

Background:

  • Anderson-Fabry disease (AFD) is a genetic disorder affecting cardiac health.
  • The prognostic impact of left ventricular (LV) function in AFD remains unclear.
  • Myocardial work (MW) assessed via pressure-strain loops is a novel echocardiographic metric.

Purpose of the Study:

  • To determine if LV function impacts cardiac outcomes in AFD patients.
  • To investigate the prognostic value of MW in AFD.
  • To assess MW's additive predictive capability.

Main Methods:

  • Retrospective analysis of 96 AFD patients with normal LV ejection fraction and ≥2-year follow-up.
  • Evaluation of standard echocardiography, myocardial mechano-energetic efficiency (MEE), global longitudinal strain (GLS), and MW.
  • Adverse cardiac events defined as a composite of cardiac death, VT, AF, or severe heart failure.

Main Results:

  • During median 63-month follow-up, 14 adverse events occurred.
  • Univariate analysis showed age, biomarkers, LV mass, LA volume, E/Ea, LVEF, MEE, GLS, and MW indices predicted outcome.
  • GLS and MW independently predicted adverse events (p<0.01), with constructive MW ≤1513 mmHg% showing high sensitivity (92.9%) and specificity (86.6%).
  • MW did not improve prediction models including clinical data, diastolic function, and GLS.

Conclusions:

  • LV systolic and diastolic dysfunction are associated with adverse cardiac events in AFD.
  • Myocardial work (MW) does not offer additional prognostic information beyond established clinical and echocardiographic parameters in AFD.