Value of Speckle Tracking-Based Deformation Analysis in Screening Relatives of Patients With Asymptomatic Dilated Cardiomyopathy

Job A. J. Verdonschot; Jort J. Merken; Hans-Peter Brunner-La Rocca; Mark R. Hazebroek; Casper G. M. J. Eurlings; Eline Thijssen; Ping Wang; Jerremy Weerts; Vanessa van Empel; Georg Schummers; Marcus Schreckenberg; Arthur van den Wijngaard; Joost Lumens; Han G. Brunner; Stephane R. B. Heymans; Ingrid P. C. Krapels; Christian Knackstedt

doi:10.1016/j.jcmg.2019.02.032

Value of Speckle Tracking-Based Deformation Analysis in Screening Relatives of Patients With Asymptomatic Dilated Cardiomyopathy

Job A. J. Verdonschot, Jort J. Merken, Hans-Peter Brunner-La Rocca, Mark R. Hazebroek, Casper G. M. J. Eurlings, Eline Thijssen, Ping Wang, Jerremy Weerts, Vanessa van Empel, Georg Schummers, Marcus Schreckenberg, Arthur van den Wijngaard, Joost Lumens, Han G. Brunner, Stephane R. B. Heymans, Ingrid P. C. Krapels, Christian Knackstedt^*

^*Corresponding author for this work

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Abstract

OBJECTIVES This study sought to investigate the prevalence of systolic dysfunction using global longitudinal strain (GLS) and its prognostic value in relatives of dilated cardiomyopathy (DCM) patients that had normal left ventricular ejection fraction (LVEF).

BACKGROUND DCM relatives are advised to undergo cardiac assessment including echocardiography, irrespective of the genetic status of the index patient. Even though LVEF is normal, the question remains whether this indicates absence of disease or simply normal cardiac volumes. GLS may provide additional information regarding (sub)clinical cardiac abnormalities and thus allow earlier disease detection.

METHODS A total of 251 DCM relatives and 251 control subjects with a normal LVEF (>= 55%) were screened. Automated software measured the GLS on echocardiographic 2-, 3-, and 4-chamber views. The cutoff value for abnormal strain was >-21.5. Median follow-up was 40 months (interquartite range: 5 to 80 months). Primary outcome was the combination of death and cardiac hospitalization.

RESULTS A total of 120 relatives and 83 control subjects showed abnormal GLS (48% vs. 33%, respectively; p <0.001). Abnormal GLS was independently associated with DCM relatives and cardiovascular risk factors, rather than genetic mutations. Subjects with abnormal GLS had more frequent cardiac hospitalizations and a higher mortality as compared with subjects with normal GLS (hazard ratio: 3.29; 95% confidence interval: 1.58 to 6.87; p=0.001). Additionally, follow-up LVEF was measured in a subset of relatives, and it decreased significantly in those with abnormal as compared with normal GLS (p=0.006).

CONCLUSIONS Relatives of DCM patients had a significantly higher prevalence of systolic dysfunction detected by GLS despite normal LVEF compared with control subjects, independent of age, sex, comorbidities, and genotype. Abnormal GLS was associated with LVEF deterioration, cardiac hospitalization, and death. (C) 2020 by the American College of Cardiology Foundation.

Original language	English
Pages (from-to)	549-558
Number of pages	10
Journal	JACC-Cardiovascular Imaging
Volume	13
Issue number	2
DOIs	https://doi.org/10.1016/j.jcmg.2019.02.032
Publication status	Published - Feb 2020

Keywords

dilated cardiomyopathy
genetics
global longitudinal strain
prognosis
relatives
screening
GLOBAL LONGITUDINAL STRAIN
EUROPEAN ASSOCIATION
POSITION STATEMENT
AMERICAN SOCIETY
HEART-FAILURE
WORKING GROUP
ECHOCARDIOGRAPHY
RISK
RECOMMENDATIONS
MUTATIONS

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Verdonschot, J. A. J., Merken, J. J., Brunner-La Rocca, H.-P., Hazebroek, M. R., Eurlings, C. G. M. J., Thijssen, E., Wang, P., Weerts, J., van Empel, V., Schummers, G., Schreckenberg, M., van den Wijngaard, A., Lumens, J., Brunner, H. G., Heymans, S. R. B., Krapels, I. P. C., & Knackstedt, C. (2020). Value of Speckle Tracking-Based Deformation Analysis in Screening Relatives of Patients With Asymptomatic Dilated Cardiomyopathy. JACC-Cardiovascular Imaging, 13(2), 549-558. https://doi.org/10.1016/j.jcmg.2019.02.032

