scholarly journals Left atrial morpho-functional changes in hypertrophic cardiomyopathy and Fabry disease: a CMR-feature tracking study

2021 ◽  
Vol 22 (Supplement_2) ◽  
Author(s):  
A Moroni ◽  
L Tondi ◽  
A Camporeale ◽  
V Milani ◽  
S Pica ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Fabry Disease ◽  
Left Atrial ◽  
Feature Tracking ◽  
Extracellular Volume ◽  
Left Ventricular ◽  
Similar Degree ◽  
Functional Changes ◽  
Lv Mass ◽  
Significant Difference

Abstract Funding Acknowledgements Type of funding sources: None. Background Left ventricular (LV) diastolic dysfunction (DD) is a hallmark of hypertrophic cardiomyopathy (HCM) and its phenocopies, such as Fabry disease (FD). Together with left atrial (LA) size, LA function is emerging as a sensitive marker of the adaptive changes to backward transmission of LV cardiac filling pressure, thus implementing DD assessment. Additionally, both HCM and FD are characterized by a primitive atrial myopathy, but LA morpho-functional changes in HCM and FD have never been directly compared. More recently, LA strain by Cardiovascular Magnetic Resonance Feature Tracking (CMR-FT) has been demonstrated to be a feasible and reproducible tool to explore LA function. Purpose To compare LA morpho-functional changes in HCM and FD and to explore their correlation with tissue alterations. Methods 15 HCM and 15 sex-, age- and LV mass index-matched FD patients underwent CMR (Magnetom Aera 1.5T, Siemens) and Doppler Echocardiography for LV diastolic function assessment (E/e’ and DD grading from 0 to 3). LA phasic function was evaluated by CMR-FT strain (Qstrain Medis). The software output included passive (εe, conduit function), active (εa, booster pump function) and total strain (εs, reservoir function), along with LA volumes and ejection fraction (EF). Late gadolinium enhancement (LGE) was quantified as a percentage of LV mass using the standard deviations (SDs) method (≥ 5 SDs). Interstitial fibrosis was assessed by extracellular volume (ECV) quantification in remote myocardium. All patients were in sinus rhythm. Results In the HCM group, the proportion of patients with DD grade 2-3 was only slightly higher than in FD (p 0.26). Accordingly, no significant difference was found in E/e’ value (p 0.78). Compared to FD, HCM patients showed more severe LA morpho-functional changes, including larger LA end-systolic volume (ESV) (113 ± 35 vs 84 ± 23 ml), lower LA EF (37 ± 7 vs 44 ± 9 %) and a greater reduction of εs (-20 ± 5 vs -25 ± 6 %) and εa (-10 ± 4 vs -15 ± 4 %) (all p < 0.05). LV size and function and the burden of fibrosis (LGE quantification and ECV) were comparable between the two groups. Interestingly, in HCM population, unlike in FD, LA morpho-functional measurements significantly correlated with tissue characterization parameters (LA ESV with LGE, r 0.56, p 0.03; εs and εa with ECV, r -0.51, p 0.05 and r -0.59, p 0.02, respectively). Conclusions LA morpho-functional alterations are much more severe in HCM compared to FD with similar degree of LV hypertrophy. A more severe atrial myopathy or different mechanisms of atrial damage in the two cardiomyopathies may explain these findings. LA CMR-FT analysis may represent a sensitive tool to discriminate between HCM and FD, although larger studies are needed to confirm this finding and the possible correlation with the occurrence of atrial arrhythmias and thromboembolic risk.

scholarly journals Effects of candesartan on left ventricular hypertrophy and fibrosis in patients with hypertrophic cardiomyopathy

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
H Maqsood ◽  
H.A Shakeel ◽  
H.F Shoukat ◽  
M.D Khan ◽  
S.A.Y Shah ◽  
...  
Keyword(s):  
Late Gadolinium Enhancement ◽  
Hypertrophic Cardiomyopathy ◽  
Interstitial Fibrosis ◽  
Angiotensin Receptor Blockers ◽  
Left Ventricular ◽  
Funding Source ◽  
Gadolinium Enhancement ◽  
Percent Change ◽  
Lv Mass ◽  
Significant Difference

