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AL (light chain) amyloidosis is a life threatening disease. Untreated patients with involvement of the heart, a condition known as cardiac amyloidosis (CA), tend to have the most rapid disease progression and worst prognosis. Therefore, it is essential to early recognize the signs of symptoms of CA, and to identify the affected individuals with readily available non-invasive tests, as timely therapy can prolong life. Different imaging tests are used to diagnose and stratify the risk of the disease noninvasively, and to follow-up of the disease course and response to therapy. In this light, we present a case of a woman with cardiovascular risk factors, initially admitted for typical angina and decompensated heart failure (HF), who was later diagnosed with AL amyloidosis with cardiac involvement, by using multimodality imaging assessment in a step-by-step fashion. This changed completely the prognosis of the patient. Timely chemotherapy and stem cell transplantation led to an improvement in clinical status, biomarkers, and in a regression of amyloid myocardial infiltration showed by imaging.

Liver cirrhosis (LC) is an important cause of mortality. Access to liver transplantation (LT) has significantly improved the prognosis of LC. A rigorous pre-transplant cardiac evaluation is mandatory, since cardiac dysfunction is considered the main cause of mortality after LT. Notwithstanding, the most updated pre-LT evaluation guidelines provide only an algorithm for the evaluation of major cardiovascular diseases, with no specific recommendations concerning cirrhotic cardiomyopathy (CCM), which is linked to various complications in LC, especially the development of heart failure after invasive procedures and surgical interventions, including LT. CCM is characterized by a cardiac dysfunction that includes systolic and/or diastolic dysfunction and/or electrophysiological abnormalities, in the absence of other known cardiac diseases. The role of the novel methods, tissue Doppler imaging and speckle tracking echocardiography, might be essential in the early detection of cardiac dysfunction, with prognosis implications in LC. All these new methods were only recently included in the CCM diagnosis algorithm. This review summarizes the old and novel techniques used for the diagnosis of CCM, with their diagnosis and prognostic role. It also highlights the strengths and the weaknesses of the new provided CCM diagnostic consensus, and proposes a step-by- step novel diagnostic algorithm, in order to better detect cardiac dysfunction.cirrhotic cardiomyopathy

The appropriate timing of intervention and follow-up in asymptomatic patients with aortic stenosis remains controversial. Risk stratification is a key, especially with the use of a multimodality imaging approach, including exercise stress echocardiography. This review focuses on the use of exercise echocardiography in asymptomatic patients with moderate and severe aortic stenosis with preserved left ventricular ejection fraction. It describes the exercise echocardiography protocol, parameters to be evaluated, and its role in guiding the timing of intervention and follow-up in these patients.

Left ventricular non-compaction (LVNC) with preserved ejection fraction (EF) is still a controverted entity. We aimed to characterize structural and functional changes in LVNC with heart failure with preserved EF (HFpEF). Methods: We enrolled 21 patients with LVNC and HFpEF and 21 HFpEF controls. For all patients, we performed CMR, speckle tracking echocardiography (STE), and biomarker assessment for HFpEF (NT-proBNP), for myocardial fibrosis (Galectin-3), and for endothelial dysfunction [ADAMTS13, von Willebrand factor, and their ratio]. By CMR, we assessed native T1 and extracellular volume (ECV) for each LV level (basal, mid, and apical). By STE, we assessed longitudinal strain (LS), globally and at each LV level, base-to-apex gradient, LS layer by layer, from epicardium to endocardium, and transmural deformation gradient. Results: In the LVNC group, mean NC/C ratio was 2.9 ± 0.4 and the percentage of NC myocardium mass was 24.4 ± 8.7%. LVNC patients, by comparison with controls, had higher apical native T1 (1061 ± 72 vs. 1008 ± 40 ms), diffusely increased ECV (27.2 ± 2.9 vs. 24.4 ± 2.5%), with higher values at the apical level (29.6 ± 3.8 vs. 25.2 ± 2.8%) (all p < 0.01); they had a lower LS only at the apical level (−21.4 ± 4.4 vs. −24.3 ± 3.2%), with decreased base-to-apex gradient (3.8 ± 4.7 vs. 6.9 ± 3.4%) and transmural deformation gradient (3.9 ± 0.8 vs. 4.8 ± 1.0%). LVNC patients had higher NT-proBNP [237 (156–489) vs. 156 (139–257) pg/mL] and Galectin-3 [7.3 (6.0–11.5) vs. 5.6 (4.8–8.3) ng/mL], and lower ADAMTS13 (767.3 ± 335.5 vs. 962.3 ± 253.7 ng/mL) and ADAMTS13/vWF ratio (all p < 0.05). Conclusion: LVNC patients with HFpEF have diffuse fibrosis, which is more extensive at the apical level, explaining the decrease in apical deformation and overexpression of Galectin-3. Lower transmural and base-to-apex deformation gradients underpin the sequence of myocardial maturation failure. Endothelial dysfunction, expressed by the lower ADAMTS13 and ADAMTS13/vWF ratio, may play an important role in the mechanism of HFpEF in patients with LVNC.

