title: Prognostic Value of Late Gadolinium Enhancement Cardiovascular Magnetic Resonance in Cardiac Amyloidosis.
abstract: BACKGROUND
  The prognosis and treatment of the 2 main types of cardiac amyloidosis, immunoglobulin light chain (AL) and transthyretin (ATTR) amyloidosis, are substantially influenced by cardiac involvement. Cardiovascular magnetic resonance with late gadolinium enhancement (LGE) is a reference standard for the diagnosis of cardiac amyloidosis, but its potential for stratifying risk is unknown.
  
  
  RESULTS
  Two hundred fifty prospectively recruited subjects, 122 patients with ATTR amyloid, 9 asymptomatic mutation carriers, and 119 patients with AL amyloidosis, underwent LGE cardiovascular magnetic resonance. Subjects were followed up for a mean of 24±13 months. LGE was performed with phase-sensitive inversion recovery (PSIR) and without (magnitude only). These were compared with extracellular volume measured with T1 mapping. PSIR was superior to magnitude-only inversion recovery LGE because PSIR always nulled the tissue (blood or myocardium) with the longest T1 (least gadolinium). LGE was classified into 3 patterns: none, subendocardial, and transmural, which were associated with increasing amyloid burden as defined by extracellular volume (P<0.0001), with transitions from none to subendocardial LGE at an extracellular volume of 0.40 to 0.43 (AL) and 0.39 to 0.40 (ATTR) and to transmural at 0.48 to 0.55 (AL) and 0.47 to 0.59 (ATTR). Sixty-seven patients (27%) died. Transmural LGE predicted death (hazard ratio, 5.4; 95% confidence interval, 2.1-13.7; P<0.0001) and remained independent after adjustment for N-terminal pro-brain natriuretic peptide, ejection fraction, stroke volume index, E/E', and left ventricular mass index (hazard ratio, 4.1; 95% confidence interval, 1.3-13.1; P<0.05).
  
  
  CONCLUSIONS
  There is a continuum of cardiac involvement in systemic AL and ATTR amyloidosis. Transmural LGE is determined reliably by PSIR and represents advanced cardiac amyloidosis. The PSIR technique provides incremental information on outcome even after adjustment for known prognostic factors.
authors.affiliation: From Heart Hospital, London, UK (M.F., S.P., P.R., A.A.-G., T.A.T., S.M.B., V.M., S.R., H.B., A.S.H., C.H.M., J.C.M.); Institute of Cardiovascular Science (M.F., A.A.-G., T.A.T., S.M.B., H.B., J.C.M.) and Department of Statistical Science (W.B.), University College London, UK; National Amyloidosis Centre, University College London, Royal Free Hospital, London, UK (M.F., S.M.B., R.H.S., K.P., S.M., C.J.W., H.J.L., A.D.W., J.D.G., P.N.H.); Bristol Heart Institute, University of Bristol, UK (C.B.-D.); University of Pittsburgh School of Medicine, PA (E.B.S.); and National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD (P.K.).
authors.forename: Marianna
authors.initials: M
authors.lastname: Fontana
authors.affiliation: From Heart Hospital, London, UK (M.F., S.P., P.R., A.A.-G., T.A.T., S.M.B., V.M., S.R., H.B., A.S.H., C.H.M., J.C.M.); Institute of Cardiovascular Science (M.F., A.A.-G., T.A.T., S.M.B., H.B., J.C.M.) and Department of Statistical Science (W.B.), University College London, UK; National Amyloidosis Centre, University College London, Royal Free Hospital, London, UK (M.F., S.M.B., R.H.S., K.P., S.M., C.J.W., H.J.L., A.D.W., J.D.G., P.N.H.); Bristol Heart Institute, University of Bristol, UK (C.B.-D.); University of Pittsburgh School of Medicine, PA (E.B.S.); and National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD (P.K.).
