Identification and assessment of Anderson-Fabry disease by cardiovascular magnetic resonance noncontrast myocardial T1 mapping.
Daniel Sado,Steven K White,Stefan K. Piechnik,Sanjay M Banypersad,Thomas A. Treibel,Gabriella Captur,Marianna Fontana,Viviana Maestrini,Andrew S. Flett,Matthew D. Robson,Robin H. Lachmann,Elaine Murphy,Atul Mehta,Derralynn Hughes,Stefan Neubauer,Perry M. Elliott,James C. Moon +16 more
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TLDR
Noncontrast T1 mapping shows potential as a unique and powerful measurement in the imaging assessment of LVH and AFD.Abstract:
Background— Anderson-Fabry disease (AFD) is a rare but underdiagnosed intracellular lipid disorder that can cause left ventricular hypertrophy (LVH). Lipid is known to shorten the magnetic resonance imaging parameter T1. We hypothesized that noncontrast T1 mapping by cardiovascular magnetic resonance would provide a novel and useful measure in this disease with potential to detect early cardiac involvement and distinguish AFD LVH from other causes.
Methods and Results— Two hundred twenty-seven subjects were studied: patients with AFD (n=44; 55% with LVH), healthy volunteers (n=67; 0% with LVH), patients with hypertension (n=41; 24% with LVH), patients with hypertrophic cardiomyopathy (n=34; 100% with LVH), those with severe aortic stenosis (n=21; 81% with LVH), and patients with definite amyloid light-chain (AL) cardiac amyloidosis (n=20; 100% with LVH). T1 mapping was performed using the shortened modified Look-Locker inversion sequence on a 1.5-T magnet before gadolinium administration with primary results derived from the basal and midseptum. Compared with health volunteers, septal T1 was lower in AFD and higher in other diseases (AFD versus healthy volunteers versus other patients, 882±47, 968±32, 1018±74 milliseconds; P <0.0001). In patients with LVH (n=105), T1 discriminated completely between AFD and other diseases with no overlap. In AFD, T1 correlated inversely with wall thickness ( r =−0.51; P =0.0004) and was abnormal in 40% of subjects who did not have LVH. Segmentally, AFD showed pseudonormalization or elevation of T1 in the left ventricular inferolateral wall, correlating with the presence or absence of late gadolinium enhancement (1001±82 versus 891±38 milliseconds; P <0.0001).
Conclusions— Noncontrast T1 mapping shows potential as a unique and powerful measurement in the imaging assessment of LVH and AFD.read more
Citations
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2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy The Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology (ESC)
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Clinical recommendations for cardiovascular magnetic resonance mapping of T1, T2, T2 and extracellular volume: A consensus statement by the Society for Cardiovascular Magnetic Resonance (SCMR) endorsed by the European Association for Cardiovascular Imaging (EACVI)
Daniel Messroghli,James C. Moon,Vanessa M Ferreira,Lars Grosse-Wortmann,Taigang He,Peter Kellman,Julia Mascherbauer,Reza Nezafat,Michael Salerno,Erik B. Schelbert,Andrew J. Taylor,Richard B. Thompson,Martin Ugander,Ruud B. van Heeswijk,Matthias G. Friedrich +14 more
TL;DR: This document provides a summary of the existing evidence for the clinical value of parametric mapping in the heart as of mid 2017, and gives recommendations for practical use in different clinical scenarios for scientists, clinicians, and CMR manufacturers.
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Paul A. Heidenreich,Biykem Bozkurt,David Aguilar,Larry A. Allen,Joni J. Byun,Monica Colvin,Anita Deswal,Mark H. Drazner,Shannon M. Dunlay,Linda R. Evers,James C. Fang,Savitri Fedson,Gregg C. Fonarow,Salim S. Hayek,Adrian F. Hernandez,Prateeti Khazanie,Michelle M. Kittleson,Christopher S. Lee,Mark S. Link,Carmelo A. Milano,Lorraine C. Nnacheta,Alexander T. Sandhu,Lynne W. Stevenson,Orly Vardeny,Amanda R. Vest,Clyde W. Yancy +25 more
TL;DR: The "2022 AHA/ACC/HFSA Guideline for the Management of Heart Failure" as discussed by the authors provides patient-centric recommendations for clinicians to prevent, diagnose, and manage patients with heart failure.
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Myocardial T1 mapping and extracellular volume quantification: a Society for Cardiovascular Magnetic Resonance (SCMR) and CMR Working Group of the European Society of Cardiology consensus statement
James C. Moon,Daniel Messroghli,Peter Kellman,Stefan K. Piechnik,Matthew D. Robson,Martin Ugander,Peter D. Gatehouse,Andrew E. Arai,Matthias G. Friedrich,Stefan Neubauer,Jeanette Schulz-Menger,Jeanette Schulz-Menger,Erik B. Schelbert +12 more
TL;DR: This document provides recommendations for clinical and research T1 and ECV measurement, based on published evidence when available and expert consensus when not, and addresses controversies in the field.
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TL;DR: The technical aspects of key T1-mapping methods and imaging protocols are described and their limitations including the factors that influence their accuracy, precision, and reproducibility are described.
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