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. 2020 Feb 4;10(1):1767.
doi: 10.1038/s41598-020-58662-8.

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

Tarek Alsaied  1 , Omar Niss  2 , Justin T Tretter  3 , Adam W Powell  3 , Clifford Chin  3 , Robert J Fleck  4 , James F Cnota  3 , Punam Malik  2   5 , Charles T Quinn  2 , Sherif F Nagueh  6 , Michael D Taylor  3 , Wojciech M Mazur  7
Affiliations

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

Tarek Alsaied et al. Sci Rep. .

Erratum in

Abstract

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.

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Conflict of interest statement

The authors declare no competing interests.

Figures

Figure 1
Figure 1
Atrial strain and strain rate by echocardiogram. (A) represent atrial strain curves measured over the cardiac cycle. The white curve represents average atrial strain curve. (B) represents atrial strain rate curve.
Figure 2
Figure 2
Left atrial late gadolinium enhancement in a patient with SCA in four chamber and three chamber views (blue arrows).
Figure 3
Figure 3
Left ventricular extracellular volume (ECV) negatively associated with atrial ejection fraction (A), positive strain rate (B) and atrial reservoir strain (C).
Figure 4
Figure 4
Left atrial stiffness negatively associated with percent predicted VO2 (A) and positively associated with VE/VCO2 slope at maximum exercise.
Figure 5
Figure 5
Left atrial stiffness correlates positively with tricuspid regurgitation velocity.
Figure 6
Figure 6
Summary of cardiac involvement in sickle cell anemia.
See this image and copyright information in PMC

References

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