Focal reduction in left ventricular
3-Iodobenzylguanidine
/ pharmacokinetics
Adult
Fabry Disease
/ complications
Female
Heart Ventricles
/ innervation
Humans
Male
Middle Aged
Radiopharmaceuticals
/ pharmacokinetics
Sympathetic Nervous System
/ physiopathology
Systole
/ physiology
Tomography, Emission-Computed, Single-Photon
Ventricular Dysfunction, Left
/ etiology
Young Adult
Cardiac innervation
Cardiomyopathy
Echo
SPECT
Journal
Journal of nuclear cardiology : official publication of the American Society of Nuclear Cardiology
ISSN: 1532-6551
Titre abrégé: J Nucl Cardiol
Pays: United States
ID NLM: 9423534
Informations de publication
Date de publication:
Apr 2021
Apr 2021
Historique:
received:
17
01
2019
accepted:
15
04
2019
revised:
21
03
2019
pubmed:
16
5
2019
medline:
1
2
2022
entrez:
16
5
2019
Statut:
ppublish
Résumé
Abnormalities of cardiac sympathetic innervation have been demonstrated in Anderson-Fabry disease (AFD). We aimed to investigate the relationship between regional left ventricular (LV) denervation and regional function abnormalities. Twenty-four AFD patients (43.7 ± 12.8 years) were studied by Segmental longitudinal strain worsened as MIBG uptake score increased (P < 0.001). By ROC analysis, a segmental longitudinal strain > - 16.2% predicted a segmental MIBG uptake score ≥1, with 79.7% sensitivity and 65.3% specificity. Segmental MIBG uptake defects were found in 13 out 24 AFD patients. LV mass index (60.8 ± 10.1 vs. 41.4 ± 9.8 g/h Reduced cardiac MIBG uptake reflects the severity of cardiac involvement in AFD patients. LV longitudinal function impairment seems to be an earlier disease feature than regional myocardial denervation.
Sections du résumé
BACKGROUND
BACKGROUND
Abnormalities of cardiac sympathetic innervation have been demonstrated in Anderson-Fabry disease (AFD). We aimed to investigate the relationship between regional left ventricular (LV) denervation and regional function abnormalities.
METHODS
METHODS
Twenty-four AFD patients (43.7 ± 12.8 years) were studied by
RESULTS
RESULTS
Segmental longitudinal strain worsened as MIBG uptake score increased (P < 0.001). By ROC analysis, a segmental longitudinal strain > - 16.2% predicted a segmental MIBG uptake score ≥1, with 79.7% sensitivity and 65.3% specificity. Segmental MIBG uptake defects were found in 13 out 24 AFD patients. LV mass index (60.8 ± 10.1 vs. 41.4 ± 9.8 g/h
CONCLUSIONS
CONCLUSIONS
Reduced cardiac MIBG uptake reflects the severity of cardiac involvement in AFD patients. LV longitudinal function impairment seems to be an earlier disease feature than regional myocardial denervation.
Identifiants
pubmed: 31087266
doi: 10.1007/s12350-019-01734-8
pii: 10.1007/s12350-019-01734-8
doi:
Substances chimiques
Radiopharmaceuticals
0
3-Iodobenzylguanidine
35MRW7B4AD
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
641-649Références
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