Relationship between left ventricular diastolic function and myocardial sympathetic denervation measured by 123I-meta-iodobenzylguanidine imaging in Anderson-Fabry disease

Letizia Spinelli, Teresa Pellegrino, Antonio Pisani, Caterina Anna Giudice, Eleonora Riccio, Massimo Imbriaco, Marco Salvatore, Bruno Trimarco, Alberto Cuocolo

Research output: Contribution to journalArticlepeer-review


Purpose: Whether cardiac sympathetic nervous function abnormalities may be present in patients with Anderson-Fabry disease (AFD) remains unexplored. We investigated the relationship between left ventricular (LV) function and cardiac sympathetic nervous function in patients with AFD. Methods: Twenty-five patients (12 men, mean age 43 ± 13 years) with genetically proved AFD and preserved LV ejection fraction and ten age and gender-matched control subjects underwent speckle tracking echocardiography and 123I-meta-iodobenzylguanidine (MIBG) imaging from which early and late heart to mediastinum (H/M) ratios and myocardial washout rate values were calculated. Results: In AFD patients, a significant correlation between late H/M ratio and LV mass index (r = −61, p = 0.001), left atrial volume (r = −0.72, p <0.001), systolic pulmonary artery pressure (r = −0.75, p <0.001), and early diastolic untwisting rate (r = −0.66, p <0.001) was found. Ten AFD patients exhibited a late H/M ratio below two fold standard deviation of control subjects (≤1.75). Patients showing late H/M ratio ≤ 1.75 had significantly higher LV mass index, relative wall thickness, left atrial volume and systolic pulmonary artery pressure, lower systolic longitudinal strain and an early diastolic untwisting rate compared to patients with late H/M ratio > 1.75. At multivariable linear regression analysis, early diastolic untwisting rate was the only independent predictor of late H/M ratio ≤ 1.75 (odds ratio 1.15, 95 % confidence interval 1.07–1.31, p <0.05). Conclusion: The present findings provide the first demonstration of a cardiac sympathetic derangement in AFD patients with preserved LV ejection fraction, which is mostly related to LV diastolic dysfunction.

Original languageEnglish
Pages (from-to)729-739
Number of pages11
JournalEuropean Journal of Nuclear Medicine and Molecular Imaging
Issue number4
Publication statusPublished - Apr 1 2016


  • I-metaiodobenzylguanidine
  • Anderson-Fabry disease
  • Cardiac sympathetic function
  • Diastolic dysfunction

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging


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