Abstract Introduction Hypertrophic cardiomyopathy (HCM) is characterized by left ventricular (LV) hypertrophy in the absence of pressure overload. Manifestations of the disease include heart failure associated with diastolic dysfunction and atrial and ventricular tachyarrhythmias. Pathological features of HCM include myocyte hypertrophy, interstitial fibrosis, and myocyte disarray and are mediated by angiotensin II. Purpose This study aimed to evaluate the effects of candesartan on left ventricular (LV) hypertrophy and fibrosis in patients with hypertrophic cardiomyopathy (HCM). Methods In double-blind fashion, 30 patients (6 women, 24 men; age: 55±11 years) with HCM were randomly assigned to receive placebo (n=13) or candesartan 50 mg twice a day (n=17) for 1 year. To measure LV mass and extent of fibrosis, cardiac magnetic resonance imaging was performed at baseline and 1 year as assessed by late gadolinium enhancement. Results There was a trend toward a significant difference in the percent change in LV mass (median: +5% with placebo vs. −5% with candesartan; p=0.06). There was a significant difference in the percent change in the extent of late gadolinium enhancement, with the placebo group experiencing a larger increase (+30±27% with placebo vs. −22±44% with candesartan; p=0.03). Conclusion Our study concludes reduction of the progression of myocardial hypertrophy and fibrosis with candesartan in patients with hypertrophic cardiomyopathy. Our study population was limited so we warrant larger trials to confirm a place for angiotensin receptor blockers in the management of patients with hypertrophic cardiomyopathy. Figure 1 Funding Acknowledgement Type of funding source: Other. Main funding source(s): Self funding

P5553Right ventricular involvement in hypertrophic cardiomyopathy: insights from a tertiary centre

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
A R Pereira ◽  
I Cruz ◽  
A R Almeida ◽  
A Marques ◽  
S Alegria ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Ejection Fraction ◽  
Left Atrial ◽  
Independent Predictor ◽  
Hemodynamic Instability ◽  
Tissue Doppler ◽  
Cardiovascular Death ◽  
Left Ventricular ◽  
S Wave ◽  
Significant Difference

Abstract Introduction Hypertrophic cardiomyopathy (HCM) is the main cause of sudden cardiac death in the young and a cause of heart failure (HF) and death at any age. Nevertheless, adverse long-term outcomes are not easy to predict. Objectives To assess the prevalence and prognostic value of right ventricular (RV) involvement in patients (pts) with HCM. Methods Retrospective single-centre study of consecutive pts with HCM evaluated in a specialized consultation. Selected those submitted to cardiac magnetic resonance imaging (CMR) as the gold-standard for RV assessment. The primary endpoint (PE) was a composite of cardiovascular death, nonfatal myocardial infarction, nonfatal stroke, ventricular arrhythmias with hemodynamic instability and unplanned HF admission. Results Of a total of 181 pts, 104 fulfilled the inclusion criteria (mean age at first consultation 62.1±9.7 years, 63.5% male). Septal asymmetric phenotype was the most frequent (73.1%) and 24 pts (23.1%) had rest LV outflow tract obstruction. Mean value of maximum wall thickness was 18.8±4.6 mm. Regarding CMR parameters (Fig A), 5.8% had RV dysfunction and 2.9% RV free wall hypertrophy; no patient presented RV dilation. Late gadolinium enhancement (LGE) of joint points was observed in 47.1%. During follow-up (FU, mean 56.6±29.5 months), survival free of RV dysfunction was 94.3%. Only 5 pts developed RV compromise assessed by echocardiographic parameters: TAPSE 12.0±3.4 mm and pulsed tissue Doppler systolic annular velocity (tricuspid S') wave 7.3±0.9 cm/s. These pts were significantly older (p<0.01) and had higher values of average tissue doppler E/E' ratio at diagnosis (p<0.01). Global RV involvement (at diagnosis or during FU) were associated with increased values of indexed left atrial area (p<0.01), LV dysfunction (p=0.01), LGE of joint points (p=0.01) and higher values of NT-proBNP (p=0.01). In multivariate logistic regression, left atrial enlargement was the only independent predictor of global RV dysfunction (OR 1.9, 95% CI 1.1–3.2, p=0.01) and average E/E' ratio an independent predictor of RV dysfunction during FU (OR 1.3, 95% CI 1.1–1.5, p<0.01). PE rate was 10.6%. It was significantly higher in pts with global RV involvement and there was a significant difference in survival analysis (Fig B). Average E/E' ratio (OR 1.5, 95% CI 1.1–1.9, p=0.01) and RV ejection fraction (OR 0.8, 95% CI 0.7–0.9, p=0.01) were independent predictors of the outcome. Conclusions Although not common, RV dysfunction was associated with a higher rate of cardiovascular events. Average E/E' ratio, as a measure of left ventricular filling pressure, was a risk factor for both RV dysfunction and PE. Higher values of RV ejection fraction were protective of adverse events occurrence. Together, these results support a potential role of RV function in the risk stratification of HCM pts.

