scholarly journals P1543 The assessment of regional myocardial strain in classifying amyloid cardiomyopathy

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
P Geenty ◽  
S Shivapathan ◽  
T Deshmukh ◽  
P Brown ◽  
A Boyd ◽  
...  
Keyword(s):  
Ejection Fraction ◽  
Diastolic Dysfunction ◽  
Wall Thickness ◽  
Cardiac Amyloidosis ◽  
Longitudinal Strain ◽  
Treatment Options ◽  
Wild Type ◽  
Lv Mass ◽  
Lv Ejection Fraction ◽  
Al Amyloid

Abstract Background An infiltrative cardiomyopathy is a common manifestation of AL-amyloidosis, with cardiac involvement associated with a poor prognosis. Wild-type transthyretin amyloidosis (wt-TTR), is a distinct clinical entity occurring predominantly in men > 65 yrs, that has gained interest recently due to novel treatment options. Regional strain analysis has been shown to discriminate both forms of cardiac amyloidosis from other causes of concentric left ventricular hypertrophy, with a characteristic pattern of ‘apical sparing’. Due to the significant difference in both the course of the disease and treatment options between groups, a non-invasive echocardiographic method of determining subtype would be valuable. Aim/Method: We sought to compare traditional and novel echocardiographic parameters in a cohort of AL ( n = 80) and wild type (wt-TTR) amyloid ( n = 32) patients. All amyloid patients underwent comprehensive transthoracic echocardiography, including both conventional parameters and LV longitudinal strain. Further novel parameters were computed including the ratio of global longitudinal strain (GLS) to LV ejection fraction (LVEF), as well as GLS to indexed LV mass. Results wt-TTR patients had significantly greater LV mass (176 ± 59g/m2vs118 ± 37g/m2, p < 0.001), and worse diastolic dysfunction as expressed as E/E’ (21.5 ± 11vs17 ± 8, p = 0.04). LVEF was significantly lower in wt-TTR patients however remained in the normal range in both groups (53 ± 6%vs57 ± 6%, p = 0.001), whilst GLS was significantly reduced compared to AL-amyloid patients (11.5 ± 3.4%vs16.2 ± 4.6%, p < 0.001). LVEF:GLS was significantly higher in wt-TTR patients (4.93 ± 1.4vs3.87 ± 1.3, p = 0.001) reflecting a more profound reduction in strain with a relatively preserved ejection fraction. Similarly, the ratio of GLS to LV mass was significantly lower in wt-TTR amyloidosis (0.078 ± 0.05vs0.155 ± 0.07, p < 0.001), reflecting a more significant reduction in strain for a given wall thickness in wt-TTR patients. GLS:LV mass was the strongest discriminator between subtypes (AUC 0.82), with a cutoff of 0.09 giving a sensitivity and specificity of 71% and 80% respectively, for detecting wt-TTR. Conclusion In this cohort, patients with wt-TTR had significantly greater increase in LV wall thickness and diastolic dysfunction, which may in part reflect their increased age (77vs62). However, GLS was also significantly reduced compared to AL-amyloid, even when accounting for LV ejection fraction and LV mass, suggesting these composite parameters may have value in determining the subtype of cardiac amyloidosis.

scholarly journals P309 The use of echocardiographic parameters to predict clinical outcomes in AL-amyloidosis cardiomyopathy

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
P Geenty ◽  
S Sivapathan ◽  
T Deshmukh ◽  
P Brown ◽  
A Boyd ◽  
...  
Keyword(s):  
Ejection Fraction ◽  
Primary Endpoint ◽  
Cardiac Amyloidosis ◽  
Longitudinal Strain ◽  
Global Longitudinal Strain ◽  
Al Amyloidosis ◽  
Predictors Of Outcome ◽  
Lv Mass ◽  
Echocardiographic Parameters ◽  
Al Amyloid

