title: Relationship between left ventricular diastolic function and myocardial sympathetic denervation measured by (123)I-meta-iodobenzylguanidine imaging in Anderson-Fabry disease.
abstract: OBJECTIVE
  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 (123)I-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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Advanced Biomedical Sciences, University Federico II, Naples, Italy. letspine@unina.it.
authors.forename: Letizia
authors.identifier: http://orcid.org/0000-0002-9774-6268
authors.initials: L
authors.lastname: Spinelli
authors.affiliation: National Council of Research, Institute of Biostructure and Bioimaging, Naples, Italy.
authors.forename: Teresa
authors.initials: T
authors.lastname: Pellegrino
authors.affiliation: Department of Public Health, University Federico II, Naples, Italy.
authors.forename: Antonio
authors.initials: A
authors.lastname: Pisani
authors.affiliation: Department of Advanced Biomedical Sciences, University Federico II, Naples, Italy.
authors.forename: Caterina Anna
authors.initials: CA
authors.lastname: Giudice
authors.affiliation: Department of Public Health, University Federico II, Naples, Italy.
authors.forename: Eleonora
authors.initials: E
authors.lastname: Riccio
authors.affiliation: Department of Advanced Biomedical Sciences, University Federico II, Naples, Italy.
authors.forename: Massimo
authors.initials: M
authors.lastname: Imbriaco
authors.affiliation: IRCCS SDN, Naples, Italy.
authors.forename: Marco
authors.initials: M
authors.lastname: Salvatore
authors.affiliation: Department of Advanced Biomedical Sciences, University Federico II, Naples, Italy.
authors.forename: Bruno
authors.initials: B
authors.lastname: Trimarco
authors.affiliation: Department of Advanced Biomedical Sciences, University Federico II, Naples, Italy.
authors.forename: Alberto
authors.initials: A
authors.lastname: Cuocolo
mesh_terms: D019797:3-Iodobenzylguanidine; D000328:Adult; D000368:Aged; D003971:Diastole; D000795:Fabry Disease / Q000000981:diagnostic imaging* / Q000503:physiopathology; D005260:Female; D006321:Heart / Q000294:innervation; D006801:Humans; D008297:Male; D008875:Middle Aged; D049268:Positron-Emission Tomography; D019275:Radiopharmaceuticals; D013564:Sympathetic Nervous System / Q000503:physiopathology*; D016277:Ventricular Function, Left
chemical_list: D019275:Radiopharmaceuticals; D019797:3-Iodobenzylguanidine
keywords: 123I-metaiodobenzylguanidine; Anderson-Fabry disease; Cardiac sympathetic function; Diastolic dysfunction