P1023Upstream therapy with spironolactone in patients with atrial fibrillation may reduce the burden of diffuse myocardial fibrosis and arrhythmia

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
D Rujic ◽  
P L Madsen ◽  
M Pareek ◽  
T Juhl Hansen ◽  
K Egstrup
Keyword(s):  
Atrial Fibrillation ◽  
Ejection Fraction ◽  
Myocardial Fibrosis ◽  
Longitudinal Strain ◽  
Low Dose ◽  
Myocardial Strain ◽  
Diffuse Myocardial Fibrosis ◽  
Diffuse Fibrosis ◽  
Double Blind Study ◽  
Post Contrast

Abstract Background The pathophysiology of atrial fibrillation (AF) involves a continuum of structural, hemodynamic, and electrophysical remodeling that coexist in a complex interplay, often involving increased diffuse myocardial fibrosis. Purpose To examine whether low dose spironolactone can reduce the extent of diffuse fibrosis in the left atrium (LA) and left ventricle (LV), evaluated using post-contrast T1 myocardial relaxation time (T1 time) and myocardial strain derived from cardiac magnetic resonance imaging (CMR), in patients with paroxysmal and persistent AF. Methods CMR sub-study population of 100 individuals from INSPIRE-AF, an investigator-initiated, randomized, controlled, double-blind study (NCT02764619). Study participants with paroxysmal (n=49) and persistent (n=51) non-valvular AF and LV ejection fraction ≥45% were randomized to spironolactone 25 mg once daily (SPL-group, n=49) or placebo (PL-group, n=51) in addition to standard treatment. CMR scans were conducted at inclusion and after 12 months of treatment. LA and LV measures, including volumes, ejection fraction, and peak systolic longitudinal strain were assessed using cine CMR feature tracking. T1 times were estimated using a previously validated modified look-locker inversion-recovery sequence. Commercially available software (Circle, Calgary, Canada) was used. All participants were followed for recurrences of AF, and all recurrences were documented with a standard 12-lead electrocardiogram. The associations between changes in mean volume, strain, and T1 times were examined using multivariable linear regression, adjusted for baseline age, sex, and AF type. Results LV post-contrast T1 time increased significantly in the SPL group (mean increase in SPL=1,97 ms [95% CI: 0,169; 3,323] vs. PL=0,21 ms [95% CI: −0,67; 1,11], p=0,027 for difference). However, the mean change of post-contrast LA T1 time did not differ between groups. LV peak longitudinal strain was also significantly improved in the SPL group (mean decrease in SPL: 19,82% [95% CI: −0,85; 40,48] vs. PL: 14,15% [95% CI: −36,21; 7,92], p=0,047). Similarly, LA stroke volume corrected for body surface area increased in the SPL-group (mean SPL: 34,33 ml/m2 [95% CI: −2,17; 42,62] vs. PL: 8,46 ml/m2 [95% CI: −20,13; 5,94], p=0,013). All changes remained significant after adjusting for age, sex and systolic blood pressure. No difference was found in arrhythmia burden between groups (P=0,89). Conclusion Low dose spironolactone may reduce the burden of diffuse myocardial fibrosis in AF. However, there was no reduction in the burden of arrhythmia. Acknowledgement/Funding Region of Southern Denmark, University of Southern Denmark, Danish Heart Association

Abstract 17255: Standard 12-Lead Electrocardiogram Can Predict Diffuse Myocardial Fibrosis in Patients With Atrial Fibrillation

Circulation ◽  
2018 ◽  
Vol 138 (Suppl_1) ◽  
Author(s):  
Dragana Rujic ◽  
Saman Nazarian ◽  
Per L Madsen ◽  
Benoit Desjardins ◽  
Ling Kuo ◽  
...  
Keyword(s):  
Atrial Fibrillation ◽  
Sinus Rhythm ◽  
Myocardial Fibrosis ◽  
Surrogate Marker ◽  
Diffuse Myocardial Fibrosis ◽  
P Wave ◽  
Diffuse Fibrosis ◽  
Post Contrast ◽  
Wave Duration ◽  
Qrs Duration

