Reduced Myocardial 123-Iodine Metaiodobenzylguanidine Uptake A Prognostic Marker in Familial Amyloid Polyneuropathy

被引:69
作者
Azevedo Coutinho, Maria C. [1 ,3 ,4 ]
Cortez-Dias, Nuno [1 ,3 ,6 ]
Cantinho, Guilhermina [5 ]
Conceicao, Isabel [2 ,4 ]
Oliveira, Antonio [3 ]
Bordalo e Sa, Armando [1 ]
Goncalves, Susana [1 ]
Almeida, Ana G. [1 ,3 ]
de Carvalho, Mamede [2 ,4 ]
Diogo, Antonio Nunes [1 ,3 ]
机构
[1] Santa Maria Univ Hosp, Dept Cardiol, P-1649035 Lisbon, Portugal
[2] Santa Maria Univ Hosp, Dept Neurosci, P-1649035 Lisbon, Portugal
[3] Univ Lisbon, Lisbon Med Sch, Univ Clin Cardiol, P-1699 Lisbon, Portugal
[4] Univ Lisbon, Lisbon Med Sch, Translat Clin Physiol Unit, Inst Med Mol, P-1699 Lisbon, Portugal
[5] Univ Lisbon, Lisbon Med Sch, Inst Nucl Med, P-1699 Lisbon, Portugal
[6] Minist Hlth & Fdn Sci & Technol, Programme Adv Med Educ, Fundacao Calouste Gulbenkian, Lisbon, Portugal
关键词
amyloid; prognosis; radionuclide imaging; LIVER-TRANSPLANTATION; SYMPATHETIC INNERVATION; CLINICAL-FEATURES; FIBRIL PROTEIN; PORTUGUESE; HEART; CARDIOMYOPATHY; PROGRESSION; DENERVATION; FAP;
D O I
10.1161/CIRCIMAGING.112.000367
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Background Transthyretin familial amyloid polyneuropathy is a hereditary form of amyloidosis characterized by sensorimotor and autonomic neuropathy, cardiac conduction defects, and infiltrative cardiomyopathy. Previous studies have suggested that myocardial sympathetic denervation assessed by 123-iodine metaiodobenzylguanidine (MIBG) imaging occurs early in disease progression. However, its prognostic significance was never evaluated. We aimed to study the long-term prognostic value of myocardial sympathetic denervation detected by MIBG imaging in transthyretin familial amyloid polyneuropathy. Methods and Results A total of 143 individuals with V30M transthyretin mutation underwent Holter, ambulatory blood pressure monitoring, echocardiography, and MIBG imaging. Time to all-cause death was compared with late heart-to-mediastinum MIBG uptake ratio (H/M; either in relation to the estimated lower limit of normal [1.60] or as a continuous variable) using Cox proportional hazards regression. Multivariable analyses were performed to test the prognostic accuracy of clinical, neurological, and cardiovascular parameters. During a median follow-up of 5.5 years, 32 (22%) patients died. Five-year mortality rate was 42% for late H/M <1.60 and 7% for late H/M 1.60 (hazard ratio, 7.19; P<0.001). Late H/M was identified as an independent prognostic predictor. Fifty-three patients were submitted to liver transplantation. In comparison with neurophysiological score-matched controls, transplanted patients had lower long-term mortality (hazard ratio, 0.32; P=0.012). Patients with late H/M<1.60 were at higher risk of unfavorable outcome but seemed to have benefited from liver transplantation. Conclusions Cardiac sympathetic denervation as assessed by MIBG imaging is a useful prognostic marker in transthyretin familial amyloid polyneuropathy.
引用
收藏
页码:627 / 636
页数:10
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