scholarly journals Effect of migalastat on cardiac involvement in Fabry disease: preliminary results from MAIORA study

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
A Camporeale ◽  
F Bandera ◽  
M Pieroni ◽  
F Pieruzzi ◽  
A Bersano ◽  
...  
Keyword(s):  
Fabry Disease ◽  
Functional Capacity ◽  
Cardiac Involvement ◽  
Extracellular Volume ◽  
Left Ventricular ◽  
Real World Data ◽  
Private Company ◽  
Native T1 ◽  
Lv Mass ◽  
Treatment Naïve

Abstract Background Fabry Disease (FD) is a rare X-linked lysosomal storage disorder. Since 2016, pharmacological chaperone Migalastat has been approved for treatment of FD patients with amenable mutations to stabilize defective forms of the enzyme α-galactosidase A. A small but significant reduction in left ventricular (LV) mass after 18 months of Migalastat treatment has been previously reported by echocardiography. However, an integrated assessment of the effect of Migalastat on cardiac involvement, combining LV morphology and tissue composition by CMR with exercise capacity by cardiopulmonary test, is lacking. Purpose To determine the effects of 18 month treatment with Migalastat on LV mass, native T1 value and functional capacity in naïve patients with genetically confirmed FD cardiomyopathy. Methods Sixteen treatment naïve FD patients (4 females, mean age 46.4±16.2) with amenable mutations and signs of cardiac involvement underwent CMR with T1 mapping and cardio-pulmonary testing before and after 18 months of migalastat therapy as a part of MAIORA Study. Cardiac involvement was defined as presence of reduced native T1 values at CMR (a surrogate of myocardial glycosphingolipid storage) and/or LV hypertrophy (LVH). Nine patients (56%, 2 females, mean age 56.4±12.7 years) had LVH at baseline. Results Migalastat treatment was well tolerated in all patients, with no serious adverse event. No change in LV mass was detected at 18 months compared to baseline (95.2 (66.0–184.0) vs 103.0 (71.0–182.0) g/m2; p=0.5516). The same result was found after stratifying patients according to presence/absence of Late Gadolinium Enhancement (LGE) (LGE+ n=8, 2 females, mean age 56.2±13.1 years). There was a trend towards an increased native septal T1 value (870.0 (848–882) vs 860.0 (833.0–875.0) ms at baseline; p 0.056) with unchanged extracellular volume (ECV) (0.26 (0.23–0.028) vs 0.26 (0.22–0.29) at baseline; p 0.276) in the overall cohort. An improvement in functional capacity with a trend towards an increase in percent-predicted peak VO2 (72.0 (61.25–80.75) vs 67.0 (45.2–79.2) at baseline; p 0.056) and a significant increase in VO2 at anaerobic threshold (14.8 (12.6–20.0) vs 13.10 (6.8–18.6) ml/kg/min at baseline; p 0.004) was reported in the total population. Conclusion In treatment naïve FD patients with amenable mutations and signs of early or overt cardiac involvement, 18-month treatment with Migalastat stabilized LV mass both in patients with and without LGE and was associated with an improvement in exercise tolerance. The trend towards an increase in T1 value associated with unchanged ECV suggests partial clearance of cardiomyocyte glycoshingolipid storage. These real-world data are consistent with a beneficial impact of migalastat on the progression of cardiac involvement in FD. FUNDunding Acknowledgement Type of funding sources: Private company. Main funding source(s): Amicus Therapeutics

Increased remote extracellular volume measured by CMR T1 mapping allows early identification of left atrial dysfunction in hypertrophic cardiomyopathy

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
L Tondi ◽  
S Pica ◽  
A Camporeale ◽  
S Figliozzi ◽  
A Bernardini ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Left Atrial ◽  
T1 Mapping ◽  
Interstitial Fibrosis ◽  
Systolic Function ◽  
Extracellular Volume ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Maximal Wall Thickness ◽  
Left Atrial Dysfunction

