Left Atrial structure and function in hypertrophic cardiomyopathy sarcomere mutation carriers with and without left ventricular hypertrophy

Hoshang Farhad, Sara B. Seidelmann, Davis Vigneault, Siddique A. Abbasi, Eunice Yang, Sharlene M. Day, Steven D. Colan, Mark W. Russell, Jeffrey Towbin, Mark V. Sherrid, Charles E. Canter, Ling Shi, Michael Jerosch-Herold, David A. Bluemke, Carolyn Ho, Tomas G. Neilan

Research output: Contribution to journalArticle

6 Citations (Scopus)

Abstract

Background: Impaired left atrial (LA) function is an early marker of cardiac dysfunction and predictor of adverse cardiac events. Herein, we assess LA structure and function in hypertrophy in hypertrophic cardiomyopathy (HCM) sarcomere mutation carriers with and without left ventricular hypertrophy (LVH). Method: Seventy-three participants of the HCMNet study who underwent cardiovascular magnetic resonance (CMR) imaging were studied, including mutation carriers with overt HCM (n = 34), preclinical mutation carriers without HCM (n = 24) and healthy, familial controls (n = 15). Results: LA volumes were similar between preclinical, control and overt HCM cohorts after covariate adjustment. However, there was evidence of impaired LA function with decreased LA total emptying function in both preclinical (64 ± 8%) and overt HCM (59 ± 10%), compared with controls (70 ± 7%; p = 0.002 and p = 0.005, respectively). LA passive emptying function was also decreased in overt HCM (35 ± 11%) compared with controls (47 ± 10%; p = 0.006). Both LAtotal emptying function and LA passive emptying function were inversely correlated with the extent of late gadolinium enhancement (LGE; p = 0.005 and p < 0.05, respectively), LV mass (p = 0.02 and p < 0.001) and interventricular septal thickness (p < 0.001 for both) and serum NT-proBNP levels (p < 0.001 for both). Conclusion: LA dysfunction is detectable by CMR in preclinical HCM mutation carriers despite non-distinguishable LV wall thickness and LA volume. LA function appears most impaired in subjects with overt HCM and a greater extent of LV fibrosis.

Original languageEnglish (US)
Article number107
JournalJournal of Cardiovascular Magnetic Resonance
Volume19
Issue number1
DOIs
StatePublished - Dec 28 2017

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Left Atrial Function
Sarcomeres
Hypertrophic Cardiomyopathy
Left Ventricular Hypertrophy
Mutation
Gadolinium
Hypertrophy
Fibrosis
Magnetic Resonance Spectroscopy
Magnetic Resonance Imaging

All Science Journal Classification (ASJC) codes

  • Radiological and Ultrasound Technology
  • Radiology Nuclear Medicine and imaging
  • Cardiology and Cardiovascular Medicine

Cite this

Left Atrial structure and function in hypertrophic cardiomyopathy sarcomere mutation carriers with and without left ventricular hypertrophy. / Farhad, Hoshang; Seidelmann, Sara B.; Vigneault, Davis; Abbasi, Siddique A.; Yang, Eunice; Day, Sharlene M.; Colan, Steven D.; Russell, Mark W.; Towbin, Jeffrey; Sherrid, Mark V.; Canter, Charles E.; Shi, Ling; Jerosch-Herold, Michael; Bluemke, David A.; Ho, Carolyn; Neilan, Tomas G.

In: Journal of Cardiovascular Magnetic Resonance, Vol. 19, No. 1, 107, 28.12.2017.

Research output: Contribution to journalArticle

Farhad, H, Seidelmann, SB, Vigneault, D, Abbasi, SA, Yang, E, Day, SM, Colan, SD, Russell, MW, Towbin, J, Sherrid, MV, Canter, CE, Shi, L, Jerosch-Herold, M, Bluemke, DA, Ho, C & Neilan, TG 2017, 'Left Atrial structure and function in hypertrophic cardiomyopathy sarcomere mutation carriers with and without left ventricular hypertrophy', Journal of Cardiovascular Magnetic Resonance, vol. 19, no. 1, 107. https://doi.org/10.1186/s12968-017-0420-0
Farhad, Hoshang ; Seidelmann, Sara B. ; Vigneault, Davis ; Abbasi, Siddique A. ; Yang, Eunice ; Day, Sharlene M. ; Colan, Steven D. ; Russell, Mark W. ; Towbin, Jeffrey ; Sherrid, Mark V. ; Canter, Charles E. ; Shi, Ling ; Jerosch-Herold, Michael ; Bluemke, David A. ; Ho, Carolyn ; Neilan, Tomas G. / Left Atrial structure and function in hypertrophic cardiomyopathy sarcomere mutation carriers with and without left ventricular hypertrophy. In: Journal of Cardiovascular Magnetic Resonance. 2017 ; Vol. 19, No. 1.
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abstract = "Background: Impaired left atrial (LA) function is an early marker of cardiac dysfunction and predictor of adverse cardiac events. Herein, we assess LA structure and function in hypertrophy in hypertrophic cardiomyopathy (HCM) sarcomere mutation carriers with and without left ventricular hypertrophy (LVH). Method: Seventy-three participants of the HCMNet study who underwent cardiovascular magnetic resonance (CMR) imaging were studied, including mutation carriers with overt HCM (n = 34), preclinical mutation carriers without HCM (n = 24) and healthy, familial controls (n = 15). Results: LA volumes were similar between preclinical, control and overt HCM cohorts after covariate adjustment. However, there was evidence of impaired LA function with decreased LA total emptying function in both preclinical (64 ± 8{\%}) and overt HCM (59 ± 10{\%}), compared with controls (70 ± 7{\%}; p = 0.002 and p = 0.005, respectively). LA passive emptying function was also decreased in overt HCM (35 ± 11{\%}) compared with controls (47 ± 10{\%}; p = 0.006). Both LAtotal emptying function and LA passive emptying function were inversely correlated with the extent of late gadolinium enhancement (LGE; p = 0.005 and p < 0.05, respectively), LV mass (p = 0.02 and p < 0.001) and interventricular septal thickness (p < 0.001 for both) and serum NT-proBNP levels (p < 0.001 for both). Conclusion: LA dysfunction is detectable by CMR in preclinical HCM mutation carriers despite non-distinguishable LV wall thickness and LA volume. LA function appears most impaired in subjects with overt HCM and a greater extent of LV fibrosis.",
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T1 - Left Atrial structure and function in hypertrophic cardiomyopathy sarcomere mutation carriers with and without left ventricular hypertrophy

