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Abstract Background Careful interpretation of the relation between phenotype changes of the heart and gene variants detected in dilated cardiomyopathy (DCM) is important for patient care and monitoring. Objective We sought to assess the association between cardiac-related genes and whole-heart myocardial mechanics or morphometrics in nonischemic dilated cardiomyopathy (NIDCM). Methods It was a prospective study consisting of patients with NIDCM. All patients were referred for genetic testing and a genetic analysis was performed using Illumina NextSeq 550 and a commercial gene capture panel of 233 genes (Systems Genomics, Cardiac-GeneSGKit®). It was analyzed whether there are significant differences in clinical, two-dimensional (2D) echocardiographic, and magnetic resonance imaging (MRI) parameters between patients with the genes variants and those without. 2D echocardiography and MRI were used to analyze myocardial mechanics and morphometrics. Results The study group consisted of 95 patients with NIDCM and the average age was 49.7 ± 10.5. All echocardiographic and MRI parameters of myocardial mechanics (left ventricular ejection fraction 28.4 ± 8.7 and 30.7 ± 11.2, respectively) were reduced and all values of cardiac chambers were increased (left ventricular end-diastolic diameter 64.5 ± 5.9 mm and 69.5 ± 10.7 mm, respectively) in this group. It was noticed that most cases of whole-heart myocardial mechanics and morphometrics differences between patients with and without gene variants were in the genes GATAD1, LOX, RASA1, KRAS, and KRIT1. These genes have not been previously linked to DCM. It has emerged that KRAS and KRIT1 genes were associated with worse whole-heart mechanics and enlargement of all heart chambers. GATAD1, LOX, and RASA1 genes variants showed an association with better cardiac function and morphometrics parameters. It might be that these variants alone do not influence disease development enough to be selective in human evolution. Conclusions Combined variants in previously unreported genes related to DCM might play a significant role in affecting clinical, morphometrics, or myocardial mechanics parameters.

Background: While tricuspid annuloplasty (TAP) is an effective treatment option for tricuspid regurgitation (TR), understanding the echocardiographic factors contributing to recurrent TR can help in developing more effective preventive measures to reduce the rate of recurrent TR after TAP. Methods: This study was designed as a prospective observational cohort study to investigate factors contributing to recurrent TR following surgical tricuspid valve (TV) repair in patients with moderate or severe functional TR caused by left heart valvular disease, with severe mitral regurgitation as the dominant pathology. The study included 66 patients who underwent preoperative two-dimensional (2D) and three-dimensional (3D) echocardiographic assessments. Patients were divided into two groups based on TAP outcomes: the effective TAP group and the recurrent TR group. Results: The analysis revealed that 3D-derived both septal–lateral diastolic and systolic tricuspid annulus (TA) diameter (odds ratio (OR) 1.77; 95% confidence interval (CI) 1.17–2.68 and OR 1.62; 95% CI 1.14–2.29, respectively), and major axis diastolic TA diameter (OR 1.59; 95% CI 1.15–2.2) had the highest OR among all echocardiographic parameters. The further univariate analysis of predefined echocardiographic values unveiled that the combined effect of heightened 3D-measured TA major axis diastolic diameter and increased right ventricle (RV) basal diameter exhibited the highest OR at 12.8 (95% CI 2.3–72.8) for a recurrent TR. Using ROC analysis, diastolic major axis (area under the curve (AUC) 0.848; cut-off 48.5 mm), septal-lateral systolic (AUC 0.840; cut-off 43.5 mm) and diastolic (AUC 0.840; cut-off 46.5 mm) TA diameter demonstrated the highest predictive value for recurrent TR from all TV parameters. Conclusions: Recurrent moderate or severe TR after TAP is associated with preoperative TA size, right atrium and RV geometry, but not with changes of RV function. The predictive capacity of 2D-assessed echocardiographic parameters was found to be lower when compared to their corresponding 3D parameters.

The study of the pathophysiology and possible interventions for non-ST-segment elevation myocardial infarction is hindered by the lack of a reproducible pre-clinical model. Here, authors develop an ovine model to reproduce post-ischemic remodeling in non-ST myocardial infarction and reveal distinct complex sugar moieties in cellular membranes and extracellular matrix patterns in infarcted tissue.

