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Focal reduction in left ventricular 123 I-metaiodobenzylguanidine uptake and impairment in systolic function in patients with Anderson-Fabry disease.
- Source :
-
Journal of nuclear cardiology : official publication of the American Society of Nuclear Cardiology [J Nucl Cardiol] 2021 Apr; Vol. 28 (2), pp. 641-649. Date of Electronic Publication: 2019 May 13. - Publication Year :
- 2021
-
Abstract
- 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.<br />Methods: Twenty-four AFD patients (43.7 ± 12.8 years) were studied by <superscript>123</superscript> I-metaiodobenzylguanidine (MIBG) cardiac imaging and speckle-tracking echocardiography. Segmental tracer uptake was estimated according to 0 to 4 score, and total defect score (TDS) was calculated for each patient.<br />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 <superscript>2.7</superscript> ), relative wall thickness (0.51 ± 0.06 vs. 0.40 ± 0.06), systolic pulmonary artery pressure (35.2 ± 6.7 vs. 27.2 ± 4.2 mmHg), and longitudinal strain (- 14.3 ± 2.7 vs. -19.4 ± 1.8%) were significantly higher in patients with segmental defect (all P < 0.01). At multivariate linear regression analysis, global longitudinal strain was independently associated with TDS (B = 3.007, 95% confidence interval 1.384 to 4.630, P = 0.001).<br />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.
- Subjects :
- Adult
Fabry Disease complications
Female
Heart Ventricles innervation
Humans
Male
Middle Aged
Tomography, Emission-Computed, Single-Photon
Young Adult
3-Iodobenzylguanidine pharmacokinetics
Fabry Disease physiopathology
Radiopharmaceuticals pharmacokinetics
Sympathetic Nervous System physiopathology
Systole physiology
Ventricular Dysfunction, Left etiology
Subjects
Details
- Language :
- English
- ISSN :
- 1532-6551
- Volume :
- 28
- Issue :
- 2
- Database :
- MEDLINE
- Journal :
- Journal of nuclear cardiology : official publication of the American Society of Nuclear Cardiology
- Publication Type :
- Academic Journal
- Accession number :
- 31087266
- Full Text :
- https://doi.org/10.1007/s12350-019-01734-8