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Left atrial dysfunction in sickle cell anemia is associated with diffuse myocardial fibrosis, increased right ventricular pressure and reduced exercise capacity

Increased extracellular volume (ECV) by CMR is a marker of interstitial myocardial fibrosis and is associated with diastolic dysfunction in sickle cell anemia (SCA). Left atrial (LA) dysfunction and stiffness contribute to the development of diastolic heart failure in other settings. We aimed to eva...

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Detalles Bibliográficos
Autores principales: Alsaied, Tarek, Niss, Omar, Tretter, Justin T., Powell, Adam W., Chin, Clifford, Fleck, Robert J., Cnota, James F., Malik, Punam, Quinn, Charles T., Nagueh, Sherif M., Taylor, Michael D., Mazur, Wojciech M.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7000788/
https://www.ncbi.nlm.nih.gov/pubmed/32019991
http://dx.doi.org/10.1038/s41598-020-58662-8
Descripción
Sumario:Increased extracellular volume (ECV) by CMR is a marker of interstitial myocardial fibrosis and is associated with diastolic dysfunction in sickle cell anemia (SCA). Left atrial (LA) dysfunction and stiffness contribute to the development of diastolic heart failure in other settings. We aimed to evaluate LA function and stiffness associations with ECV, tricuspid regurgitation jet velocity (TRV) and exercise abnormalities in SCA. In a prospective study, individuals with SCA underwent CMR, echocardiography and exercise test. ECV was measured using MOLLI sequence. Atrial strain was studied in the 4- and 2-chamber views. LA stiffness was calculated as the ratio of echocardiographic E/e’-to-LA reservoir strain. Twenty-four participants with SCA were included (median age 20 years). ECV was increased in participant with SCA compared to our lab normal values (mean 0.44 ± 0.08 vs 0.26 ± 0.02, P < 0.0001). Six (25%) had LA LGE. ECV positively correlated with LA stiffness (r = 0.45, p = 0.04). There was a negative correlation between LA stiffness and %predicted VO2 (r = −0.50, p = 0.04). LA stiffness was moderately associated with increased TRV (r = 0.55, p < 0.005). LA stiffness is associated with ECV, exercise impairment and increased TRV. This study sheds insights on the interaction between LA function, RV hypertension, and myocardial fibrosis in SCA.