Background: The pathophysiology of atrial fibrillation (AF) involves a continuum of myocardial remodeling with increased diffuse fibrosis. Purpose: To examine whether the extent of diffuse fibrosis in the left atrium (LA) and left ventricle (LV), evaluated using the surrogate marker, post-contrast T1 myocardial relaxation time on cardiac magnetic resonance imaging (CMR), in patients with paroxysmal and persistent AF is associated with ECG markers obtained during sinus rhythm. Methods: This is a prespecified secondary analysis from the randomized controlled trial INSPIRE-AF (n=95) (ClinicalTrials.gov NCT02764619). Patients with paroxysmal and persistent AF underwent CMR. Post-contrast T1 times were quantified in 47 paroxysmal AF (mean age, 63 ±8 years), and 43 persistent AF (mean age, 54 ±7 years) patients using a previously validated modified look-locker inversion-recovery sequence. Standard resting 12-lead ECGs were analyzed using commercially available software. Results: Baseline characteristics were generally similarly distributed between AF groups. Mean LA T1 time was 492 +/- 72 ms and mean LV T1 time was 494 +/- 49 ms. P-wave area, P-wave terminal force, PR-interval, P-wave peak amplitude, and P-wave duration were unassociated with LA T1 time, and no significant interactions with AF type were detected. Similarly, neither R-wave duration, corrected QT interval (Bazett’s formula), nor QRS duration were associated with LV T1 time. P-wave terminal force in lead V1 did not differ between groups of investigation, but the joint mean value of -0.04 mm/s in the two groups is prominently different from normal controls. AF type significantly interacted with the association between R-wave duration and T1 time (P=0.03 for interaction), and between QRS duration and T1 time (P=0.009 for interaction). Specifically, R-wave duration and QRS duration were only significantly associated with T1 time among patients with persistent AF. Conclusions: Diffuse fibrosis in the LV, defined as post-contrast T1 time, was associated with R-wave duration and QRS duration in patients with persistent AF. Our findings suggest that surface 12-lead ECG obtained in sinus rhythm could provide predictive information regarding diffuse myocardial fibrosis.

scholarly journals Diffuse myocardial fibrosis precedes impairment of myocardial strain in patients with systemic sclerosis

2021 ◽  
Vol 22 (Supplement_2) ◽  
Author(s):  
A Gotschy ◽  
S Jordan ◽  
CT Stoeck ◽  
C Von Deuster ◽  
M Gastl ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Myocardial Fibrosis ◽  
Functional Impairment ◽  
Myocardial Strain ◽  
Extracellular Volume ◽  
Diffuse Myocardial Fibrosis ◽  
Healthy Controls ◽  
Post Contrast ◽  
Native T1 ◽  
Myocardial Involvement

Abstract Funding Acknowledgements Type of funding sources: None. Background - Myocardial involvement is common in patients with systemic sclerosis (SSc) and causes myocardial fibrosis and subtle ventricular dysfunction. However, the temporal onset of myocardial involvement during the progression of the disease is yet unknown. Purpose - To investigated the presence of subclinical functional impairment and diffuse myocardial fibrosis in patients with very early diagnosis of SSc (VEDOSS) and to compared the findings to patients with established SSc and healthy controls. Methods - 110 SSc patients (86 with established SSc and 24 with VEDOSS) and 15 healthy controls were prospectively recruited. The study subjects underwent cardiovascular magnetic resonance on a clinical 1.5T system. Pre- and post-contrast T1 mapping was performed using a MOLLI (Modified Look-Locker Inversion Recovery) sequence. For extracellular volume (ECV) measurements, a single bolus protocol with image acquisition 15-20 min. post-contrast injection was used. For the assessment of subtle functional impairment, global longitudinal (GLS) and circumferential (GCS) myocardial strain were evaluated. Results - Native T1 values and ECV were elevated in VEDOSS and in patients with established SSc compared to controls (p < 0.001; Figure 1 A & B). GLS was similar in VEDOSS and controls but significantly reduced in patients with established SSc (p < 0.001; Figure 1 C). GCS was similar over all groups (p = 0.88). Patients with clinical evidence of pulmonary or gastrointestinal involvement had higher ECV or T1 values, respectively. Patients with clinical signs of cardiac involvement had lower absolute GLS. SSc subtype, classification or disease duration were not associated with the extent of myocardial fibrosis or impaired strain. Conclusion - Subclinical myocardial involvement first manifests as diffuse myocardial fibrosis identified by expansion of ECV and increased native T1 in VEDOSS patients while subtle functional impairment as measured by GLS only occurs in established SSc. No single clinical feature of SSc shows a strong association with subtle myocardial involvement.

scholarly journals The link between left ventricular extracellular volume determined by T1 myocardial mapping and gut microbiota composition in individuals with heart failure and preserved ejection fraction