authors.forename: Silvia
authors.initials: S
authors.lastname: Pica
authors.affiliation: From Heart Hospital, London, UK (M.F., S.P., P.R., A.A.-G., T.A.T., S.M.B., V.M., S.R., H.B., A.S.H., C.H.M., J.C.M.); Institute of Cardiovascular Science (M.F., A.A.-G., T.A.T., S.M.B., H.B., J.C.M.) and Department of Statistical Science (W.B.), University College London, UK; National Amyloidosis Centre, University College London, Royal Free Hospital, London, UK (M.F., S.M.B., R.H.S., K.P., S.M., C.J.W., H.J.L., A.D.W., J.D.G., P.N.H.); Bristol Heart Institute, University of Bristol, UK (C.B.-D.); University of Pittsburgh School of Medicine, PA (E.B.S.); and National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD (P.K.).
authors.forename: Patricia
authors.initials: P
authors.lastname: Reant
authors.affiliation: From Heart Hospital, London, UK (M.F., S.P., P.R., A.A.-G., T.A.T., S.M.B., V.M., S.R., H.B., A.S.H., C.H.M., J.C.M.); Institute of Cardiovascular Science (M.F., A.A.-G., T.A.T., S.M.B., H.B., J.C.M.) and Department of Statistical Science (W.B.), University College London, UK; National Amyloidosis Centre, University College London, Royal Free Hospital, London, UK (M.F., S.M.B., R.H.S., K.P., S.M., C.J.W., H.J.L., A.D.W., J.D.G., P.N.H.); Bristol Heart Institute, University of Bristol, UK (C.B.-D.); University of Pittsburgh School of Medicine, PA (E.B.S.); and National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD (P.K.).
authors.forename: Amna
authors.initials: A
authors.lastname: Abdel-Gadir
authors.affiliation: From Heart Hospital, London, UK (M.F., S.P., P.R., A.A.-G., T.A.T., S.M.B., V.M., S.R., H.B., A.S.H., C.H.M., J.C.M.); Institute of Cardiovascular Science (M.F., A.A.-G., T.A.T., S.M.B., H.B., J.C.M.) and Department of Statistical Science (W.B.), University College London, UK; National Amyloidosis Centre, University College London, Royal Free Hospital, London, UK (M.F., S.M.B., R.H.S., K.P., S.M., C.J.W., H.J.L., A.D.W., J.D.G., P.N.H.); Bristol Heart Institute, University of Bristol, UK (C.B.-D.); University of Pittsburgh School of Medicine, PA (E.B.S.); and National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD (P.K.).
authors.forename: Thomas A
authors.initials: TA
authors.lastname: Treibel
authors.affiliation: From Heart Hospital, London, UK (M.F., S.P., P.R., A.A.-G., T.A.T., S.M.B., V.M., S.R., H.B., A.S.H., C.H.M., J.C.M.); Institute of Cardiovascular Science (M.F., A.A.-G., T.A.T., S.M.B., H.B., J.C.M.) and Department of Statistical Science (W.B.), University College London, UK; National Amyloidosis Centre, University College London, Royal Free Hospital, London, UK (M.F., S.M.B., R.H.S., K.P., S.M., C.J.W., H.J.L., A.D.W., J.D.G., P.N.H.); Bristol Heart Institute, University of Bristol, UK (C.B.-D.); University of Pittsburgh School of Medicine, PA (E.B.S.); and National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD (P.K.).
authors.forename: Sanjay M
authors.initials: SM
authors.lastname: Banypersad
authors.affiliation: From Heart Hospital, London, UK (M.F., S.P., P.R., A.A.-G., T.A.T., S.M.B., V.M., S.R., H.B., A.S.H., C.H.M., J.C.M.); Institute of Cardiovascular Science (M.F., A.A.-G., T.A.T., S.M.B., H.B., J.C.M.) and Department of Statistical Science (W.B.), University College London, UK; National Amyloidosis Centre, University College London, Royal Free Hospital, London, UK (M.F., S.M.B., R.H.S., K.P., S.M., C.J.W., H.J.L., A.D.W., J.D.G., P.N.H.); Bristol Heart Institute, University of Bristol, UK (C.B.-D.); University of Pittsburgh School of Medicine, PA (E.B.S.); and National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD (P.K.).