Abstract Background Myocardial fibrosis is a hallmark of hypertrophic cardiomyopathy (HCM). Cardiac magnetic resonance (CMR) detects replacement fibrosis (RF) through late gadolinium enhancement (LGE) and interstitial fibrosis (IF) in apparently unscarred myocardium by T1 mapping-derived increased extracellular volume (ECV). Differently from LGE, to date only few small studies have explored the clinical significance of IF in HCM and a correlation between IF and diastolic dysfunction (DD) has been proposed. However, DD detection is challenging in this population since the accuracy of standard echocardiographic parameters is controversial, especially in presence of left ventricular outflow tract obstruction (LVOTO). Left atrial (LA) dysfunction is associated with high left ventricular (LV) filling pressures and may represent an early marker of DD in HCM. Purpose To explore the correlation between IF and LA dysfunction in HCM patients with preserved systolic function. Methods 93 consecutive HCM patients with preserved EF underwent a standard CMR scan. Semi-automatic threshold-based quantification of ventricular volumes, function and mass was performed. LA volumes (LAV) and function were evaluated by CMR feature-tracking (FT) analysis. The three atrial phasic functions were analyzed: (i) passive strain (εe), (ii) active strain (εa) and (iii) total strain (εs). LGE was quantified using the standard deviations (SDs) method (≥4 SDs). IF was assessed by T1 mapping-derived ECV quantification in remote myocardium (r-ECV). A matched group of 15 healthy subjects (HS) served as controls. Results Compared to HS, HCM patients showed increased LAV (LAV max: HS 39±9ml, HCM 59±20 ml; LAV min: HS 16±4 ml, HCM 34±17 ml; p&lt;0.001), reduced LA EF (HS 61±3%, HCM 45±12%, p&lt;0.001), impaired εs (HS 40±7%, HCM 29±11%, p&lt;0.001) and εe (HS 26±7%, HCM 15±7%, p&lt;0.001). No differences in εa were observed (HS 13±4%, HCM 14±7%, p 0.56). HCM patients were divided into 2 groups according to the presence of IF, defined as r-ECV values ≥29%. The two ECV groups did not differ in terms of LV EF, LA EF, LAV, LA area, E/E', LGE, LV mass, maximal wall thickness and LVOTO (all p&gt;0.05). HCM patients with increased r-ECV showed significantly impaired LA function in terms of all three strain parameters vs. normal r-ECV group (HCM r-ECV &lt;29%: εs 31±12%, εe15±7%, εa 15±5%; HCM r-ECV≥29%: εs 24±7%, εe 12±4%, εa 12±5%; all p&lt;0.05). Conclusions In HCM patients increased r-ECV correlates with LA dysfunction, hinting towards a possible role for IF in determining altered LV relaxation and DD. LA strain in controls and HCM ECV groups Funding Acknowledgement Type of funding source: None

scholarly journals Cardiac MRI T1 mapping and extracellular volume application in hypertrophic cardiomyopathy

2021 ◽  
Vol 52 (1) ◽  
Author(s):  
Nahla D. Ali ◽  
Noha Behairy ◽  
Ahmed Kharabish ◽  
Wesam Elmozy ◽  
Ahmed Yahya Hegab ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Myocardial Fibrosis ◽  
T1 Mapping ◽  
Extracellular Volume ◽  
Left Ventricular ◽  
Diffuse Myocardial Fibrosis ◽  
Diffuse Fibrosis ◽  
Proton Relaxation ◽  
Wide Range ◽  
Significant Difference

