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

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

doi:10.1007/s00259-015-3273-5

Cited by 14 publications

(7 citation statements)

References 34 publications

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“…ese pathological changes, associated with GL-3 related damage to the sympathetic nervous system, cause the onset of cardiac conduction disorders, left ventricular hypertrophy, and heart failure even with preserved ejection fraction [45,46].…”

Section: Anderson-fabry Disease and Endothelial Dysfunctionmentioning

confidence: 99%

“…In parallel to a drastic reduction in plasma, urine, and tissue concentrations of GL-3, ERT was found to determine a variety of clinical effects: from slowing the decline in estimated glomerular filtration rate (eGFR), to reducing or stabilizing cardiac remodelling and ameliorating neurological and gastrointestinal symptoms [42,48]. e lack of clinical improvements, but also of further deterioration, during ERT was regarded as a marker of clinical benefit in an otherwise progressive, debilitating disease associated with a risk of premature mortality [42,45,46,48,51,52].…”

Section: Enzyme Replacement Therapy and Emergingmentioning

confidence: 99%

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Anderson–Fabry Disease: From Endothelial Dysfunction to Emerging Therapies

Stamerra

Pinto

Giosia

et al. 2021

Advances in Pharmacological and Pharmaceutical Sciences

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The Anderson–Fabry disease is a rare, X-linked, multisystemic, progressive lysosomal storage disease caused by α-galactosidase A total or partial deficiency. The resulting syndrome is mainly characterized by early-onset autonomic neuropathy and life-threatening multiorgan involvement, including renal insufficiency, heart disease, and early stroke. The enzyme deficiency leads to tissue accumulation of the glycosphingolipid globotriaosylceramide and its analogues, but the mechanisms linking such accumulation to organ damage are only partially understood. In contrast, enzyme replacement and chaperone therapies are already fully available to patients and allow substantial amelioration of quality and quantity of life. Substrate reduction, messenger ribonucleic acid (mRNA)-based, and gene therapies are also on the horizon. In this review, the clinical scenario and molecular aspects of Anderson–Fabry disease are described, along with updates on disease mechanisms and emerging therapies.

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Section: Anderson-fabry Disease and Endothelial Dysfunctionmentioning

confidence: 99%

Section: Enzyme Replacement Therapy and Emergingmentioning

confidence: 99%

Anderson–Fabry Disease: From Endothelial Dysfunction to Emerging Therapies

Stamerra

Pinto

Giosia

et al. 2021

Advances in Pharmacological and Pharmaceutical Sciences

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“…Spinelli et al reported a reduced H/M ratio in AFD patients, but did not show localization of the reduced MIBG uptake. 4 A possible explanation for this observation is regional differences in cardiac innervation. The inferior wall, as compared with the anteroseptal wall, is predominantly innervated by parasympathetic nerves and less innervated by sympathetic nerves.…”

Section: Discussionmentioning

confidence: 99%

Focal Reduction in Cardiac <sup>123</sup>I-Metaiodobenzylguanidine Uptake in Patients With Anderson-Fabry Disease

Yamamoto

Suzuki

Sugimura

et al. 2016

Circ J

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Background: It remains to be elucidated whether cardiac sympathetic nervous activity is impaired in patients with Anderson-Fabry disease (AFD). Methods and Results:We performed 123 I-meta-iodobenzylguanidine (MIBG) scintigraphy and gadoliniumenhanced cardiovascular magnetic resonance (CMR) in 5 AFD patients. MIBG uptake in the inferolateral wall, where wall thinning and delayed enhancement were noted on CMR, was significantly lower compared with the anteroseptal wall. The localized reduction in MIBG uptake was also noted in 2 patients with no obvious abnormal findings on CMR.Conclusions: Cardiac sympathetic nervous activity is impaired in AFD before development of structural myocardial abnormalities. (Circ J 2016; 80: 2550 -2551

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“…Aberrant autonomic nervous system responses cause sympathetic hyperactivity and reduced parasympathetic activity. 10,11 Besides, impaired cardiac innervation is related to ventricular dysfunction. 12 It has been shown that hyperactivation of the sympathetic fibers provide inotropic support to the failing heart and peripheral vasoconstriction to maintain arterial pressure.…”

Section: Discussionmentioning

confidence: 99%

Evaluation of Ventricular Diastolic Function in Patients With Fibromyalgia Syndrome

Sayılır

Ergün

Ekiz³

2018

Arch Rheumatol

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Objectives:This study aims to compare the diastolic functions between fibromyalgia (FM) patients and control subjects by using transthoracic echocardiography. Patients and methods:This case-control and cross-sectional study included 34 female FM patients (mean age 43.6±8.2 years; range 28 to 57 years), who were diagnosed by The American College of Rheumatology 1990 Criteria for the Classification of Fibromyalgia and defined as the FM group. A control group was defined consisting of 30 healthy females (mean age 41.2±9.1 years; range 22 to 54 years). Echocardiography findings of the groups were compared. Results: The body mass index and mean disease duration of the FM group were 25.37±2.71 kg/m 2 and 12.24 months, respectively. The body mass index of the control group was 25.58±1.49 kg/m 2 . There was no significant difference between the groups in terms of age and body mass index. Isovolumetric relaxation time and mitral E-wave deceleration time values were significantly higher in the FM group than in the control group (p=0.047, p=0.003, respectively). Conclusion: Isovolumetric relaxation time and mitral valve deceleration time are significantly prolonged in FM patients compared with healthy subjects. Female patients with FM seem to be under risk of impaired relaxation and diastolic function of the left ventricle.

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

Abstract: 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.

Cited by 14 publications

References 34 publications

Anderson–Fabry Disease: From Endothelial Dysfunction to Emerging Therapies

Anderson–Fabry Disease: From Endothelial Dysfunction to Emerging Therapies

Focal Reduction in Cardiac <sup>123</sup>I-Metaiodobenzylguanidine Uptake in Patients With Anderson-Fabry Disease

Evaluation of Ventricular Diastolic Function in Patients With Fibromyalgia Syndrome

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