Aims None of the conventional echocardiographic parameters alone predict increased NTproBNP level and symptoms, making diagnosis of heart failure with preserved ejection fraction (HFpEF) very difficult in some cases, in resting condition. We evaluated LA functions by 2D speckle tracking echocardiography (STE) on top of conventional parameters in HFpEF and preHF patients with diastolic dysfunction (DD), in order to establish the added value of the LA deformation parameters in the diagnosis of HFpEF. Methods We prospectively enrolled 125 patients, 88 with HFpEF (68±9 yrs), and 37 asymptomatic with similar risk factors with DD (preHF) (61±8 yrs). We evaluated them by NTproBNP, conventional DD parameters, and STE. Global longitudinal strain (GS) was added. LA reservoir (R), conduit (C), and pump function (CT) were assessed both by volumetric and STE. 2 reservoir strain (S) derived indices were also measured, stiffness (SI) and distensibility index (DI). Results LA R and CT functions were significantly reduced in HFpEF compared to preHF group (all p2 = 0.506, p Conclusions By adding LA functional analysis, we might improve the HFpEF diagnosis accuracy, compared to present guidelines. LA pump function is the only one able to differentiates preHF from HFpEF patients at rest. A value of SR_CT < -1.66/s outperformed conventional parameters from the scoring system, reservoir strain, and LA overload indices in HFpEF diagnosis. We suggest that LA function by STE could be incorporated in the current protocol for HFpEF diagnosis at rest as a major functional criterion, in order to improve diagnostic algorithm, and also in the follow-up of patients with risk factors and DD, as a prognostic marker. Future studies are needed to validate our findings.

Background In patients with heart failure with reduced ejection fraction (HFrEF), right ventricular (RV) size and dysfunction by 2-dimensional echocardiography (2DE) were identified as risk factors for mortality and morbidity, but 3-dimensional echocardiography (3DE) enabled itself as a more reproducible and accurate method. Aim To assess the comparative prognostic value of parameters of RV size and dysfunction, measured by 2DE and 3DE, in patients with ischemic and non-ischemic HFrEF, on optimal clinical care, at long-term follow-up. Methods 142 consecutive patients (62±12 yrs, 104 males) with HFrEF, in sinus rhythm, were assessed by 2DE and 3DE, including RV full-volume acquisitions. RV diameter (RVd), RV end-systolic (RV_EDA) and end-diastolic areas (RV_ESA), RV fractional area change (RVFAC), and 2D_TAPSE were measured by 2DE. RV end-diastolic (RV_EDV) and end-systolic volumes (RV_ESV), RV ejection fraction (RV_EF), and 3D_TAPSE were measured by a dedicated 3DE software. Patients were followed for 37±16 months after the index event. Primary outcome was cardiac death (CD). Secondary outcomes were: 1) HF hospitalizations (HFH); 2) a composite cardiac events (CE) end-point of CD or HFH, myocardial infarction, coronary revascularization, arrhythmias, or CRT. Results 38 CD, 47 HFH, and 62 CE occurred during follow-up. Mean RVd was 34±7 mm, RV_EDA 20±11 cm2, RV_ESA 12±5 cm2, RV_FAC 37±13%, RV_EDV 84±25 ml/m2, RV_ESV 52±22 ml/m2, and RV_EF 39±10%. Mean 2D_TAPSE was 18±4 mm, while mean 3D_TAPSE was 16±4 mm. By 2DE, only RV_ESA and RV_FAC, but not RV_EDA or RVd, correlated with CD, HFH, and CE. 2D_TAPSE correlated with HFH, but not with CD or CE, while 3D_TAPSE correlated with all primary and secondary outcomes. By 3DE, RV_ESV, but not RV_EDV, correlated with CD, HFH, and CE. Moreover, 3D RV_EF had better correlations with primary and secondary outcomes than 2D RV_FAC (z=3.8, z=2.5, and z=2.5, all p Conclusion In patients with ischemic and non-ischemic HFrEF, 3DE parameters of RV size and dysfunction are better predictors for death and re-hospitalization than 2DE parameters. The RV_EF measured by 3DE was the best predictor for death in patients with HFrEF. Funding Acknowledgement Type of funding source: None