authors.forename: Viviana
authors.initials: V
authors.lastname: Maestrini
authors.affiliation: From Heart Hospital, London, UK (M.F., S.P., P.R., A.A.-G., T.A.T., S.M.B., V.M., S.R., H.B., A.S.H., C.H.M., J.C.M.); Institute of Cardiovascular Science (M.F., A.A.-G., T.A.T., S.M.B., H.B., J.C.M.) and Department of Statistical Science (W.B.), University College London, UK; National Amyloidosis Centre, University College London, Royal Free Hospital, London, UK (M.F., S.M.B., R.H.S., K.P., S.M., C.J.W., H.J.L., A.D.W., J.D.G., P.N.H.); Bristol Heart Institute, University of Bristol, UK (C.B.-D.); University of Pittsburgh School of Medicine, PA (E.B.S.); and National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD (P.K.).
authors.forename: William
authors.initials: W
authors.lastname: Barcella
authors.affiliation: From Heart Hospital, London, UK (M.F., S.P., P.R., A.A.-G., T.A.T., S.M.B., V.M., S.R., H.B., A.S.H., C.H.M., J.C.M.); Institute of Cardiovascular Science (M.F., A.A.-G., T.A.T., S.M.B., H.B., J.C.M.) and Department of Statistical Science (W.B.), University College London, UK; National Amyloidosis Centre, University College London, Royal Free Hospital, London, UK (M.F., S.M.B., R.H.S., K.P., S.M., C.J.W., H.J.L., A.D.W., J.D.G., P.N.H.); Bristol Heart Institute, University of Bristol, UK (C.B.-D.); University of Pittsburgh School of Medicine, PA (E.B.S.); and National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD (P.K.).
authors.forename: Stefania
authors.initials: S
authors.lastname: Rosmini
authors.affiliation: From Heart Hospital, London, UK (M.F., S.P., P.R., A.A.-G., T.A.T., S.M.B., V.M., S.R., H.B., A.S.H., C.H.M., J.C.M.); Institute of Cardiovascular Science (M.F., A.A.-G., T.A.T., S.M.B., H.B., J.C.M.) and Department of Statistical Science (W.B.), University College London, UK; National Amyloidosis Centre, University College London, Royal Free Hospital, London, UK (M.F., S.M.B., R.H.S., K.P., S.M., C.J.W., H.J.L., A.D.W., J.D.G., P.N.H.); Bristol Heart Institute, University of Bristol, UK (C.B.-D.); University of Pittsburgh School of Medicine, PA (E.B.S.); and National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD (P.K.).
authors.forename: Heerajnarain
authors.initials: H
authors.lastname: Bulluck
authors.affiliation: From Heart Hospital, London, UK (M.F., S.P., P.R., A.A.-G., T.A.T., S.M.B., V.M., S.R., H.B., A.S.H., C.H.M., J.C.M.); Institute of Cardiovascular Science (M.F., A.A.-G., T.A.T., S.M.B., H.B., J.C.M.) and Department of Statistical Science (W.B.), University College London, UK; National Amyloidosis Centre, University College London, Royal Free Hospital, London, UK (M.F., S.M.B., R.H.S., K.P., S.M., C.J.W., H.J.L., A.D.W., J.D.G., P.N.H.); Bristol Heart Institute, University of Bristol, UK (C.B.-D.); University of Pittsburgh School of Medicine, PA (E.B.S.); and National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD (P.K.).
