Document Detail

Impact of liver transplantation on cardiac autonomic denervation in familial amyloid polyneuropathy.
MedLine Citation:
PMID:  16862048     Owner:  NLM     Status:  MEDLINE    
Familial amyloid polyneuropathy (FAP) is a rare and severe hereditary form of amyloidosis, due to the deposition of a genetic variant transthyretin essentially produced by the liver, and characterized by both sensorimotor and autonomic neuropathy. Liver transplantation (LT) is the most effective treatment to stop the progression of the disease. Cardiac amyloid infiltration is usually associated with cardiac denervation, restrictive cardiomyopathy, conduction disturbances, and sometimes sudden death. Whether the cardiac involvement related to amyloid deposition may be altered after LT remains unclear. We conducted the present study to define the outcome of cardiac involvement after LT in 31 patients with FAP (age, 39 +/- 12 yr). Patients were evaluated before and after LT (24 +/- 15 mo). Cardiac sympathetic denervation was assessed by both iodine-123 metaiodobenzylguanidine (MIBG) scintigraphy and heart rate variability (HRV) analysis. The scintigraphic importance of sympathetic denervation was evaluated globally on planar imaging using heart-to-mediastinum activity ratio (H/M) measured 4 hours after injection, and regionally using single-photon emission tomography (SPET) imaging. Amyloid myocardial infiltration was assessed by echocardiography. Diffuse sympathetic denervation was found when using cardiac MIBG planar imaging in patients evaluated before LT and compared with 12 control subjects (H/M: 1.45 +/- 0.29 vs. 1.98 +/- 0.35, p < 0.001). On SPET images, defects were diffuse in 12 patients and focal in 19 patients, with predominance at the inferior and apical segments. No change in sympathetic innervation was found in patients after LT as assessed either with planar imaging (H/M after LT: 1.46 +/- 0.28, p = not significant vs. H/M before LT) or with SPET imaging. HRV nonspectral indexes showed that the standard deviation of all cycles was significantly lower in patients compared with control subjects, and remained unchanged after LT. Conduction disturbances and ventricular arrhythmias were associated with low cardiac MIBG uptake, and progressed after LT. The left ventricular wall was slightly thickened in patients, and a further increase was observed after LT (posterior wall from 9.2 +/- 1.8 to 10.1 +/- 2.3 mm, p = 0.02; septal wall from 10.6 +/- 2.7 to 12.1 +/- 4, p = 0.046). Neurologic status stabilized in 26 patients, but worsened in the 5 patients who had the most severe cardiac sympathetic denervation before LT as measured by MIBG imaging. The magnitude of the cardiac sympathetic denervation remained stable 2 years after LT in patients with FAP, whereas the cardiac amyloid infiltration progressed. The importance of cardiac sympathetic denervation found in FAP patients before LT was associated with a neurologic worsening after LT.
Nicolas Delahaye; François Rouzet; Laure Sarda; Carmen Tamas; Sylvie Dinanian; Violaine Plante-Bordeneuve; David Adams; Didier Samuel; Pascal Merlet; André Syrota; Michel S Slama; Dominique Le Guludec
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Medicine     Volume:  85     ISSN:  0025-7974     ISO Abbreviation:  Medicine (Baltimore)     Publication Date:  2006 Jul 
Date Detail:
Created Date:  2006-07-24     Completed Date:  2006-08-31     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  2985248R     Medline TA:  Medicine (Baltimore)     Country:  United States    
Other Details:
Languages:  eng     Pagination:  229-38     Citation Subset:  AIM; IM    
Service de Médecine Nucléaire, Hôpital Bichat, AP-HP, Paris.
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MeSH Terms
3-Iodobenzylguanidine / diagnostic use
Amyloid Neuropathies, Familial / complications*,  diagnosis,  surgery*
Electrocardiography, Ambulatory
Heart / innervation*
Heart Catheterization
Liver Transplantation*
Middle Aged
Radiopharmaceuticals / diagnostic use
Severity of Illness Index
Sympathetic Nervous System / physiopathology*
Reg. No./Substance:
0/Radiopharmaceuticals; 77679-27-7/3-Iodobenzylguanidine

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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