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
A.N Kaburova ◽  
O.M Drapkina ◽  
S.M Uydin ◽  
M.V Vishnyakova ◽  
M.S Pokrovskaya ◽  
...  
Keyword(s):  
Heart Failure ◽  
Ejection Fraction ◽  
Gut Microbiota ◽  
Myocardial Fibrosis ◽  
Volume Fraction ◽  
Extracellular Volume ◽  
Left Ventricular ◽  
Diffuse Myocardial Fibrosis ◽  
Rrna Gene ◽  
Preserved Ejection Fraction

Abstract Introduction Heart failure with preserved ejection fraction (HFpEF) represents a major challenge in modern cardiology. As described previously, in HFpEF comorbidities promote a systemic inflammatory state, leading to diffuse myocardial fibrosis resulting in myocardial stiffening. Gut dysbiosis which is considered as the novel source of chronic systemic inflammation has been actively investigated as the risk factor for the development and aggravation of cardiovascular diseases including heart failure. Cardiac magnetic resonance T1-mapping is a novel tool, which allows noninvasive quantification of the extracellular space and diffuse myocardial fibrosis. Moreover, the extracellular volume (ECV) fraction can be calculated, providing information on the relative expansion of the extracellular matrix, thus being a noninvasive alternative to myocardial biopsy studies. Purpose The research was aimed at investigating the correlation between the left ventricular ECV and gut microbial genera in patients with HFpEF. Methods 42 patients with confirmed HF-pEF (mediana and interquartile range of age 67 [64; 72] years, 47% men, body mass index <35 kg/m2 with no history of myocardial infarction or diabetes mellitus) were enrolled in the study. The patients underwent transthoracic echocardiography with Doppler study, HF-pEF was confirmed according to the recent ESC guidelines (based on E/e' ratio, N-terminal pro-B type natriuretic peptide >125 pg/ml and symptoms of heart failure). The intestinal microbiome was investigated using high-throughput sequencing of bacterial 16S rRNA gene. As the last step of research T1-myocardial mapping with the modified look-locker inversion-recovery protocol (MOLLI) sequence at 1.5 Tesla was performed to assess left ventricular extracellular volume fraction. Results The mean±std in ECV was 31.02±4.4%. The relative abundance (%) of the most prevalent phyla in gut microbiota was 48±22.5 for Firmicutes, 47.4±22.8 for Bacteroidetes and 1.5 [1.5; 2.5] for Proteobacteria. The analysis showed significant negative correlations between ECV and the following bacterial genera: Faecalibacterium (r=−0.35), Blautia (r=−0.43), Lachnoclostridium (r=−0.32). Moreover ECV positively correlated with Holdemania (r=0.4), Victivallis (r=0.38), Dehalobacterium (r=0.38), Enterococcus (r=0.33) and Catabacter (r=0.32). All correlation values with p<0.05. Conclusion We discovered both negative and positive significant correlations between ECV – the non-invasive marker of myocardial fibrosis and several bacterial genera, which may have negative impact on myocardial remodeling in HF-pEF. Funding Acknowledgement Type of funding source: None

scholarly journals Predictive values of multiple non-invasive markers for myocardial fibrosis in hypertrophic cardiomyopathy patients with preserved ejection fraction

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Yumin Li ◽  
Jia Liu ◽  
Yukun Cao ◽  
Xiaoyu Han ◽  
Guozhu Shao ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Ejection Fraction ◽  
Myocardial Fibrosis ◽  
Troponin I ◽  
Myocardial Strain ◽  
Left Ventricular ◽  
Preserved Ejection Fraction ◽  
Predictive Values ◽  
Non Invasive ◽  
Potential Biomarker