Background Left ventricular non-compaction (LVNC) is associated with increased risk of heart failure (HF). If LVNC or hyper-trabeculation in HF with preserved ejection fraction (HFpEF) is an adaptive or stand-alone condition that contribute to generation of HF is not clearly understood yet. Aim To describe LV functional and structural parameters in HFpEF with LVNC by comparison with HFpEF without LVNC. Methods We assessed 42 patients with HFpEF, 21 with LVNC (61±9 yrs) and 21 without LVNC, age and risk factors matched (LVC), by NTproBNP, 2D echocardiography (2DE), speckle-tracking (STE), and cardiac magnetic resonance (CMR) (Figure 1). LVNC diagnosis was based on Petersen and Jacquier criteria, by the NC/C ratio and the percentage of NC myocardium. Two gradients were calculated: a base to apex gradient (LVbase-apex) and an endo-epicardial gradient (LVendo-epi). LV mass, LV end-diastolic volume (LVEDV), and T1 mapping with extracellular volume (ECV) were measured, while mean value of native T1 for apical segments (apicalT1), mean value of ECV for apical (apical ECV), and basal segments (basal ECV), and gradient between them (ECV base-apex) were calculated. Results In the LVNC, mean NC/C ratio was 2.9±0.5mm and the percentage of NC myocardium 24.4±8.8%. NTproBNP was higher in LVNC group (294±282 vs. 163±71 pg/ml, p=0.047). Functional findings were consistent with the structural changes from CMR. LVNC patients have higher native T1 in the apical segments (Table). ECV was globally expanded in LVNC compared to LVC (p=0.002) suggesting diffuse fibrosis, but the difference between groups was more relevant for apical ECV (29.6±3.9% vs 25.1±2.8%, p Conclusion Patients with HFpEF with LVNC have more fibrosis, with more severe changes in the apical segments on CMR than HFpEF without NC. They have also significantly decreased apical deformation, lower base to apex deformation gradient and lower transmural deformation gradient, due to non-compaction itself, which involves the endocardial layer. These findings suggests that NC in HFpEF is an independent condition rather than an adaptive one. Figure 1 Funding Acknowledgement Type of funding source: Public grant(s) – National budget only. Main funding source(s): Ministry of Research and Innovation, CNCS-UEFISCDI.

Background Patients with heart failure with reduced ejection fraction (HFrEF) are at high risk for mortality and morbidity. Left atrial volumes (LAVs), measured by 2-dimensional echocardiography (2DE), have been suggested as possible prognostic markers. However, LAVs measured by 2DE are prone to errors due to geometric assumptions and increased variability, whereas 3-dimensional echocardiography (3DE) has been proved to be a more accurate and reproducible method for volume assessment. Purpose To assess the comparative prognostic value of LAVs, measured by 2DE and 3DE, in patients with ischemic and non-ischemic HFrEF, on best clinical care. Methods 135 consecutive patients (59 ± 15 years, 91 males), diagnosed with HFrEF, in sinus rhythm, were assessed by 2DE and 3DE, including full-volume multi-beat acquisitions of the LA and left ventricle (LV). Maximal and minimal indexed LAVs were measured from the 2DE datasets using biplane area-length formula (2D_LAVmax and 2D_LAVmin); and from the 3DE datasets using a dedicated software package (3D_LAVmax and 3D_LAVmin). Patients were followed for 23 ± 14 months after the index event. Primary outcome was cardiac death (CD); secondary outcomes were: 1) HF hospitalization (HFH); 2) a composite cardiac events (CE) end-point, of cardiac death or hospitalization for heart failure, myocardial infarction, coronary revascularization, arrhythmias, or cardiac resynchronization therapy. Results 26 CD, 32 HFH, and 48 CE occurred during follow-up. 2DE and 3DE measurements are in the table. LAVs measured by 2DE did not correlate with outcome. However, 3D_LAVmax and 3D_LAVmin correlated with CD (r = 0.40 and r = 0.38) and CE (r = 0.30 and r = 0.29), all p Conclusion In patients with HFrEF, LA volumes measured by 3DE, but not by 2DE, were independent predictors for cardiac death and events. However, even LA volumes by 3DE did not have predictive value for future HF rehospitalizations. Echo features for patients with HFrEF 3D end-diastolic LV volume (ml/m2) 101 ± 32 3D end-systolic LV volume (ml/m2) 71 ± 29 3D LV ejection fraction (%) 30 ± 8 2D_LAVmax (ml/m2) 52 ± 18 2D_LAVmin (ml/m²) 31 ± 15 3D_LAVmax (ml/m2) 43 ± 18 3D_LAVmin (ml/m2) 29 ± 16