Abstract Background AL-amyloidosis has a rapid clinical progression, with cardiac involvement associated with a particularly poor prognosis. Cardiac amyloidosis is diagnosed by either invasive biopsy or conventional echocardiographic parameters such as increased wall thickness, in the absence of other causes. More recently, novel parameters including 2D longitudinal strain have demonstrated diagnostic utility in a range of infiltrative cardiomyopathies including cardiac amyloidosis. Aim/Method: We sought to evaluate traditional and novel echocardiographic parameters in their ability to predict adverse outcomes in a cohort of AL-amyloid patients. 80 patients who had transthoracic echocardiograms at a single centre were included. Comprehensive echocardiographic assessment was performed, including left ventricular ejection fraction (LVEF), LV Global Longitudinal Strain (GLS), LV mass (indexed to BSA). The primary endpoint was a composite of of major adverse cardiac events (MACE) and all-cause mortality, that was assessed by interrogation of the medical records on a specified censor date. Results At a mean follow-up (time from echo to censor date) of 5.4 ± 2.6years, 38/80 (47.5%) of patients experienced the primary endpoint of MACE or death, of which 25/80 (31%) were deaths. LVEF (59 ± 5.6%vs56 ± 6.4%, p = 0.04), GLS (17.4 ± 3.9%vs14.8 ± 4.9%, p = 0.01) basal longitudinal strain (12.3 ± 3.2%vs9.6 ± 3.9%, p = 0.002), indexed LV mass (107 ± 36g/m2vs130 ± 34g/m2, p = 0.06) and E/E’ (13.7 ± 4.9vs20.6 ± 9.6, p < 0.001) were all significantly different between patients who experienced the primary endpoint and those that didn’t. The strongest predictors of outcome were E/E’ (AUC 0.74), LV mass (AUC 0.73) and the ratio GLS:LV mass (AUC 0.73). An E/E’ of 15 had a sensitivity of 71% and specificity of 69%, while an indexed LV mass of 108 had a sensitivity and specificity of 74% and 67% respectively. GLS to LV mass as a cutoff of 0.16 had a sensitivity and specificity of 70% and 69% respectively. Conclusion In a cohort of 80 patients with AL-amyloid cardiomyopathy, almost half (47.5%) reached the primary composite endpoint. Diastolic dysfunction as expressed as E/E’, and LV mass were the most powerful predictors of outcome, while global longitudinal strain and LV basal strain were also reduced, and showed superiority over LV ejection fraction in predicting prognosis.

scholarly journals 665 Longitudinal strain impairment in organic mitral regurgitation: look beyond the valve

2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Giovanni Diana ◽  
Laura Manfredonia ◽  
Monica Filice ◽  
Emanuele Ravenna ◽  
Francesca Graziani ◽  
...  
Keyword(s):  
Mitral Regurgitation ◽  
Ejection Fraction ◽  
Longitudinal Strain ◽  
Global Longitudinal Strain ◽  
Left Ventricular ◽  
Cardiac Damage ◽  
Lv Dysfunction ◽  
Lv Mass ◽  
Significant Difference ◽  
Lv Ejection Fraction