AbstractMyocardial fibrosis assessed by late gadolinium enhancement (LGE) on cardiovascular magnetic resonance (CMR) is associated with cardiovascular outcomes in hypertrophic cardiomyopathy (HCM) patients, but little is known about the utility of non-invasive markers for detecting LGE. This study aims to explore the association between cardiac-specific biomarkers, CMR myocardial strain, left ventricular (LV) hypertrophy and LGE in HCM patients with preserved ejection fraction (EF) and investigate the predictive values of these indexes for LGE. We recruited 33 healthy volunteers and 86 HCM patients with preserved EF to undergo contrast-enhanced CMR examinations. In total, 48 of 86 HCM patients had the presence of LGE. The LGE-positive patients had significant higher serum high-sensitivity cardiac troponin I (hs-cTnI) and N-terminal pro b-type natriuretic peptide (Nt-proBNP) levels and lower global longitudinal (GLS) and circumferential (GCS) strains than the LGE-negative group. The LGE% was independently associated with the Nt-proBNP levels, GCS, LV end-diastolic maximum wall thickness (MWT) and beta-blocker treatment. In the receiver operating characteristic curve analysis, the combined parameters of Nt-proBNP ≥ 108.00 pg/mL and MWT ≥ 17.30 mm had good diagnostic performance for LGE, with a specificity of 81.25% and sensitivity of 70.00%. These data indicate that serum Nt-proBNP is a potential biomarker associated with LGE% and, combined with MWT, were useful for identifying myocardial fibrosis in HCM patients with preserved EF. Additionally, LV GCS may be a more sensitive indicator for reflecting the presence of myocardial fibrosis than GLS.

Abstract 18922: Radiofrequency Catheter Ablation Reduces Diffuse Myocardial Fibrosis in Atrial Fibrillation

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Promporn Suksaranjit ◽  
Brent D Wilson ◽  
Christopher J McGann ◽  
Eugene G Kholmovski ◽  
Imran Haider ◽  
...  
Keyword(s):  
Atrial Fibrillation ◽  
Catheter Ablation ◽  
Myocardial Fibrosis ◽  
Radiofrequency Catheter Ablation ◽  
T1 Mapping ◽  
Left Ventricular ◽  
Diffuse Myocardial Fibrosis ◽  
T1 Relaxation ◽  
Lv Remodeling ◽  
The Impact

Introduction: Atrial fibrillation (AF) is associated with diffuse myocardial fibrosis as quantified by cardiac magnetic resonance (CMR) using T1 mapping methods. Radiofrequency catheter ablation (RFCA) is evolving, and the role in rhythm control may be ideal for reversing left ventricular (LV) remodeling. Hypothesis: We aimed to study the impact of RFCA on diffuse myocardial fibrosis in AF patients. Methods: We retrospectively collected data from consecutive AF patients who underwent RFCA with modified Look-Locker Inversion recovery T1 mapping sequences on pre/post procedural CMR at 3.0-Tesla. Precontrast T1 relaxation time of the mid-LV short-axis view was used as an index of diffuse LV fibrosis. Primary outcome was the change in diffuse LV fibrosis after RFCA. Results: A total of 11 patients (mean age 67 years, 72% male, 67% paroxysmal AF) were enrolled. Median AF duration was 24.6 months [Interquartile range (IQR): 13.3-45.3)] and median CHA2DS2-VASc was 2 [IQR: 1-3]. Post RFCA CMR was obtained 99.5±18.1 days after the RFCA procedure. Mean precontrast T1 time was significantly lower after RFCA (1182ms vs 1158ms; p=0.0157). Conclusions: Based on our preliminary results, RFCA in AF reduces diffuse myocardial fibrosis and may play a role in reverse LV remodeling.

scholarly journals Association Between 2D- and 3D-Speckle-Tracking Longitudinal Strain and Cardiovascular Magnetic Resonance Evidence of Diffuse Myocardial Fibrosis in Heart Transplant Recipients

2021 ◽  
Vol 8 ◽  
Author(s):  
Wei Sun ◽  
Xuehua Shen ◽  
Jing Wang ◽  
Shuangshuang Zhu ◽  
Yanting Zhang ◽  
...  
Keyword(s):  
Cardiovascular Magnetic Resonance ◽  
Magnetic Resonance ◽  
Myocardial Fibrosis ◽  
Heart Transplant ◽  
Speckle Tracking ◽  
Longitudinal Strain ◽  
Myocardial Strain ◽  
Left Ventricular ◽  
Healthy Controls ◽  
2D And 3D

