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Background/Introduction Previous studies using conventional echocardiographic measurements have reported subclinical left diastolic dysfunction in patients with Marfan syndrome (MFS). Certain speckle-tracking echocardiography (STE) derived parameters have shown good correlation with invasive measurements of LV relaxation. Purpose To evaluate diastolic function in Marfan patients using STE-derived parameters along with traditional diastolic measurements and compare them with healthy controls. Methods We consecutively included 127 MFS patients with normal LV ejection fraction and no previous cardiovascular surgery or significant valvular regurgitation. We also studied 38 healthy controls (age and sex-matched). We performed LV and LA strain analysis using 2D STE (QLAB 10, Philips). Echocardiographic parameters recommended to assess diastolic function (according to ASE/EACVI 2016 guidelines) were also considered. Results Reported use of BB/ARB was 28/39% in MFS patients. According to current guidelines, all participants had normal diastolic function. MFS patients showed lower TDI e' velocities (septal and lateral) and higher average E/e' ratio compared to controls, but still within normal range. Regarding STE-derived measurements, LVGLS, EDLSR and PALS were significantly reduced in MFS. E/EDLSR ratio was higher among MFS patients, reflecting impaired LV relaxation. Interestingly, E/LVGLS was not different between both groups. Conclusion STE-derived parameters showed impaired LV relaxation in MFS patients compared to controls. Our results suggest that STE derived parameters can be markers of early diastolic dysfunction and provide a better insight into Marfan-related cardiomyopathy. STE derived parameters Funding Acknowledgement Type of funding source: Public Institution(s). Main funding source(s): Programa de Actividades de I+D de la Comunidad de Madrid

Background Evolution of left and right ventricular (LV and RV) function after heart transplantation (HT) has not been well described. Our objective was to evaluate the normal evolution of echocardiographic parameters of both ventricles and to explore if there is a link between the decrease of strain values and acute rejection (AR) or coronary allograft vasculopathy (CAV) Methods We followed 29 HT recipients with serial echocardiograms performed between 2011 and 2018, with a median follow-up of 5 years. LV global longitudinal strain (LV GLS) was analyzed by speckle tracking in 12 LV segments in 4 and 2 chamber views, and RV free wall longitudinal strain (RV free Wall LS) was measured in 4 chamber view. Acute rejection was diagnosed by EMB following our HT protocol. We take into consideration only moderate or severe rejection episodes (grade ≥2R).The presence of CAV was studied by coronariography or IVUS one year post-HT. Results As shown in the table below, LVEF was preserved from the begining of the follow up while LV GLS reached the normality in the 6th month, and both remained in normal ranges untill the 5th year. Regarding RV function, TAPSE was impaired in the early post-HT period and increased progressively and reached normality 1 year after HT. RV lateral wall LS rose during follow-up as well, reaching normal values 6 months after HT. Nevertheless, we noticed an impairment in this parameter at 5 years (−20.1±2.7, p=0.001), although it remained within normal ranges compared to guidelines reference parameters. We did not find any correlation between any parameter evaluated and the presence of AR or CAV at five years of follow-up. LV and RV function parameters LVEF LV GLS TAPSE FAC RV free wall LS Basal (14 days) 63.0±7.9 −17.2±3.6 12.1±2.9* 43.7±9.8 −19.3±4.2 3 months 65.0±8.6 −17.7±2.8 14.8±3.4* 45.3±8.2 −22.0±4.6 6 months 65.8±9.6 −18.7±3.4 16.1±3.6 44.6±9.6 −24.6±4.9* 1 year 63.5±8.1 −18.1±2.2 17.1±4.1 44.0±8.1 −26.7±7.1* 2 years 63.8±6.8 −18.3±9.0 19.4±3.7 45.3±7.9 −27.6±6.3* 5 years 64.4±7.3 −18.1±3.3 17.9±3.9 46.6±12.1 −20.1±2.8 P (Anova) 0.85 0.85 Conclusion As we show in this series of HT recipients with uneventful postoperative course, all LV and RV function parameters showed normal values 1 year after HT and manteined them during long-term follow-up. The presence of AR or CAV did not have any influence in ventricular function.

