María Gallego-Delgado1, Esther González-López2, Francisco Muñoz-Beamud3, Juan Buades4, Lucía Galán5, Jose Luis Muñoz-Blanco6, Javier Sánchez-González7, Borja Ibáñez8, Jesus G Mirelis9, Pablo García-Pavía10. 1. Servicio de Cardiología, Hospital Universitario Puerta de Hierro, Madrid, Spain. 2. Servicio de Cardiología, Hospital Universitario Puerta de Hierro, Madrid, Spain; Área de Fisiopatología del Miocardio, Centro Nacional de Investigaciones Cardiovasculares (CNIC), Madrid, Spain. 3. Servicio de Medicina Interna, Hospital Juan Ramón Jiménez, Huelva, Spain. 4. Servicio de Medicina Interna, Hospital Son Llàtzer, Palma de Mallorca, Spain. 5. Servicio de Neurología, Hospital Clínico San Carlos, Madrid, Spain. 6. Servicio de Neurología, Hospital General Gregorio Marañón, Madrid, Spain. 7. Philips Healthcare Iberia, Madrid, Spain; Unidad de Imagen Cardiaca Avanzada, Centro Nacional de Investigaciones Cardiovasculares (CNIC), Madrid, Spain. 8. Unidad de Imagen Cardiaca Avanzada, Centro Nacional de Investigaciones Cardiovasculares (CNIC), Madrid, Spain. 9. Servicio de Cardiología, Hospital Universitario Puerta de Hierro, Madrid, Spain; Unidad de Imagen Cardiaca Avanzada, Centro Nacional de Investigaciones Cardiovasculares (CNIC), Madrid, Spain. 10. Servicio de Cardiología, Hospital Universitario Puerta de Hierro, Madrid, Spain; Área de Fisiopatología del Miocardio, Centro Nacional de Investigaciones Cardiovasculares (CNIC), Madrid, Spain. Electronic address: JESUS.GMIRELIS@telefonica.net.
Abstract
INTRODUCTION AND OBJECTIVES: 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 (RS = 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 (RS = 0.604; P = .001), Norfolk Quality of Life questionnaire (RS = 0.529; P = .003) and Karnofsky index (RS= -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.
INTRODUCTION AND OBJECTIVES: 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 amyloidosispatients (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 (RS = 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 (RS = 0.604; P = .001), Norfolk Quality of Life questionnaire (RS = 0.529; P = .003) and Karnofsky index (RS= -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.
Authors: Maaike van den Boomen; Riemer H J A Slart; Enzo V Hulleman; Rudi A J O Dierckx; Birgitta K Velthuis; Pim van der Harst; David E Sosnovik; Ronald J H Borra; Niek H J Prakken Journal: J Magn Reson Imaging Date: 2017-11-13 Impact factor: 4.813