authors.forename: Rabya H
authors.initials: RH
authors.lastname: Sayed
authors.affiliation: From Heart Hospital, London, UK (M.F., S.P., P.R., A.A.-G., T.A.T., S.M.B., V.M., S.R., H.B., A.S.H., C.H.M., J.C.M.); Institute of Cardiovascular Science (M.F., A.A.-G., T.A.T., S.M.B., H.B., J.C.M.) and Department of Statistical Science (W.B.), University College London, UK; National Amyloidosis Centre, University College London, Royal Free Hospital, London, UK (M.F., S.M.B., R.H.S., K.P., S.M., C.J.W., H.J.L., A.D.W., J.D.G., P.N.H.); Bristol Heart Institute, University of Bristol, UK (C.B.-D.); University of Pittsburgh School of Medicine, PA (E.B.S.); and National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD (P.K.).
authors.forename: Ketna
authors.initials: K
authors.lastname: Patel
authors.affiliation: From Heart Hospital, London, UK (M.F., S.P., P.R., A.A.-G., T.A.T., S.M.B., V.M., S.R., H.B., A.S.H., C.H.M., J.C.M.); Institute of Cardiovascular Science (M.F., A.A.-G., T.A.T., S.M.B., H.B., J.C.M.) and Department of Statistical Science (W.B.), University College London, UK; National Amyloidosis Centre, University College London, Royal Free Hospital, London, UK (M.F., S.M.B., R.H.S., K.P., S.M., C.J.W., H.J.L., A.D.W., J.D.G., P.N.H.); Bristol Heart Institute, University of Bristol, UK (C.B.-D.); University of Pittsburgh School of Medicine, PA (E.B.S.); and National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD (P.K.).
authors.forename: Shameem
authors.initials: S
authors.lastname: Mamhood
authors.affiliation: From Heart Hospital, London, UK (M.F., S.P., P.R., A.A.-G., T.A.T., S.M.B., V.M., S.R., H.B., A.S.H., C.H.M., J.C.M.); Institute of Cardiovascular Science (M.F., A.A.-G., T.A.T., S.M.B., H.B., J.C.M.) and Department of Statistical Science (W.B.), University College London, UK; National Amyloidosis Centre, University College London, Royal Free Hospital, London, UK (M.F., S.M.B., R.H.S., K.P., S.M., C.J.W., H.J.L., A.D.W., J.D.G., P.N.H.); Bristol Heart Institute, University of Bristol, UK (C.B.-D.); University of Pittsburgh School of Medicine, PA (E.B.S.); and National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD (P.K.).
authors.forename: Chiara
authors.initials: C
authors.lastname: Bucciarelli-Ducci
authors.affiliation: From Heart Hospital, London, UK (M.F., S.P., P.R., A.A.-G., T.A.T., S.M.B., V.M., S.R., H.B., A.S.H., C.H.M., J.C.M.); Institute of Cardiovascular Science (M.F., A.A.-G., T.A.T., S.M.B., H.B., J.C.M.) and Department of Statistical Science (W.B.), University College London, UK; National Amyloidosis Centre, University College London, Royal Free Hospital, London, UK (M.F., S.M.B., R.H.S., K.P., S.M., C.J.W., H.J.L., A.D.W., J.D.G., P.N.H.); Bristol Heart Institute, University of Bristol, UK (C.B.-D.); University of Pittsburgh School of Medicine, PA (E.B.S.); and National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD (P.K.).
authors.forename: Carol J
authors.initials: CJ
authors.lastname: Whelan
authors.affiliation: From Heart Hospital, London, UK (M.F., S.P., P.R., A.A.-G., T.A.T., S.M.B., V.M., S.R., H.B., A.S.H., C.H.M., J.C.M.); Institute of Cardiovascular Science (M.F., A.A.-G., T.A.T., S.M.B., H.B., J.C.M.) and Department of Statistical Science (W.B.), University College London, UK; National Amyloidosis Centre, University College London, Royal Free Hospital, London, UK (M.F., S.M.B., R.H.S., K.P., S.M., C.J.W., H.J.L., A.D.W., J.D.G., P.N.H.); Bristol Heart Institute, University of Bristol, UK (C.B.-D.); University of Pittsburgh School of Medicine, PA (E.B.S.); and National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD (P.K.).