Abstract Aims Global longitudinal strain (GLS) is a hallmark of cardiac damage in mitral regurgitation (MR). GLS > −18% in patients with severe organic MR (OMR) and normal LV ejection fraction (LVEF) is an independent predictor of postoperative LV dysfunction. While it is known that GLS is impaired in less than severe functional ischaemic MR (FMR), the value of GLS in less than severe OMR is not known. We aimed to determine prevalence and determinants of any GLS impairment in OMR, in comparison to FMR. Methods We retrospectively evaluated 51 consecutive patients (33 OMR and 18 FMR) with mild-to-moderate, moderate and moderate-to-severe MR (Table*). Overall, GLS was higher in OMR than FMR (17.9±4.5 vs. 10.3±5.3, P<0.001), with rate of impairment of 45% in OMR and 89% in FMR (P= 0.0024). Results However, no significant difference was found in GLS between mild-to-moderate, moderate and moderate-to-severe MR patients within OMR (17.7±4.7 vs. 16.9±3.9 vs. 22.4±3, respectively, P>0.05), as well as FMR (9.8±6.6 vs. 10.7±5.3 vs. 10.4±5.3, respectively, P>0.05) groups. GLS correlated directly with left ventricular (LV) ejection fraction (EF) in both OMR (r=0.69, P<0.001) and FMR (r=0.90, P<0.001), and inversely with LV mass indexed for body surface area (LVMi) in both OMR (r = −0.50, P=0.005) and FMR (r = −0.48, P=0.042). While correlation with LVEF was better for FMR than OMR (Z − 1.95, P=0.026), correlation with LVMi was similar for OMR and FMR groups (Z − 0.082, P>0.05). Conclusions In patients with OMR, GLS may be reduced, despite normal LVEF, in less than severe MR. Prevalence and degree of GLS impairment in OMR is less than in FMR. In OMR, as well as in FMR, GLS impairment is independent of entity of MR, but rather correlates with LVMi, maybe reflecting impact of myocardial fibrosis derived by increased LVMi on GLS.

Prominent involvement of basal left ventricular longitudinal strain in patients with monoclonal gammopathy of undetermined significance

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
M.E Canonico ◽  
L Fiorillo ◽  
C Santoro ◽  
M Lembo ◽  
L Esposito ◽  
...  
Keyword(s):  
Ejection Fraction ◽  
Healthy Subjects ◽  
Monoclonal Gammopathy ◽  
Speckle Tracking ◽  
Cardiac Amyloidosis ◽  
Longitudinal Strain ◽  
Strain Ratio ◽  
Left Ventricular ◽  
Regional Strain ◽  
Lv Ejection Fraction

Abstract Background In cardiac amyloidosis the application of Speckle Tracking Echocardiography allows to identify a specific left ventricular (LV) longitudinal strain (LS) pattern characterized by “apical sparing”, with a prominent involvement of basal and middle segments and normal LS of apical cap. The pattern of regional LS has been never investigated in monoclonal gammopathy of undetermined significance (MGUS), a condition which can predispose to cardiac amyloidosis. Purpose To compare LV regional LS patterns and LS base-to-apex behaviour of patients affected by MGUS in comparison with healthy subjects. Methods We enrolled 40 patients affected by MGUS (M/F=20/20; age 62.6±13.8 years), asymptomatic for cardiac symptoms, and a control group of 40 healthy subjects, matched for sex and age. Nineteen (47%) MGUS patients showed prevalent free K light chain and 21 (53%) had prevalent free λ light chain. Participants underwent standard echo-Doppler exam, including Speckle Tracking of the three apical views. Global longitudinal strain (GLS), the average LS of six basal (BLS), six middle (MLS), and six apical (ALS) segments (considered in absolute values) and relative regional strain ratio RRSR [=ALS/(BLS+MLS)] were computed. Exclusion criteria were overt heart failure, LV ejection fraction <53%, coronary artery and congenital heart disease, moderate to severe valvular disease, primary cardiomyopathies, atrial fibrillation and inadequate echo imaging. Results The two groups were comparable for body mass index, blood pressure and heart rate. LV mass index, relative wall thickness, left atrial volume index and Doppler-derived LV diastolic parameters did not differ significantly between the two groups. LV ejection fraction was also similar in MGUS and healthy controls. GLS resulted significantly lower in MGUS group than in controls (20.5±3.0 vs. 22.4±2.0%, p<0.02). BLS (17.1±3.7 vs. 19.2±2.2%, p=0.004), MLS (24.9±3.8 vs. 27.1±3.6%, p<0.01) and ALS (25.1±3.8 vs. 27.1±3.5%, p<0.01) were significantly lower in MGUS than in controls. The intergroup difference of RRSR (0.60±0.05 vs. 0.58±0.04) did not achieve the statistical significance (p=0.26) and none of the MGUS patients had RRSR>1. The figure depicts a LS bull'eye of a MGUS patient showing the prominent involvement of LV basal segments. Conclusions In presence of a normal LV ejection fraction, MGUS patients show a subclinical LV longitudinal systolic dysfunction. This is testified by a reduction of GLS and of regional LS which involves mainly LV basal segments, without substantial changes of relative regional strain ratio. LV regional longitudinal dysfunction could be useful to monitor LV myocardial mechanics during follow-up of MGUS patients. LS bull's eye in a MGUS patient Funding Acknowledgement Type of funding source: None