Abstract Background Hypertrophic cardiomyopathy (HCM) is one of the commonest inheritable cardiac disorders. Being a global disease with diffuse myocardial fibrosis, it has a wide range of adverse outcomes ending with sudden cardiac death. Cardiac magnetic resonance (CMR) with late gadolinium enhancement (LGE) has become a reference standard for visualization of focal myocardial fibrosis. In the setting of less severe or more diffuse fibrosis, LGE is unlikely to reveal the presence of abnormal tissue given the lack of normal myocardium as a reference. Direct measurement of myocardial T1 time (T1 mapping) may improve these methodologic problems of LGE CMR in the setting of diffuse retention of gadolinium-based contrast material. So, we aim at this study to evaluate the clinical application of CMRI native and post-contrast T1 relaxation in assessing diffuse myocardial fibrosis non-invasively in hypertrophic cardiomyopathy. Results There was a significant difference between the percent of fibrosis detected by measuring the extracellular volume percent compared to that detected by LGE, with the former detecting fibrosis in 45.1% of the examined cardiac segments while the latter showed fibrosis in 20.9% of the cardiac segments. Also, measuring the native T1 values showed evidence of fibrosis in about 32.2% of the cardiac segments superseding the percent of fibrosis detected using the LGE alone. The ejection fraction percent showed a negative correlation with the left ventricular mass with a correlation coefficient value of − 0.139 where both interstitial and replacement fibrosis play an important role in the pathophysiology of diastolic dysfunction as well as impairing the myocardial contractility. Also, in cases of obstruction, the extracellular volume (ECV) is more likely to increase in the basal anterior and antero-septal segments as well as the basal inferior segment with P values 0.015, 0.013, and 0.045, respectively. Conclusion Diffuse fibrosis was found to be difficult to be distinguished using LGE. The unique ability of CMR to use proton relaxation times provides a quantitative measurement to detect increased interstitial volume in diffuse myocardial fibrosis. Moreover, it showed that in cases of obstruction, the segments exposed to the highest pressure are more vulnerable to the fibrotic process denoting a relationship between the pressure gradient and the adverse myocardial remodeling.

Disease-specific differences of left ventricular rotational mechanics between cardiac amyloidosis and hypertrophic cardiomyopathy

2014 ◽  
Vol 307 (5) ◽  
pp. H680-H688 ◽  
Author(s):  
Gaetano Nucifora ◽  
Daniele Muser ◽  
Giorgio Morocutti ◽  
Gianluca Piccoli ◽  
Davide Zanuttini ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Feature Tracking ◽  
Cardiac Amyloidosis ◽  
Structural Heart Disease ◽  
Left Ventricular ◽  
Lv Function ◽  
Gadolinium Enhancement ◽  
Time To Peak ◽  
Lv Mass ◽  
Rotational Mechanics

Left ventricular (LV) twist (LVT) and untwisting (LVUT) rate are global and thorough parameters of LV function. The aim of the present study was to investigate the differences in LV rotational mechanics between patients with cardiac amyloidosis (CA) and hypertrophic cardiomyopathy (HCM). Twenty consecutive patients with CA, 20 consecutive patients with HCM, and 20 consecutive subjects without evidence of structural heart disease were included. Cardiac magnetic resonance (CMR) with late gadolinium enhancement (LGE) imaging was performed to evaluate biventricular function, LV mass index, and presence/extent of LGE. Feature-tracking analysis was applied to LV basal and apical short-axis images to determine peak LVT, time to peak LVT, peak LVUT rate, and time to peak LVUT rate. Peak LVT and peak LVUT rate were significantly impaired in patients with CA compared with controls ( P < 0.05 for both). In patients with HCM, peak LVT was increased ( P < 0.05) compared with controls, whereas peak LVUT rate was preserved ( P > 0.05). Time to peak LVUT rate was significantly prolonged in patients with CA and in patients with HCM compared with controls (ANOVA P < 0.001). At multivariate analysis, age ( P = 0.007), LV ejection fraction ( P = 0.035) and extent of LGE ( P < 0.001) were independently related to peak LVT, and LV mass index ( P = 0.015) and extent of LGE ( P = 0.004) were independently related to peak LVUT rate, whereas extent of LGE ( P < 0.001) was the only variable independently related to time to peak LVUT rate. In conclusion, CA and HCM have specific behavior of LV rotational mechanics. The extent of LGE significantly influences the LV rotational mechanics.