Funding Acknowledgements This work was supported by a grant of Ministery of Research and Innovation, CNCS-UEFISCDI, project number PN-III-P1-1-TE-2016-0669, within PNCDI III Background Cirrhotic cardiomyopathy (CCM) is defined as systolic and/or diastolic cardiac dysfunction, associated with high preload and low afterload. Thus, assessment of cardiac dysfunction in these circumstances is still debatable. Left ventricular (LV) deformation is still load-dependent, and does not reflect directly myocardial energy consumption. Since myocardial work (MW)incorporates both deformation and afterload, it might be a better alternative for the assessment of LV function in CCM. Methods 80 subjects were assessed by 2D conventional and speckle tracking echocardiography (STE): 40 patients with liver cirrhosis (LC) (58 ± 8 years, 23 males), free of any cardiovascular disease or diabetes, and 40 age and gender matched normal, control subjects. Left ventricular ejection fraction (LVEF) and systolic/diastolic blood pressure (SBP/DBP) were measured. A new approach was used to evaluate myocardial work by 2DSTE: global constructive work (GCW), as the "positive" work of the heart; global wasted work (GWW), as the "negative" work of the heart; global work efficiency (GWE), as the GCW/(GCW + GWW) in %; and global work index (GWI), as the GCW added to GWW. E/E’ ratio, left atrial volume index (LAVi), and systolic pulmonary arterial pressure (sPAP) were also assessed. Results Patients with LC had significantly lower SBP/DBP than controls, with similar LVEF (Table). GCW and GWI were decreased in patients with LC, probably due to decrease in afterload, which shifts LV work to a lower level of energy. GWE and GWW were similar to controls. By segmental analysis (18 segments model), apical and mid antero-lateral segments were the first affected in terms of myocardial work, with higher WW, low WE, but without a compensatory increase in CW in other segments, suggesting a regional myocardial dysfunction. All patients with LC presented significantly elevated E/E’ ratio, LAVi, and sPAP, compared to controls (Table). Conclusion Myocardial global constructive work and global work index decrease in LC patients, compared to normal individuals, probably due to augmented peripheral vasodilatation. Apical and mid antero-lateral segments are the first affected. Assessment of global and regional MW might be a potential new tool to assess CCM, and to understand the relationship between LV remodeling and increased filling pressure under different loading conditions. Comparative myocardial work indices group SBP (mmHg) DBP LVEF (%) E/E’ LAVI sPAP GWI GWE (% ) GCW (mmHg % ) GWW (mmHg %) LC (40) 111 ±14 69 ± 12 59 ± 7 8.5 ± 2.5 45.9 ± 14.5 26 ± 9 1927 ± 379 95 ± 2 2068 ± 386 90.1 ± 49 Controls (40) 126 ± 14 76 ± 8 61 ± 7 7.5 ± 2.2 31.8 ± 6.8 21 ± 8 2123 ± 353 95± 2 2302 ± 335 94.4 ± 49 P value 0.001 0.004 0.3 0.05 0.001 0.009 0.01 0.9 0.005 0.7 Abstract P1513 Figure. Myocardial Work Cirrhotic Cardiomyopathy

The liver has a complex vascular system, with a dual blood supply divided between the hepatic artery and the portal vein, which ultimately mixes within the hepatic sinusoids before draining into the systemic circulation via the hepatic venous system. Each of its components can be damaged by various types of injury. Vascular disorders of the liver are rare conditions, usually affecting young people, with high morbidity and mortality that can occur in the absence of a proper diagnosis and disease-specific management. In the last years, international collaborations provided data-supported approaches, which allowed to increase knowledge and awareness in understanding and management of these conditions. Given the rarity of some vascular disorders of the liver, not all will be discussed in this chapter. Definition, aetiology, diagnosis, treatment and prognosis of the most common vascular disorders of the liver will be considered. Moreover morphological and functional changes relevant to the clinical aspect are explained.