scholarly journals Natural history and disease progression of early cardiac amyloidosis evaluated by echocardiography

2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
O Itzhaki Ben Zadok ◽  
A Eisen ◽  
Y Shapira ◽  
D Monakier ◽  
Z Iakobishvili ◽  
...  
Keyword(s):  
Disease Progression ◽  
Wall Thickness ◽  
Cardiac Amyloidosis ◽  
Time Course ◽  
Right Ventricular Dysfunction ◽  
Disease Diagnosis ◽  
Left Ventricular ◽  
Funding Sources ◽  
Lv Mass ◽  
Echocardiographic Findings

Abstract Funding Acknowledgements Type of funding sources: None. Background Since the diagnosis of cardiac amyloidosis (CA) is often delayed, echocardiographic findings are frequently indicative of already advanced cardiomyopathy. Aims to describe early echocardiographic features in patients subsequently diagnosed with CA and to delineate disease progression. Methods Pre-amyloid diagnosis echocardiographic studies were screened for structural and functional parameters and stratified according to the pathogenetic amyloid subtype (immunoglobulin light-chain (AL) or amyloid transthyretin (ATTR)). Abnormalities were defined based on published guidelines. Results Our cohort included 75 CA patients of whom 42 (56%) were diagnosed with AL and 33 (44%) with ATTR. Forty-two patients had an earlier echocardiography exam available for review. Patients presented with increased wall thickness (1.3 (IQR 1.0, 1.5)cm) ≥3 years before the diagnosis of CA and relative wall thickness (RWT) was increased (0.47 (IQR 0.41, 0.50)) ≥7 years pre-diagnosis. Between 1 to 3 years before CA diagnosis restrictive left ventricular (LV) filling pattern was present in 19% of patients and LV ejection fraction (LVEF)≤50% was present in 21% of patients. Right ventricular dysfunction was detected concomitantly with disease diagnosis. The echocardiographic phenotype of ATTR versus AL-CA showed increased RWT (0.74 (IQR 0.62, 0.92) vs. 0.62 (IQR 0.54, 0.76), p = 0.004) and LV mass index (144 (IQR 129, 191) vs. 115 (IQR 105, 146)g/m2,p = 0.020) and reduced LVEF (50 (IQR 44, 58) vs. (60 (IQR 53, 60)%, p = 0.009) throughout the time course of CA progression, albeit survival time was similar. Conclusions Increased wall thickness and diastolic dysfunction in CA develop over a time course of several years and can be diagnosed in their earlier stages by standard echocardiography Abstract Figure. Schematic proposed timeline of CA

Myocardial work in Stage 1 and 2 hypertensive patients

2021 ◽  
Author(s):  
Akshar Jaglan ◽  
Sarah Roemer ◽  
Ana Cristina Perez Moreno ◽  
Bijoy K Khandheria
Keyword(s):  
Ejection Fraction ◽  
Longitudinal Strain ◽  
Global Longitudinal Strain ◽  
Left Ventricular ◽  
Hypertensive Patients ◽  
Non Invasive ◽  
Significant Difference ◽  
Lv Ejection Fraction ◽  
Global Work ◽  
Stage 1