P4429Cardiac magnetic resonance characteristics of professional athletes and hypertrophic cardiomyopathy patients in the grey zone of hypertrophy

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
C Czimbalmos ◽  
I Csecs ◽  
Z Dohy ◽  
A Toth ◽  
F Suhai ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Magnetic Resonance ◽  
Feature Tracking ◽  
Circumferential Strain ◽  
Left Ventricular ◽  
Grey Zone ◽  
Significant Difference ◽  
Lv Ejection Fraction ◽  
Healthy Athlete ◽  
Physiological Hypertrophy

Abstract Differentiation between athlete's heart and hypertrophic cardiomyopathy (HCM) may cause difficulties especially in patients in the grey zone of hypertrophy. We aimed to determine conventional cardiac magnetic resonance (CMR) parameters such as left ventricular (LV) ejection fraction (EF), BSA-corrected end-diastolic (EDVi), end-systolic and stroke volume (SVi), mass (Mi), derived CMR parameters such as maximal end-diastolic wall thickness to LVEDVi ratio (EDWT/LVEDVi), LVM to LVEDV ratio (LVM/LVEDV), and CMR based strain values (global longitudinal (GLS), radial (GRS) and circumferential strain (GCS)) in male HCM patients and athletes. We consecutively enrolled male HCM patients with only slightly elevated EDWT (13–18 mm) and highly trained healthy athletes (n=30, 18.7±1.2 training hrs/week) with marked LV hypertrophy. HCM patient group was divided into sedentary (n=30, <7 training hrs/week) and athletic HCM group (n=10; >7 h/week, 12.7±7.3 h training hrs/week). Both sedentary and athletic HCM patients showed higher LVEF, lower LVEDVi and LVESVi and higher EDWT compared to the healthy athletes. LVMi of both healthy athletes and athletic HCM patients was significantly higher than in sedentary HCM patients, respectively (98.9±11.4; 94.5±7.8 vs 78.1±14.4 /m2). EDWT/LVEDVi ratio was higher in both sedentary and athletic HCM patients compared to healthy athletes, respectively (0.19±0.04; 0.17±0.04 vs 0.11±0.02). LVM/LVEDV also showed significant difference between HCM patients and healthy athletes. GLS and GRS showed no significant difference between the three groups, GCS was higher in athletic HCM compared to healthy athletes (−20.7±2.2 vs −17.8±2.3%). Figure 1. Feature tracking analysis of a healthy athlete and a patient with HCM. CMR characteristics of athletic and sedentary HCM may fundamentally alter. Our preliminary data suggest that besides conventional CMR parameters, derived parameters such as EDWT/LVEDVi and LVM/LVEDV ratios and deformation imaging may also help the differentiation between pathological and physiological hypertrophy. Acknowledgement/Funding National Research, Development and Innovation Office (NKFIH) of Hungary (K 120277). Project no. NVKP_16-1-2016-0017

scholarly journals Association between Left Atrial Deformation and Brain Involvement in Patients with Anderson-Fabry Disease at Diagnosis

2020 ◽  
Vol 9 (9) ◽  
pp. 2741 ◽  
Author(s):  
Roberta Esposito ◽  
Camilla Russo ◽  
Ciro Santoro ◽  
Sirio Cocozza ◽  
Eleonora Riccio ◽  
...  
Keyword(s):  
Fabry Disease ◽  
Left Atrial ◽  
Brain Magnetic Resonance Imaging ◽  
White Matter Lesions ◽  
Left Ventricular ◽  
Volume Index ◽  
Inverse Association ◽  
Lv Mass ◽  
Function Impairment ◽  
And Function

Background: Anderson-Fabry disease (AFD) can induce both central nervous system white matter lesions (WMLs) and cardiac abnormalities including left atrial (LA) dysfunction. We sought to evaluate the possible interrelations of LA structure and function impairment with the presence of WMLs in AFD patients. Methods 22 AFD patients and 22 controls, matched for age and sex, underwent an echo-Doppler exam including quantification of peak atrial longitudinal strain (PALS). AFD patients underwent also a 3-T brain magnetic resonance imaging with a visual quantification of WMLs by Fazekas’ score (FS) on 3D FLAIR images. Results AFD patients had significantly higher left ventricular (LV) mass index (LVMi) and relative wall thickness, and lower PALS compared to controls. Among AFD patients, 9 showed a FS = 0, and 13 a FS > 1. AFD patients with FS ≥ 1 showed lower PALS (29.4 ± 6.7 vs. 37.2 ± 3.9%, p = 0.003) than those with FS = 0, without difference in LA volume index and LVMi. In AFD patients, FS was inversely related to PALS (r = −0.49, p < 0.0001), even after adjusting for LVMi (r = −0.43, p < 0.05). Conclusions In the absence of significant alterations in LA size, AFD patients had lower PALS compared to controls. The inverse association between PALS and presence of WMLs indicates a possible parallel early involvement of heart and brain.

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