Extracellular Volume Detects Amyloidotic Cardiomyopathy and Correlates With Neurological Impairment in Transthyretin-familial Amyloidosis

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• 作者：Marí ; a Gallego-Delgado^a ; ^&loz ; ; Esther Gonzá ; lez-Ló ; pez^a ; ^b ; ^&loz ; ; Francisco Muñ ; oz-Beamud^c ; Juan Buades^d ; Lucí ; a Galá ; n^e ; Jose Luis Muñ ; oz-Blanco^f ; Javier Sá ; nchez-Gonzá ; lez^g ; ^h ; Borja Ibá ; ñ ; ez^h ; Jesus G. Mirelis^a ; ^h ; Pablo Garcí ; a-Paví ; a^a ; ^b ; ^{JESUS.GMIRELIS@telefonica.net" class="auth_mail" title="E-mail the corresponding author} ; ^{pablogpavia@yahoo.es" class="auth_mail" title="E-mail the corresponding author}
• 关键词：Amyloid ; Transthyretin ; Cardiac magnetic resonance ; Extracellular cardiac volume ; T1-mapping
• 刊名：Revista Española de Cardiología (English Edition)
• 出版年：2016
• 出版时间：October 2016
• 年：2016
• 卷：69
• 期：10
• 页码：923-930
• 全文大小：659 K

文摘

Cardiac involvement determines prognosis and treatment options in transthyretin-familial amyloidosis. Cardiac magnetic resonance T₁ mapping techniques are useful to assess myocardial extracellular volume. This study hypothesized that myocardial extracellular volume allows identification of amyloidotic cardiomyopathy and correlates with the degree of neurological impairment in transthyretin-familial amyloidosis.

Methods

A total of 31 transthyretin-familial amyloidosis patients (19 mean age, 49 ± 12 years; 26 with the Val30Met mutation) underwent a T₁ mapping cardiac magnetic resonance study and a neurological evaluation with Neuropathy Impairment Score of the Lower Limb score, Norfolk Quality of Life questionnaire, and Karnofsky index.

Results

Five patients had cardiac amyloidosis (all confirmed by ^99mTc-DPD scintigraphy). Mean extracellular volume was increased in patients with cardiac amyloidosis (0.490 ± 0.131 vs 0.289 ± 0.035; P = .026). Extracellular volume correlated with age (R = 0.467; P = .008), N-terminal pro-B-type natriuretic peptide (R_S = 0.846; P < .001), maximum wall thickness (R = 0.621; P < .001), left ventricular mass index (R = 0.685; P < .001), left ventricular ejection fraction (R = –0.378; P = .036), Neuropathy Impairment Score of the Lower Limb (R_S = 0.604; P = .001), Norfolk Quality of Life questionnaire (R_S = 0.529; P = .003) and Karnofsky index (R_S= –0.517; P = .004). A cutoff value of extracellular volume of 0.357 was diagnostic of cardiac amyloidosis with 100% sensitivity and specificity (P < .001). Extracellular volume and N-terminal pro-B-type natriuretic peptide were the only cardiac parameters that significantly correlated with neurologic scores.

Conclusions

Extracellular volume quantification allows identification of cardiac amyloidosis and correlates with the degree of neurological impairment in transthyretin-familial amyloidosis. This noninvasive technique could be a useful tool for early diagnosis of cardiac amyloidosis and to track cardiac and extracardiac amyloid disease.