Background: Advances in the diagnosis and treatment of congenital heart diseases (CHDs) have resulted in improved survival rates for CHD patients. Up to 90% of individuals with mild CHD and 40% with complex CHD now reach the age of 60. Previous studies have indicated an elevated risk of atherosclerotic cardiovascular disease (ASCVD) and associated risk factors, morbidity, and mortality in adults with congenital heart disease (ACHD). However, there were no comprehensive guidelines for the prevention and management of acquired cardiovascular diseases (CVDs) in ACHD populations until recently. Case presentation: A 55-year-old man with Eisenmenger syndrome and comorbidities (arterial hypertension, heart failure, dyslipidemia, hyperuricemia, and a history of pulmonary embolism (PE)) presented with progressive breathlessness. The electrocardiogram (ECG) revealed signs of right ventricle (RV) hypertrophy and overload, while echocardiography showed reduced RV function, RV overload, and severe pulmonary hypertension (PH) signs, and preserved left ventricle (LV) function. After ruling out a new PE episode, acute coronary syndrome (ACS) was diagnosed, and percutaneous intervention was performed within 24–48 h of admission. Conclusions: This case highlights the importance of increased awareness of acquired heart diseases in patients with pulmonary hypertension due to CHD.

Background and Objectives: The management of patients with pulmonary hypertension (PH) poses a considerable challenge. While baseline cardiac magnetic resonance imaging (cMRI) indices are recognized for survival prognosis in PH, the prognostic value of one-year changes in biventricular mechanics, especially as assessed using feature tracking (FT) technology, remains underexplored. This study aims to assess the predictive value of one-year change in cMRI-derived biventricular function and mechanics parameters, along with N-terminal pro-brain natriuretic peptide (NT-proBNP) levels and six-minute walking test (6MWT) results for three-year mortality in precapillary PH patients. Materials and Methods: In this retrospective study, 36 patients diagnosed with precapillary pulmonary hypertension (mPAP 55.0 [46.3–70.5] mmHg, pulmonary capillary wedge pressure 10.0 [6.0–11.0] mmHg) were included. Baseline and one-year follow-up cMRI assessments, clinical data, and NT-proBNP levels were analyzed. FT technology was utilized to assess biventricular strain parameters. Patients were categorized into survival and non-survival groups based on three-year outcomes. Statistical analyses, including univariate logistic regression and Cox regression, were performed to identify predictive parameters. Results: The observed three-year survival rate was 83.3%. Baseline right ventricle (RV) ejection fraction (EF) was significantly higher in the survival group compared to non-survivors (41.0 [33.75–47.25]% vs. 28.0 [23.5–36.3]%, p = 0.044), and values of ≤32.5% were linked to a 20-fold increase in mortality risk. RV septum longitudinal strain (LS) and RV global LS exhibited significant improvement over a one-year period in the survival group compared to the non-survival group (−1.2 [−6.4–1.6]% vs. 4.9 [1.5–6.7]%, p = 0.038 and −3.1 [−9.1–2.6]% vs. 4.5 [−2.1–8.5]%, p = 0.048, respectively). Declines in RV septum LS by ≥2.95% and in RV GLS by ≥3.60% were associated with a 25-fold and 8-fold increase in mortality risk, respectively. Conclusions: The decrease in right ventricular septal and global longitudinal strain over a one-year period demonstrates a significant predictive value and an association with an increased three-year mortality risk in patients with precapillary PH.

