Cardiac amyloidosis without increased left ventricular wall thickness

Ga Yeon Lee, Kihyun Kim, Jin Oh Choi, Seok Jin Kim, Jung Sun Kim, Yeon Hyeon Choe, Martha Grogan, Eun Seok Jeon

Research output: Contribution to journalArticle

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Abstract

Objectives: To determine how often left ventricular wall thickness (LVWT) is normal and to assess the effect of LVWT on clinical outcomes of patients with immunoglobulin light chain (AL) cardiac amyloidosis. Patients and Methods: A total of 117 patients with systemic AL amyloidosis were retrospectively categorized from April 1, 1995, to September 15, 2012; group A included cardiac amyloidosis patients with an LVWT greater than 12 mm (45 patients); group B, cardiac amyloidosis patients with an LVWT of 12 mm or less (25 patients); and group C, no evidence of cardiac amyloidosis (47 patients). We compared echocardiographic parameters and survival rates among the 3 groups. Results: No differences were found between groups A and B in the following parameters: left ventricular ejection fraction (median, 56% [interquartile range (IQR), 46%-63%] vs 56% [IQR, 49%-63%], P=.76), left arterial volume index (median, 44.5 [IQR, 38.5-59.7] vs 43.9 [IQR, 33.8-57.1] mL/m2, P=.79), e′ (median, 0.04 [IQR, 0.03-0.05] vs 0.05 [IQR, 0.04-0.06] m/s, P=.10), and E/e′ (early diastolic mitral inflow velocity (E)/e′) (median, 18.4 [IQR, 12.0-23.3] vs 18.0 [IQR, 13.6-25.0], P=.98). Patients in group C exhibited significantly different values for these parameters (median, 65% [IQR, 61%-69%], 23.4 [IQR, 18.0-29.0] mL/m2, 0.08 [IQR, 0.06-0.09] m/s, and 8.8 [IQR, 7.2-10.5], respectively; all P<.001). The survival rates were statistically different, with median survival times of 422, 729, and 2080 days in groups A, B, and C, respectively (P=.002). Using multivariate Cox proportional hazards regression analysis, we found that age, an N-terminal proeB-type natriuretic peptide level of 1800 pg/mL or greater, E/e′, and complete hematologic remission were significant predictors of survival. Conclusions: A third of patients with AL cardiac amyloidosis were diagnosed as having an LVWT of 12 mm or less. Because appropriate therapy can improve the survival of patients with AL cardiac amyloidosis, early detection by sensitive diagnostic methods should be pursued even when LVWT is not increased.

Original languageEnglish (US)
Pages (from-to)781-789
Number of pages9
JournalMayo Clinic Proceedings
Volume89
Issue number6
DOIs
StatePublished - 2014

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Amyloidosis
Survival
Survival Rate
Immunoglobulin Light Chains
Natriuretic Peptides
Stroke Volume
Regression Analysis

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Lee, G. Y., Kim, K., Choi, J. O., Kim, S. J., Kim, J. S., Choe, Y. H., ... Jeon, E. S. (2014). Cardiac amyloidosis without increased left ventricular wall thickness. Mayo Clinic Proceedings, 89(6), 781-789. https://doi.org/10.1016/j.mayocp.2014.01.013

Cardiac amyloidosis without increased left ventricular wall thickness. / Lee, Ga Yeon; Kim, Kihyun; Choi, Jin Oh; Kim, Seok Jin; Kim, Jung Sun; Choe, Yeon Hyeon; Grogan, Martha; Jeon, Eun Seok.

In: Mayo Clinic Proceedings, Vol. 89, No. 6, 2014, p. 781-789.

Research output: Contribution to journalArticle

Lee, GY, Kim, K, Choi, JO, Kim, SJ, Kim, JS, Choe, YH, Grogan, M & Jeon, ES 2014, 'Cardiac amyloidosis without increased left ventricular wall thickness', Mayo Clinic Proceedings, vol. 89, no. 6, pp. 781-789. https://doi.org/10.1016/j.mayocp.2014.01.013
Lee, Ga Yeon ; Kim, Kihyun ; Choi, Jin Oh ; Kim, Seok Jin ; Kim, Jung Sun ; Choe, Yeon Hyeon ; Grogan, Martha ; Jeon, Eun Seok. / Cardiac amyloidosis without increased left ventricular wall thickness. In: Mayo Clinic Proceedings. 2014 ; Vol. 89, No. 6. pp. 781-789.
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abstract = "Objectives: To determine how often left ventricular wall thickness (LVWT) is normal and to assess the effect of LVWT on clinical outcomes of patients with immunoglobulin light chain (AL) cardiac amyloidosis. Patients and Methods: A total of 117 patients with systemic AL amyloidosis were retrospectively categorized from April 1, 1995, to September 15, 2012; group A included cardiac amyloidosis patients with an LVWT greater than 12 mm (45 patients); group B, cardiac amyloidosis patients with an LVWT of 12 mm or less (25 patients); and group C, no evidence of cardiac amyloidosis (47 patients). We compared echocardiographic parameters and survival rates among the 3 groups. Results: No differences were found between groups A and B in the following parameters: left ventricular ejection fraction (median, 56{\%} [interquartile range (IQR), 46{\%}-63{\%}] vs 56{\%} [IQR, 49{\%}-63{\%}], P=.76), left arterial volume index (median, 44.5 [IQR, 38.5-59.7] vs 43.9 [IQR, 33.8-57.1] mL/m2, P=.79), e′ (median, 0.04 [IQR, 0.03-0.05] vs 0.05 [IQR, 0.04-0.06] m/s, P=.10), and E/e′ (early diastolic mitral inflow velocity (E)/e′) (median, 18.4 [IQR, 12.0-23.3] vs 18.0 [IQR, 13.6-25.0], P=.98). Patients in group C exhibited significantly different values for these parameters (median, 65{\%} [IQR, 61{\%}-69{\%}], 23.4 [IQR, 18.0-29.0] mL/m2, 0.08 [IQR, 0.06-0.09] m/s, and 8.8 [IQR, 7.2-10.5], respectively; all P<.001). The survival rates were statistically different, with median survival times of 422, 729, and 2080 days in groups A, B, and C, respectively (P=.002). Using multivariate Cox proportional hazards regression analysis, we found that age, an N-terminal proeB-type natriuretic peptide level of 1800 pg/mL or greater, E/e′, and complete hematologic remission were significant predictors of survival. Conclusions: A third of patients with AL cardiac amyloidosis were diagnosed as having an LVWT of 12 mm or less. Because appropriate therapy can improve the survival of patients with AL cardiac amyloidosis, early detection by sensitive diagnostic methods should be pursued even when LVWT is not increased.",
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AU - Lee, Ga Yeon