Abstract Aims Myocardial work (MW) is a novel parameter that can be used in a clinical setting to assess left ventricular (LV) pressures and deformation. We sought to distinguish patterns of global MW index in hypertensive vs. non-hypertensive patients and to look at differences between categories of hypertension. Methods and results Sixty-five hypertensive patients (mean age 65 ± 13 years; 30 male) and 15 controls (mean age 38 ± 12 years; 7 male) underwent transthoracic echocardiography at rest. Hypertensive patients were subdivided into Stage 1 (n = 32) and Stage 2 (n = 33) hypertension based on 2017 American College of Cardiology guidelines. Exclusion criteria were suboptimal image quality for myocardial deformation analysis, reduced ejection fraction, valvular heart disease, intracardiac shunt, and arrhythmia. Global work index (GWI), global constructive work (GCW), global wasted work (GWW), and global work efficiency were estimated from LV pressure–strain loops utilizing proprietary software from speckle-tracking echocardiography. LV systolic and diastolic pressures were estimated using non-invasive brachial artery cuff pressure. Global longitudinal strain and LV ejection fraction were preserved between the groups with no statistically significant difference, whereas there was a statically significant difference between the control and two hypertension groups in GWI (P = 0.01), GCW (P < 0.001), and GWW (P < 0.001). Conclusion Non-invasive MW analysis allows better understanding of LV response under conditions of increased afterload. MW is an advanced assessment of LV systolic function in hypertension patients, giving a closer look at the relationship between LV pressure and contractility in settings of increased load dependency than LV ejection fraction and global longitudinal strain.

scholarly journals Sex-differences in left ventricular remodeling and mechanics after aortic valve surgery in patients with severe aortic valve disease

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
G K Singh ◽  
E M Vollema ◽  
E A Prihadi ◽  
M V Regeer ◽  
S H Ewe ◽  
...  
Keyword(s):  
Sex Differences ◽  
Aortic Valve ◽  
Ejection Fraction ◽  
Aortic Valve Disease ◽  
Valve Disease ◽  
Men And Women ◽  
Lv Mass ◽  
Lv Remodeling ◽  
Lv Ejection Fraction

Abstract Background Sex-differences in left ventricular (LV) remodeling in patients with aortic valve disease have been reported. However, sex-differences in LV remodeling and mechanics in response to aortic valve replacement (AVR) remained largely unexplored. Purpose The present study aimed to evaluate the sex-differences during the time course of LV remodeling and LV mechanics (by LV global longitudinal strain (GLS)) after aortic valve replacement. Methods Patients with severe aortic valve disease (aortic stenosis (AS) or aortic regurgitation (AR)) undergoing AVR with echocardiographic follow-up at 1,2, and/or 5 years were evaluated. LV mass index, LV ejection fraction, LV GLS and stroke volume (SV) were measured. Linear mixed models analyses were used to assess changes in LV mass index, LVEF, LV GLS and SV between time points. The models were corrected for age, LV end-diastolic diameter at baseline and time between echocardiograms. Results A total of 211 patients (61±14 years, 61% male) with severe aortic valve disease (AS 63% or AR 39%) were included. Before AVR, men had larger LV mass index and higher SV compared to women. Both men and women had a preserved LV ejection fraction (54±12 and 56±9, P=0.102, respectively), but moderately impaired LV GLS (14.6±4.1 and 16.1±4.1, P=0.009, respectively). After AVR, both groups showed LV mass regression, improvement in LV ejection fraction and LV GLS. LV mass index and SV remained higher in men. During follow-up women showed significantly better LV GLS compared to men (P=0.030, figure 1). Conclusion In men and women with severe aortic valve disease undergoing AVR, the time course of changes in LV mass regression, LV ejection fraction, LV GLS and SV are similar. During follow-up LV mass index remained larger in men and women showed significantly better LV GLS. FUNDunding Acknowledgement Type of funding sources: Private grant(s) and/or Sponsorship. Main funding source(s): The department of Cardiology received unrestricted research grants from Abbott Vascular, Bayer, Bioventrix, Biotronik, Boston Scientific, Edwards Lifesciences, GE Healthcare and Medtronic. Victoria Delgado received speaker fees from Abbott Vascular, Edwards Lifesciences, GE Healthcare, MSD and Medtronic. Nina Ajmone Marsan received speakers fees from Abbott Vascular and GE healthcare. Jeroen J Bax received speaker fees from Abbott Vascular. The remaining authors have nothing to disclose.

