Comparison of different techniques to identify cardiac involvement in immunoglobulin light chain (AL) amyloidosis

Mohammed A. Aljama, M. Hasib Sidiqi, Angela Dispenzieri, Morie Gertz, Martha Lacy, Francis K. Buadi, David M Dingli, Eli Muchtar, Amie L. Fonder, Suzanne R. Hayman, Miriam A. Hobbs, Wilson Gonsalves, Rahma M. Warsame, Taxiarchis Kourelis, Yi Lisa Hwa, Prashant Kapoor, Nelson Leung, Ronald S. Go, Robert A. Kyle, S Vincent Rajkumar & 1 others Shaji K Kumar

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Abstract

We retrospectively reviewed the utility of N-terminal prohormone of brain natriuretic peptide (NT-proBNP) and transthoracic echocardiogram (TTE) in diagnosing cardiac involvement in patients with biopsy-proven systemic immunoglobulin light chain amyloidosis seen at the Mayo Clinic between 1 January 2006 and 30 December 2015. We analyzed 2 cohorts: patients undergoing endomyocardial biopsy for suspicion of cardiac involvement (cohort 1) and patients who had serum NT-proBNP and comprehensive echocardiographic evaluation at diagnosis (cohort 2). Of 179 patients undergoing endomyocardial biopsy (cohort 1), 173 (97%) had evidence of amyloid deposition, with 159 having NT-proBNP performed at the time of the procedure. The NT-proBNP was elevated (>300 pg/mL) in all 159 patients (sensitivity, 100%; median NT-proBNP, 4917 pg/mL; range, 355-69 541). The left ventricular ejection fraction, interventricular septal thickness, and strain rate were abnormal in 89/168 (53%), 102/64 (61%) and 92/95 (97%), respectively. Among cohort 2 (n = 342), 259 (76%) had an elevated NT-proBNP, of whom 237 (92%) had an abnormality detected on TTE. Of 83 patients with normal NT-proBNP <300 pg/mL, 27 (33%) had an abnormality on TTE (all with borderline strain rate 218% to 215%). Only 5/27 patients were considered to have possible early cardiac involvement and none had any other diagnostic or classical features of amyloidosis on TTE. The combination of NT-proBNP and comprehensive echocardiographic evaluation can diagnose cardiac amyloidosis negating the need for endomyocardial biopsy. A negative NT-proBNP rules out clinically meaningful cardiac involvement and may obviate the routine use of TTE in patients with a low clinical suspicion of cardiac amyloidosis.

Original languageEnglish (US)
Pages (from-to)1226-1229
Number of pages4
JournalBlood Advances
Volume3
Issue number8
DOIs
StatePublished - Jan 1 2019

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Immunoglobulin Light Chains
Brain Natriuretic Peptide
Amyloidosis
Biopsy
Amyloid
Stroke Volume

ASJC Scopus subject areas

  • Medicine(all)

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Comparison of different techniques to identify cardiac involvement in immunoglobulin light chain (AL) amyloidosis. / Aljama, Mohammed A.; Sidiqi, M. Hasib; Dispenzieri, Angela; Gertz, Morie; Lacy, Martha; Buadi, Francis K.; Dingli, David M; Muchtar, Eli; Fonder, Amie L.; Hayman, Suzanne R.; Hobbs, Miriam A.; Gonsalves, Wilson; Warsame, Rahma M.; Kourelis, Taxiarchis; Hwa, Yi Lisa; Kapoor, Prashant; Leung, Nelson; Go, Ronald S.; Kyle, Robert A.; Rajkumar, S Vincent; Kumar, Shaji K.

In: Blood Advances, Vol. 3, No. 8, 01.01.2019, p. 1226-1229.

Research output: Contribution to journalArticle

Aljama, Mohammed A. ; Sidiqi, M. Hasib ; Dispenzieri, Angela ; Gertz, Morie ; Lacy, Martha ; Buadi, Francis K. ; Dingli, David M ; Muchtar, Eli ; Fonder, Amie L. ; Hayman, Suzanne R. ; Hobbs, Miriam A. ; Gonsalves, Wilson ; Warsame, Rahma M. ; Kourelis, Taxiarchis ; Hwa, Yi Lisa ; Kapoor, Prashant ; Leung, Nelson ; Go, Ronald S. ; Kyle, Robert A. ; Rajkumar, S Vincent ; Kumar, Shaji K. / Comparison of different techniques to identify cardiac involvement in immunoglobulin light chain (AL) amyloidosis. In: Blood Advances. 2019 ; Vol. 3, No. 8. pp. 1226-1229.
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abstract = "We retrospectively reviewed the utility of N-terminal prohormone of brain natriuretic peptide (NT-proBNP) and transthoracic echocardiogram (TTE) in diagnosing cardiac involvement in patients with biopsy-proven systemic immunoglobulin light chain amyloidosis seen at the Mayo Clinic between 1 January 2006 and 30 December 2015. We analyzed 2 cohorts: patients undergoing endomyocardial biopsy for suspicion of cardiac involvement (cohort 1) and patients who had serum NT-proBNP and comprehensive echocardiographic evaluation at diagnosis (cohort 2). Of 179 patients undergoing endomyocardial biopsy (cohort 1), 173 (97{\%}) had evidence of amyloid deposition, with 159 having NT-proBNP performed at the time of the procedure. The NT-proBNP was elevated (>300 pg/mL) in all 159 patients (sensitivity, 100{\%}; median NT-proBNP, 4917 pg/mL; range, 355-69 541). The left ventricular ejection fraction, interventricular septal thickness, and strain rate were abnormal in 89/168 (53{\%}), 102/64 (61{\%}) and 92/95 (97{\%}), respectively. Among cohort 2 (n = 342), 259 (76{\%}) had an elevated NT-proBNP, of whom 237 (92{\%}) had an abnormality detected on TTE. Of 83 patients with normal NT-proBNP <300 pg/mL, 27 (33{\%}) had an abnormality on TTE (all with borderline strain rate 218{\%} to 215{\%}). Only 5/27 patients were considered to have possible early cardiac involvement and none had any other diagnostic or classical features of amyloidosis on TTE. The combination of NT-proBNP and comprehensive echocardiographic evaluation can diagnose cardiac amyloidosis negating the need for endomyocardial biopsy. A negative NT-proBNP rules out clinically meaningful cardiac involvement and may obviate the routine use of TTE in patients with a low clinical suspicion of cardiac amyloidosis.",
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AU - Aljama, Mohammed A.

AU - Sidiqi, M. Hasib

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AU - Gertz, Morie

AU - Lacy, Martha

AU - Buadi, Francis K.

AU - Dingli, David M

AU - Muchtar, Eli

AU - Fonder, Amie L.

AU - Hayman, Suzanne R.

AU - Hobbs, Miriam A.

AU - Gonsalves, Wilson

AU - Warsame, Rahma M.

AU - Kourelis, Taxiarchis

AU - Hwa, Yi Lisa

AU - Kapoor, Prashant

AU - Leung, Nelson

AU - Go, Ronald S.

AU - Kyle, Robert A.

AU - Rajkumar, S Vincent

AU - Kumar, Shaji K

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