TY - JOUR
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.
N1 - Funding Information:
This work was supported by the National Heart, Lung, and Blood Institute at the National Institutes of Health [1P20HL101408 to CYH] and the NIH Intramural Research Program. Dr. Farhad was supported partly by a Swiss Society of Hypertension Grant 2013. Dr. Seidelmann is supported by NIH grant number 2T32HL094301-06. Dr. Neilan was supported in part through the Kohlberg Foundation, an American Heart Association Fellow to Faculty Award (12FTF12060588), National Institutes of Health/ National Heart, Lung, and Blood Institute (1R01HL130539-01A1; 1R01HL137562 - 01A1) and National Institutes of Health/ Harvard Center for AIDS Research (P30 AI060354).
Funding Information:
This work was supported by the National Heart, Lung, and Blood Institute at the National Institutes of Health [1P20HL101408 to CYH] and the NIH Intramural Research Program. Dr. Farhad was supported partly by a Swiss Society of Hypertension Grant 2013. Dr. Seidelmann is supported by NIH grant number 2T32HL094301–06. Dr. Neilan was supported in part through the Kohlberg Foundation, an American Heart Association Fellow to Faculty Award (12FTF12060588), National Institutes of Health/ National Heart, Lung, and Blood Institute (1R01HL130539-01A1; 1R01HL137562 - 01A1) and National Institutes of Health/ Harvard Center for AIDS Research (P30 AI060354).
Publisher Copyright:
© 2017 The Author(s).
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.
KW - Cardiovascular magnetic resonance (CMR)
KW - Hypertrophic cardiomyopathy
KW - Left Atrial function
UR - http://www.scopus.com/inward/record.url?scp=85039701325&partnerID=8YFLogxK
U2 - 10.1186/s12968-017-0420-0
DO - 10.1186/s12968-017-0420-0
M3 - Article
C2 - 29284499
AN - SCOPUS:85039701325
SN - 1097-6647
VL - 19
JO - Journal of Cardiovascular Magnetic Resonance
JF - Journal of Cardiovascular Magnetic Resonance
IS - 1
M1 - 107
ER -