Prognostic Value of Late Gadolinium Enhancement Cardiovascular Magnetic Resonance in Cardiac Amyloidosis

Marianna Fontana; Silvia Pica; Patricia Reant; Amna Abdel-Gadir; Thomas A Treibel; Sanjay M Banypersad; Viviana Maestrini; William Barcella; Stefania Rosmini; Heerajnarain Bulluck; Rabya H Sayed; Ketna Patel; Shameem Mamhood; Chiara Bucciarelli-Ducci; Carol J Whelan; Anna S Herrey; Helen J Lachmann; Ashutosh D Wechalekar; Charlotte H Manisty; Eric B Schelbert; Peter Kellman; Julian D Gillmore; Philip N Hawkins; James C Moon

doi:10.1161/CIRCULATIONAHA.115.016567

Prognostic Value of Late Gadolinium Enhancement Cardiovascular Magnetic Resonance in Cardiac Amyloidosis

Circulation. 2015 Oct 20;132(16):1570-9. doi: 10.1161/CIRCULATIONAHA.115.016567. Epub 2015 Sep 11.

Authors

Marianna Fontana¹, Silvia Pica¹, Patricia Reant¹, Amna Abdel-Gadir¹, Thomas A Treibel¹, Sanjay M Banypersad¹, Viviana Maestrini¹, William Barcella¹, Stefania Rosmini¹, Heerajnarain Bulluck¹, Rabya H Sayed¹, Ketna Patel¹, Shameem Mamhood¹, Chiara Bucciarelli-Ducci¹, Carol J Whelan¹, Anna S Herrey¹, Helen J Lachmann¹, Ashutosh D Wechalekar¹, Charlotte H Manisty¹, Eric B Schelbert¹, Peter Kellman¹, Julian D Gillmore¹, Philip N Hawkins¹, James C Moon²

Affiliations

¹ 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.).
² 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.

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.

Methods and 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.

Keywords: amyloidosis; cardiac imaging techniques; magnetic resonance imaging; prognosis.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Aged
Amyloidosis / diagnosis*
Cardiomyopathies / diagnosis*
Female
Gadolinium
Humans
Image Enhancement
Magnetic Resonance Imaging / methods*
Male
Middle Aged
Myocardium / pathology*
Prognosis

Substances

Gadolinium

Abstract

Publication types

MeSH terms

Substances

Grants and funding