Objective: This study aimed to: (1) evaluate the association between myocardial fibrosis (MF) quantified by extracellular volume fraction (ECV) and myocardial strain measured by two-dimensional (2D)- and three-dimensional speckle-tracking echocardiography (3D-STE) and (2) further investigate which strain parameter measured by 2D- and 3D-STE is the more robust predictor of MF in heart transplant (HT) recipients.Methods: A total of 40 patients with HT and 20 healthy controls were prospectively enrolled. Left ventricular (LV)-global longitudinal strain (GLS), global circumferential strain (GCS), and global radial strain (GRS) were measured by 2D- and 3D-STE. LV diffuse MF was defined by cardiovascular magnetic resonance (CMR)-ECV.Results: The HT recipients had a significantly higher native T1 and ECV than healthy controls (1043.8 ± 34.0 vs. 999.7 ± 19.7 ms, p < 0.001; 26.6 ± 2.7 vs. 24.3 ± 1.8%, p = 0.02). The 3D- and 2D-STE-LVGLS and LVGCS were lower (p < 0.005) in the HT recipients than in healthy controls. ECV showed a moderate correlation with 2D-LVGLS (r = 0.53, p = 0.002) and 3D-LVGLS (r = 0.60, p < 0.001), but it was not correlated with 2D or 3D-LVGCS, or LVGRS. Furthermore, 3D-LVGLS and 2D-LVGLS had a similar correlation with CMR-ECV (r = 0.60 vs. 0.53, p = 0.670). A separate stepwise multivariate linear analysis showed that both the 2D-LVGLS (β = 0.39, p = 0.019) and 3D-LVGLS (β = 0.54, p < 0.001) were independently associated with CMR-ECV.Conclusion: CMR marker of diffuse MF was present in asymptomatic patients with HT and appeared to be associated with decreased myocardial strain by echocardiography. Both the 2D- and 3D-LVGLS were independently correlated with diffuse LVMF, which may provide an alternative non-invasive tool for monitoring the development of adverse fibrotic remodeling during the follow-up of HT recipients.

scholarly journals Evaluation of the Right Ventricle Function Conducted in Patients after Acute Myocardial Infarction, Using Threedimensional Echocardiography and Myocardial Strain Analysis. Summary of the Doctoral Thesis

2021 ◽  
Author(s):  
◽  
Žanna Pičkure ◽  
Keyword(s):  
Myocardial Infarction ◽  
Ejection Fraction ◽  
Right Ventricle ◽  
Longitudinal Strain ◽  
Myocardial Strain ◽  
St Elevation Myocardial Infarction ◽  
Right Ventricle Function ◽  
St Elevation ◽  
The Right ◽  
Ventricle Function

It is well known that dysfunction of the right ventricle in ST segment elevation myocardial infarction causes such complications as rhythm disturbances, cardiogenic shock and others. Its presence is an independent prognostic indicator of all-cause mortality, cardiovascular mortality and development of heart failure. However, in clinical practice still too little attention is paid to the evaluation of the right ventricle function, despite the new echocardiographic methods available, which are capable of providing an accurate diagnostics of the right ventricle disfunction. The purpose of this work is to evaluate changes in the systolic function of the right ventricle in patients with proven acute ST elevation myocardial infarction by threedimensional echocardiography and myocardial strain techniques, and to select the most informative echocardiographic parameters for the size and function of the right ventricle for use in everyday practice. Based on the data gained during this study, the algorithm for the evaluation of the right ventricle function in patients with acute ST elevation myocardial infarction will be developed. A healthy individuals control group and a group of patients with ST elevation myocardial infarction were formed within the study. Each participant was examined according to standart echocardiography protocol. In each case new echocardiographic right venricle function evaluation methods also were applied – a three-dimensional echocardiography with following right ventricle reconstruction, volume and ejection fraction determination, as well as myocardial longitudinal strain measurements. Based on these methods, by comparing the data to the control group results, it was possible to etermine the pathology threshold for the right ventricular ejection fraction and longitudinal strain to detect right ventricle disfunction in the case of acute myocardial infarction. Three-dimensional echocardiography and evaluation of myocardial strain are new, relatively simple, sufficiently sensitive and specific methods for the diagnosis of right ventricular dysfunction in patients with ST elevation myocardial infarction. The methods are to be introduced for use in everyday clinical practice along with the standard ehocardiography parameters, which also change in ST elevation myocardial infarction: fractional area change, tricuspid annular plane systolic excursion, and visual evaluation of segmental systolic function of the right ventricle. Among new parameters ejection fraction of the right ventricle and right ventricle free wall longitudinal strain have to be determined. When evaluating the right chamber, it should be remembered that its function deterioration can be observed in case of myocardial infarction of any localization.

scholarly journals Diffuse Myocardial Fibrosis Is a Common Feature of Sickle Cell Anemia That Is Associated with Diastolic Dysfunction and Restrictive Cardiac Physiology