Aims To obtain the normal range for 2D echocardiographic (2DE) measurements of left ventricular (LV) layer-specific strain from a large group of healthy volunteers of both genders over a wide range of ages. Methods and results A total of 287 (109 men, mean age: 46 ± 14 years) healthy subjects were enrolled at 22 collaborating institutions of the EACVI Normal Reference Ranges for Echocardiography (NORRE) study. Layer-specific strain was analysed from the apical two-, three-, and four-chamber views using 2DE software. The lowest values of layer-specific strain calculated as ±1.96 standard deviations from the mean were −15.0% in men and −15.6% in women for epicardial strain, −16.8% and −17.7% for mid-myocardial strain, and −18.7% and −19.9% for endocardial strain, respectively. Basal-epicardial and mid-myocardial strain decreased with age in women (epicardial; P = 0.008, mid-myocardial; P = 0.003) and correlated with age (epicardial; r = −0.20, P = 0.007, mid-myocardial; r = −0.21, P = 0.006, endocardial; r = −0.23, P = 0.002), whereas apical-epicardial, mid-myocardial strain increased with the age in women (epicardial; P = 0.006, mid-myocardial; P = 0.03) and correlated with age (epicardial; r = 0.16, P = 0.04). End/Epi ratio at the apex was higher than at the middle and basal levels of LV in men (apex; 1.6 ± 0.2, middle; 1.2 ± 0.1, base 1.1 ± 0.1) and women (apex; 1.6 ± 0.1, middle; 1.1 ± 0.1, base 1.2 ± 0.1). Conclusion The NORRE study provides useful 2DE reference ranges for novel indices of layer-specific strain.

Background Left ventricular (LV) deformation is dependent on mechanical load and does not reflect directly the myocardial energy consumption. Thus, measurement of global and regional myocardial work might be alternative and complementary methods for the assessment of myocardial function. However, there is no data regarding myocardial work changes during the heart failure continuum, from normal to diastolic dysfunction (DD), and to heart failure with preserved ejection fraction (HFpEF). Methods We assessed 80 subjects by 2D conventional and speckle tracking echocardiography (2DSTE): 25 patients with DD, 30 with HFpEF, and 25 normal, control subjects. We measured NTproBNP, LV ejection fraction (EF), and E/E' ratio. We used a new approach to calculate myocardial work, during mechanical systole and isovolumetric relaxation, by 2DSTE: global constructive work (GCW), as the “positive” work of the heart; global wasted work (GWW), as the “negative” work of the heart; global work efficiency (GWE), as the GCW/(GCW + GWW) in %; and global work index (GWI), as the GCW added to GWW. Similarly, a regional, segmental analysis was performed (18 segments model) (Figure 1). Results GCW increases in patients with DD, probably as a compensatory mechanism to preserve LV function against an increased after load, and decreases back to the normal values in HFpEF, while GWE significantly decreases from normal subjects to patients with DD, and then further in patients with HFpEF (table). Meanwhile, GWW increases from normal subjects to patients with DD, and then further in patients with HFpEF. As expected, GWI does not change significantly. By segmental analysis, first segment affected in terms of myocardial work is basal antero-septal segment, with low WE and higher WW (figure), probably due to the flat shape (based on the Laplace law), with a compensatory increased CW in the apical segments. NTproBNP level and E/E' ratio correlated only with GWW (r=0.4, p=0.013). Comparative global myocardial work Group LVEF (%) E/E' NTproBNP (pg/ml) GWI (mmHg%) GWE (%) GCW (mmHg%) GWW (mmHg%) Controls 58±6 7.3±2.4 – 2102±303 95.5±1.8 2295±279 87.9±39.6 DD 57±8 7.7±2.4 36±25 2296±431 94.8±2.3 2550±463 108±50 HFpEF 63±7 10.3±3.1 349±418 2074±485 93.5±2.5 2300±535 125±51 P (Anova) 0.004 Figure 1. Myocardial Work Conclusion Myocardial work efficiency decreases and wasted work increases in parallel with the severity of LV dysfunction. The first myocardial segment affected is basal antero-septal. Therefore, new parameters of myocardial work, derived from 2DSTE, might provide a better assessment of LV function in patients with DD or HFpEF. Acknowledgement/Funding This work was supported by a grant of Ministery of Research and Innovation, CNCS-UEFISCDI, project number PN-III-P1-1-TE-2016-0669, within PNCDI III