@article{9776bc5382e54ea09122c6e05130a798,

title = "Value of Speckle Tracking-Based Deformation Analysis in Screening Relatives of Patients With Asymptomatic Dilated Cardiomyopathy",

abstract = "OBJECTIVES This study sought to investigate the prevalence of systolic dysfunction using global longitudinal strain (GLS) and its prognostic value in relatives of dilated cardiomyopathy (DCM) patients that had normal left ventricular ejection fraction (LVEF).BACKGROUND DCM relatives are advised to undergo cardiac assessment including echocardiography, irrespective of the genetic status of the index patient. Even though LVEF is normal, the question remains whether this indicates absence of disease or simply normal cardiac volumes. GLS may provide additional information regarding (sub)clinical cardiac abnormalities and thus allow earlier disease detection.METHODS A total of 251 DCM relatives and 251 control subjects with a normal LVEF (>= 55%) were screened. Automated software measured the GLS on echocardiographic 2-, 3-, and 4-chamber views. The cutoff value for abnormal strain was >-21.5. Median follow-up was 40 months (interquartite range: 5 to 80 months). Primary outcome was the combination of death and cardiac hospitalization.RESULTS A total of 120 relatives and 83 control subjects showed abnormal GLS (48% vs. 33%, respectively; p <0.001). Abnormal GLS was independently associated with DCM relatives and cardiovascular risk factors, rather than genetic mutations. Subjects with abnormal GLS had more frequent cardiac hospitalizations and a higher mortality as compared with subjects with normal GLS (hazard ratio: 3.29; 95% confidence interval: 1.58 to 6.87; p=0.001). Additionally, follow-up LVEF was measured in a subset of relatives, and it decreased significantly in those with abnormal as compared with normal GLS (p=0.006).CONCLUSIONS Relatives of DCM patients had a significantly higher prevalence of systolic dysfunction detected by GLS despite normal LVEF compared with control subjects, independent of age, sex, comorbidities, and genotype. Abnormal GLS was associated with LVEF deterioration, cardiac hospitalization, and death. (C) 2020 by the American College of Cardiology Foundation.",

keywords = "dilated cardiomyopathy, genetics, global longitudinal strain, prognosis, relatives, screening, GLOBAL LONGITUDINAL STRAIN, EUROPEAN ASSOCIATION, POSITION STATEMENT, AMERICAN SOCIETY, HEART-FAILURE, WORKING GROUP, ECHOCARDIOGRAPHY, RISK, RECOMMENDATIONS, MUTATIONS",

author = "Verdonschot, {Job A. J.} and Merken, {Jort J.} and {Brunner-La Rocca}, Hans-Peter and Hazebroek, {Mark R.} and Eurlings, {Casper G. M. J.} and Eline Thijssen and Ping Wang and Jerremy Weerts and {van Empel}, Vanessa and Georg Schummers and Marcus Schreckenberg and {van den Wijngaard}, Arthur and Joost Lumens and Brunner, {Han G.} and Heymans, {Stephane R. B.} and Krapels, {Ingrid P. C.} and Christian Knackstedt",

note = "Publisher Copyright: {\textcopyright} 2020 American College of Cardiology Foundation",

year = "2020",

month = feb,

doi = "10.1016/j.jcmg.2019.02.032",

language = "English",

volume = "13",

pages = "549--558",

journal = "JACC-Cardiovascular Imaging",

issn = "1936-878X",

publisher = "Elsevier Science",

number = "2",

}

Verdonschot, JAJ, Merken, JJ, Brunner-La Rocca, H-P, Hazebroek, MR, Eurlings, CGMJ , Thijssen, E , Wang, P , Weerts, J , van Empel, V, Schummers, G, Schreckenberg, M, van den Wijngaard, A, Lumens, J , Brunner, HG , Heymans, SRB , Krapels, IPC & Knackstedt, C 2020, 'Value of Speckle Tracking-Based Deformation Analysis in Screening Relatives of Patients With Asymptomatic Dilated Cardiomyopathy', JACC-Cardiovascular Imaging, vol. 13, no. 2, pp. 549-558. https://doi.org/10.1016/j.jcmg.2019.02.032

TY - JOUR

T1 - Value of Speckle Tracking-Based Deformation Analysis in Screening Relatives of Patients With Asymptomatic Dilated Cardiomyopathy

AU - Verdonschot, Job A. J.

AU - Merken, Jort J.

AU - Brunner-La Rocca, Hans-Peter

AU - Hazebroek, Mark R.

AU - Eurlings, Casper G. M. J.

AU - Thijssen, Eline

AU - Wang, Ping

AU - Weerts, Jerremy

AU - van Empel, Vanessa

AU - Schummers, Georg

AU - Schreckenberg, Marcus

AU - van den Wijngaard, Arthur

AU - Lumens, Joost

AU - Brunner, Han G.

AU - Heymans, Stephane R. B.