AU - Farhad, Hoshang

AU - Seidelmann, Sara B.

AU - Vigneault, Davis

AU - Abbasi, Siddique A.

AU - Yang, Eunice

AU - Day, Sharlene M.

AU - Colan, Steven D.

AU - Russell, Mark W.

AU - Towbin, Jeffrey

AU - Sherrid, Mark V.

AU - Canter, Charles E.

AU - Shi, Ling

AU - Jerosch-Herold, Michael

AU - Bluemke, David A.

AU - Ho, Carolyn

AU - Neilan, Tomas G.

PY - 2017/12/28

Y1 - 2017/12/28

N2 - Background: Impaired left atrial (LA) function is an early marker of cardiac dysfunction and predictor of adverse cardiac events. Herein, we assess LA structure and function in hypertrophy in hypertrophic cardiomyopathy (HCM) sarcomere mutation carriers with and without left ventricular hypertrophy (LVH). Method: Seventy-three participants of the HCMNet study who underwent cardiovascular magnetic resonance (CMR) imaging were studied, including mutation carriers with overt HCM (n = 34), preclinical mutation carriers without HCM (n = 24) and healthy, familial controls (n = 15). Results: LA volumes were similar between preclinical, control and overt HCM cohorts after covariate adjustment. However, there was evidence of impaired LA function with decreased LA total emptying function in both preclinical (64 ± 8%) and overt HCM (59 ± 10%), compared with controls (70 ± 7%; p = 0.002 and p = 0.005, respectively). LA passive emptying function was also decreased in overt HCM (35 ± 11%) compared with controls (47 ± 10%; p = 0.006). Both LAtotal emptying function and LA passive emptying function were inversely correlated with the extent of late gadolinium enhancement (LGE; p = 0.005 and p < 0.05, respectively), LV mass (p = 0.02 and p < 0.001) and interventricular septal thickness (p < 0.001 for both) and serum NT-proBNP levels (p < 0.001 for both). Conclusion: LA dysfunction is detectable by CMR in preclinical HCM mutation carriers despite non-distinguishable LV wall thickness and LA volume. LA function appears most impaired in subjects with overt HCM and a greater extent of LV fibrosis.

AB - Background: Impaired left atrial (LA) function is an early marker of cardiac dysfunction and predictor of adverse cardiac events. Herein, we assess LA structure and function in hypertrophy in hypertrophic cardiomyopathy (HCM) sarcomere mutation carriers with and without left ventricular hypertrophy (LVH). Method: Seventy-three participants of the HCMNet study who underwent cardiovascular magnetic resonance (CMR) imaging were studied, including mutation carriers with overt HCM (n = 34), preclinical mutation carriers without HCM (n = 24) and healthy, familial controls (n = 15). Results: LA volumes were similar between preclinical, control and overt HCM cohorts after covariate adjustment. However, there was evidence of impaired LA function with decreased LA total emptying function in both preclinical (64 ± 8%) and overt HCM (59 ± 10%), compared with controls (70 ± 7%; p = 0.002 and p = 0.005, respectively). LA passive emptying function was also decreased in overt HCM (35 ± 11%) compared with controls (47 ± 10%; p = 0.006). Both LAtotal emptying function and LA passive emptying function were inversely correlated with the extent of late gadolinium enhancement (LGE; p = 0.005 and p < 0.05, respectively), LV mass (p = 0.02 and p < 0.001) and interventricular septal thickness (p < 0.001 for both) and serum NT-proBNP levels (p < 0.001 for both). Conclusion: LA dysfunction is detectable by CMR in preclinical HCM mutation carriers despite non-distinguishable LV wall thickness and LA volume. LA function appears most impaired in subjects with overt HCM and a greater extent of LV fibrosis.

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DO - 10.1186/s12968-017-0420-0

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