authors.forename: Anna S
authors.initials: AS
authors.lastname: Herrey
authors.affiliation: From Heart Hospital, London, UK (M.F., S.P., P.R., A.A.-G., T.A.T., S.M.B., V.M., S.R., H.B., A.S.H., C.H.M., J.C.M.); Institute of Cardiovascular Science (M.F., A.A.-G., T.A.T., S.M.B., H.B., J.C.M.) and Department of Statistical Science (W.B.), University College London, UK; National Amyloidosis Centre, University College London, Royal Free Hospital, London, UK (M.F., S.M.B., R.H.S., K.P., S.M., C.J.W., H.J.L., A.D.W., J.D.G., P.N.H.); Bristol Heart Institute, University of Bristol, UK (C.B.-D.); University of Pittsburgh School of Medicine, PA (E.B.S.); and National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD (P.K.).
authors.forename: Helen J
authors.initials: HJ
authors.lastname: Lachmann
authors.affiliation: From Heart Hospital, London, UK (M.F., S.P., P.R., A.A.-G., T.A.T., S.M.B., V.M., S.R., H.B., A.S.H., C.H.M., J.C.M.); Institute of Cardiovascular Science (M.F., A.A.-G., T.A.T., S.M.B., H.B., J.C.M.) and Department of Statistical Science (W.B.), University College London, UK; National Amyloidosis Centre, University College London, Royal Free Hospital, London, UK (M.F., S.M.B., R.H.S., K.P., S.M., C.J.W., H.J.L., A.D.W., J.D.G., P.N.H.); Bristol Heart Institute, University of Bristol, UK (C.B.-D.); University of Pittsburgh School of Medicine, PA (E.B.S.); and National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD (P.K.).
authors.forename: Ashutosh D
authors.initials: AD
authors.lastname: Wechalekar
authors.affiliation: From Heart Hospital, London, UK (M.F., S.P., P.R., A.A.-G., T.A.T., S.M.B., V.M., S.R., H.B., A.S.H., C.H.M., J.C.M.); Institute of Cardiovascular Science (M.F., A.A.-G., T.A.T., S.M.B., H.B., J.C.M.) and Department of Statistical Science (W.B.), University College London, UK; National Amyloidosis Centre, University College London, Royal Free Hospital, London, UK (M.F., S.M.B., R.H.S., K.P., S.M., C.J.W., H.J.L., A.D.W., J.D.G., P.N.H.); Bristol Heart Institute, University of Bristol, UK (C.B.-D.); University of Pittsburgh School of Medicine, PA (E.B.S.); and National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD (P.K.).
authors.forename: Charlotte H
authors.initials: CH
authors.lastname: Manisty
authors.affiliation: From Heart Hospital, London, UK (M.F., S.P., P.R., A.A.-G., T.A.T., S.M.B., V.M., S.R., H.B., A.S.H., C.H.M., J.C.M.); Institute of Cardiovascular Science (M.F., A.A.-G., T.A.T., S.M.B., H.B., J.C.M.) and Department of Statistical Science (W.B.), University College London, UK; National Amyloidosis Centre, University College London, Royal Free Hospital, London, UK (M.F., S.M.B., R.H.S., K.P., S.M., C.J.W., H.J.L., A.D.W., J.D.G., P.N.H.); Bristol Heart Institute, University of Bristol, UK (C.B.-D.); University of Pittsburgh School of Medicine, PA (E.B.S.); and National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD (P.K.).