AU - Krapels, Ingrid P. C.

AU - Knackstedt, Christian

PY - 2020/2

Y1 - 2020/2

N2 - OBJECTIVES This study sought to investigate the prevalence of systolic dysfunction using global longitudinal strain (GLS) and its prognostic value in relatives of dilated cardiomyopathy (DCM) patients that had normal left ventricular ejection fraction (LVEF).BACKGROUND DCM relatives are advised to undergo cardiac assessment including echocardiography, irrespective of the genetic status of the index patient. Even though LVEF is normal, the question remains whether this indicates absence of disease or simply normal cardiac volumes. GLS may provide additional information regarding (sub)clinical cardiac abnormalities and thus allow earlier disease detection.METHODS A total of 251 DCM relatives and 251 control subjects with a normal LVEF (>= 55%) were screened. Automated software measured the GLS on echocardiographic 2-, 3-, and 4-chamber views. The cutoff value for abnormal strain was >-21.5. Median follow-up was 40 months (interquartite range: 5 to 80 months). Primary outcome was the combination of death and cardiac hospitalization.RESULTS A total of 120 relatives and 83 control subjects showed abnormal GLS (48% vs. 33%, respectively; p <0.001). Abnormal GLS was independently associated with DCM relatives and cardiovascular risk factors, rather than genetic mutations. Subjects with abnormal GLS had more frequent cardiac hospitalizations and a higher mortality as compared with subjects with normal GLS (hazard ratio: 3.29; 95% confidence interval: 1.58 to 6.87; p=0.001). Additionally, follow-up LVEF was measured in a subset of relatives, and it decreased significantly in those with abnormal as compared with normal GLS (p=0.006).CONCLUSIONS Relatives of DCM patients had a significantly higher prevalence of systolic dysfunction detected by GLS despite normal LVEF compared with control subjects, independent of age, sex, comorbidities, and genotype. Abnormal GLS was associated with LVEF deterioration, cardiac hospitalization, and death. (C) 2020 by the American College of Cardiology Foundation.

AB - OBJECTIVES This study sought to investigate the prevalence of systolic dysfunction using global longitudinal strain (GLS) and its prognostic value in relatives of dilated cardiomyopathy (DCM) patients that had normal left ventricular ejection fraction (LVEF).BACKGROUND DCM relatives are advised to undergo cardiac assessment including echocardiography, irrespective of the genetic status of the index patient. Even though LVEF is normal, the question remains whether this indicates absence of disease or simply normal cardiac volumes. GLS may provide additional information regarding (sub)clinical cardiac abnormalities and thus allow earlier disease detection.METHODS A total of 251 DCM relatives and 251 control subjects with a normal LVEF (>= 55%) were screened. Automated software measured the GLS on echocardiographic 2-, 3-, and 4-chamber views. The cutoff value for abnormal strain was >-21.5. Median follow-up was 40 months (interquartite range: 5 to 80 months). Primary outcome was the combination of death and cardiac hospitalization.RESULTS A total of 120 relatives and 83 control subjects showed abnormal GLS (48% vs. 33%, respectively; p <0.001). Abnormal GLS was independently associated with DCM relatives and cardiovascular risk factors, rather than genetic mutations. Subjects with abnormal GLS had more frequent cardiac hospitalizations and a higher mortality as compared with subjects with normal GLS (hazard ratio: 3.29; 95% confidence interval: 1.58 to 6.87; p=0.001). Additionally, follow-up LVEF was measured in a subset of relatives, and it decreased significantly in those with abnormal as compared with normal GLS (p=0.006).CONCLUSIONS Relatives of DCM patients had a significantly higher prevalence of systolic dysfunction detected by GLS despite normal LVEF compared with control subjects, independent of age, sex, comorbidities, and genotype. Abnormal GLS was associated with LVEF deterioration, cardiac hospitalization, and death. (C) 2020 by the American College of Cardiology Foundation.

KW - dilated cardiomyopathy

KW - genetics

KW - global longitudinal strain

KW - prognosis

KW - relatives

KW - screening

KW - GLOBAL LONGITUDINAL STRAIN

KW - EUROPEAN ASSOCIATION

KW - POSITION STATEMENT

KW - AMERICAN SOCIETY

KW - HEART-FAILURE

KW - WORKING GROUP

KW - ECHOCARDIOGRAPHY

KW - RISK

KW - RECOMMENDATIONS

KW - MUTATIONS

U2 - 10.1016/j.jcmg.2019.02.032

DO - 10.1016/j.jcmg.2019.02.032

M3 - Article

C2 - 31202754

SN - 1936-878X

VL - 13

SP - 549

EP - 558

JO - JACC-Cardiovascular Imaging

JF - JACC-Cardiovascular Imaging

IS - 2

ER -