Background Elastic properties of the thoracic aorta in patients with Marfan Syndrome (MS) have already been evaluated with classic echocardiographic parameters. In the latest years the use of Speckle-Tracking (STE) ecocardiography has been widely extended. Our aim is to describe and provide new parameters of aortic deformation measured by STE in patients with MS. Methods 95 unoperated adult patients with MS and 32 healthy controls were prospectively enrolled. We measured classic parameters of the aortic root using 2D echocardiography. We calculated the posterior aortic wall systolic excursion at the sinuses of Valsalva and ascending aorta using M Mode in TDI colour; with ST 2D ecocardiography we measured the aortic strain at the sinuses of Valsalva (SV) and the anterior and posterior aortic wall displacement at the SV. Aortic distensibility was calculated using the formula: 1000 * (Ds − Dd)/Dd * 1/(Ps − Pd) in mmHg–1 (Ds: systolic and Dd: diastolic diameters, Ps systolic and Pd diastolic blood pressure). Aortic stiffness index was calculated as Ln((Ps/Pd)/(Ds-Dd)/Dd)). Results As shown in the table bellow, patients with MS had lower aortic strain, aortic anterior and posterior wall displacement and impaired aortic distensibility and stiffness index compared to healthy controls. We found a strong negative linear correlation between aortic root diameter at the SV and aortic root strain (r=−0.56, figure 1). Results of statistical analysis MS (n=95) Controls (n=32) p Age (years) 32.84±12.35 32.41±7.98 0.85 Aortic root diameter at the sinuses of Valsalva (mm) 38.82±5.35 30.92±3.65 Figure 1. Dispersion plot Conclusions Our results suggest that aortic deformation and displacement obtained by STE echocardiography is impaired in MS, showing a reduced distensibility and an increased stiffness of the aortic wall, with a strong negative correlation between aortic root dilation and aortic strain. All these parameters may be useful as additional tools for the diagnosis and follow-up of Marfan patients, and could be useful to to improve the echocardiographic evaluation of the aortic root.

Background Left Ventricular systolic disfunction has already been described in Marfan Syndrome (MS) in patients without valvular dysfunction using 2D and 3D speckle tracking echocardiography (STE). This dysfunction has been related to a more severe causal genetic mutation, which suggest the presence of a primary cardiomiopathy in these patients. Right ventricular function has been less studied so far. We sought to evaluate biventricular function in our cohort of MS patients with 2D-STE. Methods 95 unoperated adult patients with MS and 32 healthy controls were prospectively enrolled. Patients with more than mild mitral or aortic regurgitation were excluded. Using STE we obtanied left ventricular global longitudinal strain (LVGLS) from the average of 16 segments from 4,2 and 3-chamber views and RVGLS values were obtained from the average of 6 segments from the apical 4-chamber view. We also measured classic parameters of systolic biventricular function (LVEF and TAPSE). Results Compared to controls, patients with MFS had significantly lower LVGLS and RVGLS (table 1). Values obtanied for LVGLS in MS patients were at the lower limit of normality stablished in the latest cuantification guidelines, while RVGLS and RV free wall LS were slightly above the limit of normality. LVEF and TAPSE were also slightly diminished in MS patients, though the differences found were clinically not relevant. Results of statistical analysis MS (n=93) Controls (n=32) p Age (years) 32.84±12.4 32.41±7.98 0.85 Aortic Root Diameter Valsalva Sinuses (mm) 38.82±5.35 30.91±5.3 Conclusions Our study suggests that patients with MFS show lower biventricular strain compared with healthy controls. 2D-STE imaging may be useful to detect subclinical changes in cardiac function in patients with MFS and should be added to routine ecocardiographic evaluation in order to improve the follow-up and treatment of these patients.

Background/Introduction Previous studies using conventional echocardiographic measurements have reported subclinical left diastolic dysfunction in patients with Marfan syndrome (MFS). Left atrial strain (LAS) has been shown to be an accurate predictor of left ventricular diastolic dysfunction. However, there is no evidence regarding the use of LAS in MFS. Purpose To assess feasibility of LAS and compare LAS derived measurements along with traditional diastolic parameters in MFS patients vs healthy controls. Methods 46 MFS patients (normal LV ejection fraction, no previous cardiovascular surgery, no significant valvular regurgitation) vs. 20 healthy controls (age and sex-matched). We performed LAS analysis using 2D speckle-tracking (QLAB 10, Philips). LA strain was determined as the average value of the longitudinal deformation (7 segments) in the apical 4-chamber view (RR gating). Results LAS analysis was feasible in 40 MFS patients (87%). All participants had normal diastolic function according to current guidelines (ASE/EACVI 2016). MFS patients showed lower TDI e' velocities and higher average E/e' ratio, but still within normal range. Similarly, LVEF was normal but slightly reduced in MFS patients. LA strain and strain rate parameters during reservoir and conduit phase were significantly impaired in MFS patients compared to controls. MFS vs controls MFS patients (n=40) Controls (n=20) p MFS patients (n=40) Controls (n=20) p Age 33.8±12.4 34.4±8.3 0.846 Septal e' (cm/s) 9.7±2.5 11.7±2.3 0.006 Male (%) 24 (60%) 12 (60%) 1.000 Average E/e' ratio 6.8±1.5 5.5±1.1 0.002 SBP (mmHg) 120.3±12.4 120.1±9.4 0.969 TR velocity (cm/s) 208.6±21.4 201.6±22.9 0.390 DBP (mmHg) 72.0±10.1 67.1±6.2 0.069 LAVi (ml/m2) 23.5±7.1 25.5±4.8 0.260 Aortic root (mm) 40.3±4.6 31.7±3.7 Example of LA strain and strain rate Conclusion MFS patients showed a subtle impairment in diastolic function compared to controls. Although further evidence is needed, LAS derived parameters could be early markers of diastolic dysfunction in this group of patients. Acknowledgement/Funding Programa de Actividades de I+D de la Comunidad de Madrid