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
N1 - Publisher Copyright:
© 2020 American College of Cardiology Foundation
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 -