scholarly journals Prognostic Value of Pulmonary Transit Time by Cardiac Magnetic Resonance on Mortality and Heart Failure Hospitalization in Patients With Advanced Heart Failure and Reduced Ejection Fraction

2021 ◽  
Vol 14 (1) ◽  
Author(s):  
Laura Houard ◽  
Mihaela S. Amzulescu ◽  
Geoffrey Colin ◽  
Helene Langet ◽  
Sebastian Militaru ◽  
...  
Keyword(s):  
Heart Failure ◽  
Chronic Heart Failure ◽  
Ejection Fraction ◽  
Feature Tracking ◽  
Prognostic Value ◽  
Longitudinal Strain ◽  
Global Longitudinal Strain ◽  
Reduced Ejection Fraction ◽  
Lv Ejection Fraction ◽  
Overall Mortality

Background: Pulmonary transit time (PTT) from first-pass perfusion imaging is a novel parameter to evaluate hemodynamic congestion by cardiac magnetic resonance (cMR). We sought to evaluate the additional prognostic value of PTT in heart failure with reduced ejection fraction over other well-validated predictors of risk including the Meta-Analysis Global Group in Chronic Heart Failure risk score and ischemic cause. Methods: We prospectively followed 410 patients with chronic heart failure with reduced ejection fraction (61±13 years, left ventricular (LV) ejection fraction 24±7%) who underwent a clinical cMR to assess the prognostic value of PTT for a primary endpoint of overall mortality and secondary composite endpoint of cardiovascular death and heart failure hospitalization. Normal reference values of PTT were evaluated in a population of 40 asymptomatic volunteers free of cardiovascular disease. Results PTT was significantly increased in patients with heart failure with reduced ejection fraction as compared to controls (9±6 beats and 7±2 beats, respectively, P <0.001), and correlated not only with New York Heart Association class, cMR–LV and cMR–right ventricular (RV) volumes, cMR-RV and cMR-LV ejection fraction, and feature tracking global longitudinal strain, but also with cardiac output. Over 6-year median follow-up, 182 patients died and 200 reached the secondary endpoint. By multivariate Cox analysis, PTT was an independent and significant predictor of both endpoints after adjustment for Meta-Analysis Global Group in Chronic Heart Failure risk score and ischemic cause. Importantly in multivariable analysis, PTT in beats had significantly higher additional prognostic value to predict not only overall mortality (χ 2 to improve, 12.3; hazard ratio, 1.35 [95% CI, 1.16–1.58]; P <0.001) but also the secondary composite endpoints (χ 2 to improve=20.1; hazard ratio, 1.23 [95% CI, 1.21–1.60]; P <0.001) than cMR-LV ejection fraction, cMR-RV ejection fraction, LV–feature tracking global longitudinal strain, or RV–feature tracking global longitudinal strain. Importantly, PTT was independent and complementary to both pulmonary artery pressure and reduced RV ejection fraction<42% to predict overall mortality and secondary combined endpoints. Conclusions: Despite limitations in temporal resolution, PTT derived from first-pass perfusion imaging provides higher and independent prognostic information in heart failure with reduced ejection fraction than clinical and other cMR parameters, including LV and RV ejection fraction or feature tracking global longitudinal strain. Registration: URL: https://www.clinicaltrials.gov ; Unique identifier: NCT03969394.

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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