AU - Kim, Kihyun

AU - Choi, Jin Oh

AU - Kim, Seok Jin

AU - Kim, Jung Sun

AU - Choe, Yeon Hyeon

AU - Grogan, Martha

AU - Jeon, Eun Seok

PY - 2014

Y1 - 2014

N2 - Objectives: To determine how often left ventricular wall thickness (LVWT) is normal and to assess the effect of LVWT on clinical outcomes of patients with immunoglobulin light chain (AL) cardiac amyloidosis. Patients and Methods: A total of 117 patients with systemic AL amyloidosis were retrospectively categorized from April 1, 1995, to September 15, 2012; group A included cardiac amyloidosis patients with an LVWT greater than 12 mm (45 patients); group B, cardiac amyloidosis patients with an LVWT of 12 mm or less (25 patients); and group C, no evidence of cardiac amyloidosis (47 patients). We compared echocardiographic parameters and survival rates among the 3 groups. Results: No differences were found between groups A and B in the following parameters: left ventricular ejection fraction (median, 56% [interquartile range (IQR), 46%-63%] vs 56% [IQR, 49%-63%], P=.76), left arterial volume index (median, 44.5 [IQR, 38.5-59.7] vs 43.9 [IQR, 33.8-57.1] mL/m2, P=.79), e′ (median, 0.04 [IQR, 0.03-0.05] vs 0.05 [IQR, 0.04-0.06] m/s, P=.10), and E/e′ (early diastolic mitral inflow velocity (E)/e′) (median, 18.4 [IQR, 12.0-23.3] vs 18.0 [IQR, 13.6-25.0], P=.98). Patients in group C exhibited significantly different values for these parameters (median, 65% [IQR, 61%-69%], 23.4 [IQR, 18.0-29.0] mL/m2, 0.08 [IQR, 0.06-0.09] m/s, and 8.8 [IQR, 7.2-10.5], respectively; all P<.001). The survival rates were statistically different, with median survival times of 422, 729, and 2080 days in groups A, B, and C, respectively (P=.002). Using multivariate Cox proportional hazards regression analysis, we found that age, an N-terminal proeB-type natriuretic peptide level of 1800 pg/mL or greater, E/e′, and complete hematologic remission were significant predictors of survival. Conclusions: A third of patients with AL cardiac amyloidosis were diagnosed as having an LVWT of 12 mm or less. Because appropriate therapy can improve the survival of patients with AL cardiac amyloidosis, early detection by sensitive diagnostic methods should be pursued even when LVWT is not increased.

AB - Objectives: To determine how often left ventricular wall thickness (LVWT) is normal and to assess the effect of LVWT on clinical outcomes of patients with immunoglobulin light chain (AL) cardiac amyloidosis. Patients and Methods: A total of 117 patients with systemic AL amyloidosis were retrospectively categorized from April 1, 1995, to September 15, 2012; group A included cardiac amyloidosis patients with an LVWT greater than 12 mm (45 patients); group B, cardiac amyloidosis patients with an LVWT of 12 mm or less (25 patients); and group C, no evidence of cardiac amyloidosis (47 patients). We compared echocardiographic parameters and survival rates among the 3 groups. Results: No differences were found between groups A and B in the following parameters: left ventricular ejection fraction (median, 56% [interquartile range (IQR), 46%-63%] vs 56% [IQR, 49%-63%], P=.76), left arterial volume index (median, 44.5 [IQR, 38.5-59.7] vs 43.9 [IQR, 33.8-57.1] mL/m2, P=.79), e′ (median, 0.04 [IQR, 0.03-0.05] vs 0.05 [IQR, 0.04-0.06] m/s, P=.10), and E/e′ (early diastolic mitral inflow velocity (E)/e′) (median, 18.4 [IQR, 12.0-23.3] vs 18.0 [IQR, 13.6-25.0], P=.98). Patients in group C exhibited significantly different values for these parameters (median, 65% [IQR, 61%-69%], 23.4 [IQR, 18.0-29.0] mL/m2, 0.08 [IQR, 0.06-0.09] m/s, and 8.8 [IQR, 7.2-10.5], respectively; all P<.001). The survival rates were statistically different, with median survival times of 422, 729, and 2080 days in groups A, B, and C, respectively (P=.002). Using multivariate Cox proportional hazards regression analysis, we found that age, an N-terminal proeB-type natriuretic peptide level of 1800 pg/mL or greater, E/e′, and complete hematologic remission were significant predictors of survival. Conclusions: A third of patients with AL cardiac amyloidosis were diagnosed as having an LVWT of 12 mm or less. Because appropriate therapy can improve the survival of patients with AL cardiac amyloidosis, early detection by sensitive diagnostic methods should be pursued even when LVWT is not increased.

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