authors.forename: Eric B
authors.initials: EB
authors.lastname: Schelbert
authors.affiliation: From Heart Hospital, London, UK (M.F., S.P., P.R., A.A.-G., T.A.T., S.M.B., V.M., S.R., H.B., A.S.H., C.H.M., J.C.M.); Institute of Cardiovascular Science (M.F., A.A.-G., T.A.T., S.M.B., H.B., J.C.M.) and Department of Statistical Science (W.B.), University College London, UK; National Amyloidosis Centre, University College London, Royal Free Hospital, London, UK (M.F., S.M.B., R.H.S., K.P., S.M., C.J.W., H.J.L., A.D.W., J.D.G., P.N.H.); Bristol Heart Institute, University of Bristol, UK (C.B.-D.); University of Pittsburgh School of Medicine, PA (E.B.S.); and National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD (P.K.).
authors.forename: Peter
authors.initials: P
authors.lastname: Kellman
authors.affiliation: From Heart Hospital, London, UK (M.F., S.P., P.R., A.A.-G., T.A.T., S.M.B., V.M., S.R., H.B., A.S.H., C.H.M., J.C.M.); Institute of Cardiovascular Science (M.F., A.A.-G., T.A.T., S.M.B., H.B., J.C.M.) and Department of Statistical Science (W.B.), University College London, UK; National Amyloidosis Centre, University College London, Royal Free Hospital, London, UK (M.F., S.M.B., R.H.S., K.P., S.M., C.J.W., H.J.L., A.D.W., J.D.G., P.N.H.); Bristol Heart Institute, University of Bristol, UK (C.B.-D.); University of Pittsburgh School of Medicine, PA (E.B.S.); and National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD (P.K.).
authors.forename: Julian D
authors.initials: JD
authors.lastname: Gillmore
authors.affiliation: From Heart Hospital, London, UK (M.F., S.P., P.R., A.A.-G., T.A.T., S.M.B., V.M., S.R., H.B., A.S.H., C.H.M., J.C.M.); Institute of Cardiovascular Science (M.F., A.A.-G., T.A.T., S.M.B., H.B., J.C.M.) and Department of Statistical Science (W.B.), University College London, UK; National Amyloidosis Centre, University College London, Royal Free Hospital, London, UK (M.F., S.M.B., R.H.S., K.P., S.M., C.J.W., H.J.L., A.D.W., J.D.G., P.N.H.); Bristol Heart Institute, University of Bristol, UK (C.B.-D.); University of Pittsburgh School of Medicine, PA (E.B.S.); and National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD (P.K.).
authors.forename: Philip N
authors.initials: PN
authors.lastname: Hawkins
authors.affiliation: From Heart Hospital, London, UK (M.F., S.P., P.R., A.A.-G., T.A.T., S.M.B., V.M., S.R., H.B., A.S.H., C.H.M., J.C.M.); Institute of Cardiovascular Science (M.F., A.A.-G., T.A.T., S.M.B., H.B., J.C.M.) and Department of Statistical Science (W.B.), University College London, UK; National Amyloidosis Centre, University College London, Royal Free Hospital, London, UK (M.F., S.M.B., R.H.S., K.P., S.M., C.J.W., H.J.L., A.D.W., J.D.G., P.N.H.); Bristol Heart Institute, University of Bristol, UK (C.B.-D.); University of Pittsburgh School of Medicine, PA (E.B.S.); and National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD (P.K.). j.moon@ucl.ac.uk.
authors.forename: James C
authors.initials: JC
authors.lastname: Moon
mesh_terms: D000368:Aged; D000686:Amyloidosis / Q000175:diagnosis*; D009202:Cardiomyopathies / Q000175:diagnosis*; D005260:Female; D005682:Gadolinium; D006801:Humans; D007089:Image Enhancement; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D008875:Middle Aged; D009206:Myocardium / Q000473:pathology*; D011379:Prognosis
chemical_list: D005682:Gadolinium
keywords: amyloidosis; cardiac imaging techniques; magnetic resonance imaging; prognosis