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 8-8
Author(s):  
Omar Niss ◽  
Michael D. Taylor ◽  
Robert Fleck ◽  
Tarek Alsaied ◽  
Jeffrey Towbin ◽  
...  
Keyword(s):  
Ejection Fraction ◽  
Diastolic Dysfunction ◽  
Myocardial Fibrosis ◽  
Research Funding ◽  
Systolic Function ◽  
Diffuse Myocardial Fibrosis ◽  
Normal Systolic Function ◽  
Lv Ejection Fraction ◽  
Focal Fibrosis ◽  
Restrictive Physiology

Abstract Background: We have recently shown that the cardiomyopathy of sickle cell anemia (SCA) is characterized by restrictive physiology (diastolic dysfunction, left atrial [LA] enlargement and normal systolic function) superimposed on hyperdynamic features (left ventricular [LV] enlargement and eccentric hypertrophy) (JACC Cardiovasc Imaging 9:244-253;2016; PNAS 2016 in press). Similar to other restrictive cardiomyopathies, SCA-related cardiomyopathy may lead to mild, secondary pulmonary hypertension (PH) with elevated tricuspid regurgitant jet velocity (TRV), and can be complicated by arrhythmias and sudden death. Diastolic dysfunction is the principal pathology leading to restrictive physiology. Myocardial fibrosis is a common cause of non-SCA restrictive physiology, but the cause of the diastolic dysfunction that underlies SCA-related cardiomyopathy is undetermined. Focal fibrosis, as detected by late gadolinium enhancement (LGE) on cardiac magnetic resonance imaging (CMR), is rare in SCA. However, diffuse myocardial fibrosis, which is not detected by LGE, has not been studied before in SCA. Therefore, we aimed to detect myocardial fibrosis in SCA using a novel CMR T1-mapping technique to quantify the myocardial extracellular volume (ECV) fraction, which correlates with histologic diffuse fibrosis. Methods: We conducted a prospective study of children and adults with SCA (NCT02410811) who underwent CMR, echocardiography, and laboratory testing (including N-terminal pro-brain type natriuretic peptide [NT-proBNP], a marker of ventricular stress). ECV was measured from pre- and post-gadolinium T1 maps using a modified Look-Locker inversion recovery (MOLLI) sequence. Chamber sizes and cardiac performance were evaluated using CMR, while TRV and diastolic parameters were measured by echocardiography. Results: Twenty-five patients with a median age of 19 years (range 6-61 years) were evaluated. ECV was increased in all SCA patients (mean 44 ± 8% vs. 25 ± 3% in normal subjects, P<0.001). One patient had focal fibrosis by LGE and one had systolic dysfunction. Among patients with normal systolic function, 17 patients (71%) had diastolic abnormalities and 7 (29%) had normal diastolic function. Seven out of 17 patients with diastolic abnormalities (29% of the total group) met the definition of diastolic dysfunction, and 10 had inconclusive classification. Patients with diastolic dysfunction had significantly higher ECV (49 ± 7% vs 37 ± 4%, P=0.01; Panel A), NT-proBNP (191 ± 261 vs. 33 ± 33 pg/mL, P=0.04; Panel B), and lower hemoglobin (8.4±0.3 vs.10.9±1.4 g/dL, P=0.004, Panel C) compared to patients with normal diastolic function. Systolic function was similar in both groups (LV ejection fraction 61 ± 4% vs. 62 ± 3.4%, P=0.86). In patients with higher ECV (³40%), LV diastolic abnormalities were more common (99% vs 33%, P=0.003) and LA volume index was significantly increased (57 ± 11 vs. 46 ± 12 mL/m2, P=0.04) compared to patients with ECV <40%. Increased ECV was associated with anemia (R=-0.46, P=0.03; Panel D) and elevated NT-proBNP (R=0.62, P=0.001; Panel E), but not with LV ejection fraction (P=0.66; Panel F), LV mass (P=0.92) or TRV (P=0.65). Conclusions: ECV is markedly elevated in SCA, indicating the presence of significant diffuse myocardial fibrosis in all patients studied. High ECV is associated with more severe anemia, diastolic dysfunction, and high NT-proBNP. Diffuse myocardial fibrosis is a novel process underlying diastolic dysfunction and SCA-related cardiomyopathy, the features of which may be mistaken for pulmonary arterial hypertension. Identifying and therapeutically targeting the root-cause of myocardial fibrosis, or interrupting the development of myocardial fibrosis, should be studied to mitigate cardiopulmonary disease and decrease early mortality in SCA. Figure. Figure. Disclosures Quinn: Silver Lake Research Corporation: Consultancy; Eli Lilly: Research Funding; Amgen: Research Funding.

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