Background: The aim of this pilot study was to determine the potential prognostic relevance of novel multidirectional myocardial and volumetric echocardiographic parameters in patients with non-ischemic dilated cardiomyopathy (NIDCM). Methods: Multidirectional myocardial parameters (longitudinal, radial, and circumferential left-ventricular (LV) strain using speckle tracking echocardiography) and a new volumetric parameter (the sum of the mitral and tricuspid regurgitation volume (mitral–tricuspid regurgitation volume) were assessed. The cardiovascular (CV) outcome was a composite of cardiac death and hospitalization for heart failure (HF) at 1 year. Results: Approximately 102 patients were included in this pilot study. The mean LV ejection fraction (LVEF) was 28.4 ± 8.9%. During a follow-up of 1 year, the CV outcome occurred in 39 patients (10 HF deaths, and 36 hospitalizations for HF). The LV global longitudinal systolic strain (GLS) and mitral–tricuspid regurgitation volume were the main parameters that were seen to be significantly altered in the comparison of patients with events vs. those without events (GLS (absolute values) 7.4 ± 2.7% vs. 10.3 ± 2.6%; mitral–tricuspid regurgitation volume 61.1 ± 20.4 mL vs. 40.9 ± 22.9 mL, respectively; p-value < 0.01). In line with these findings, in a multivariate continuous logistic regression analysis, the GLS and mitral–tricuspid regurgitation volume were the main parameters associated with worse CV outcomes (GLS: OR 0.77 (95%CI 0.65–0.92); mitral–tricuspid regurgitation volume OR 1.09 (95%CI 1.01–1.25)), whereas the radial and circumferential LV global strain and mitral regurgitation volume and tricuspid regurgitation volume were not linked to the CV outcome. Furthermore, in a receiver operating characteristic curve analysis, a GLS cutoff of 60 mL were the identified values for the parameters associated with worse CV outcomes. Conclusions: The findings of this pilot study suggest that the GLS and a novel volumetric parameter (the sum of the mitral and tricuspid regurgitation volume) are linked to worse CV outcomes in patients with non-ischemic dilated cardiomyopathy. Hence, these promising results warrant further validation in larger studies.

Background: Obstructive sleep apnea (OSA) is a condition with a high prevalence, linked to an increased risk of cardiovascular disease as well as increased morbidity and death. CPAP is currently considered the “gold standard” treatment for OSA, but more thorough research and testing are required to assess its efficacy on cardiopulmonary function. Objectives: To evaluate pulmonary function of OSA patients, cardiopulmonary exercise tolerance test (CPET) performance, cardiac magnetic resonance imaging (MRI) parameters, and polysomnographic changes before and after 3 months of CPAP therapy. Materials and methods: A total of 34 patients diagnosed with moderate or severe OSA, as well as 17 patients as a control group for the evaluation of the cardiac MRI, were included in this study. All the subjects were obese (body mass index (BMI) > 30 kg/m2). Lung function tests, CPETs, cardiac MRIs, and polysomnography were performed at the time of the study’s enrolment before the initiation of the CPAP therapy and after 3 months of the CPAP treatment. Results: The patients‘ VO2max during the CPAP treatment tended to increase, but no statistical significance was found (before treatment it was 17.52 ± 3.79 mL/kg/min and after 3 months of treatment, it was 18.6 ± 3,4 mL/kg/min; p = 0.255). The CPAP treatment had positive effects on pulmonary ventilation at the anaerobic threshold (VEAT): 44.51 L/min (43.21%) during the baseline visit and 38.60 L/min (37.86%) after the 3-month treatment period (p = 0.028). The ventilator equivalent for the carbon dioxide slope (VE/VCO2) at peak exercise decreased from 23.47 to 20.63 (p = 0.042). The patients’ pulmonary function tests were without abnormalities and did not change after treatment. When assessing cardiac the MRIs, the RV ejection fraction was lower in the OSA group compared to that of the control subjects (53.69 ± 8.91 and 61.35 ± 9.08, p = 0.016). Both LA and RA global longitudinal strains (GLS) improved after 3 months of treatment with CPAP (20.45 ± 7.25 and 26.05 ± 14.00, p = 0.043; 21.04 ± 7.14 and 26.18 ± 7.17, p = 0.049, respectively). Additionally, it was found that CPAP therapy led to statistical improvements in RV end-diastolic volume (164.82 ± 32.57 and 180.16 ± 39.09, p = 0.042). The AHI and oxygen desaturation index (ODI) significantly changed after 3 months of the initiation of the CPAP treatment (p = 0.049 and p = 0.001, respectively). The REM sleep duration decreased, while the duration of non-REM sleep increased after treatment initiation with CPAP (p = 0.016 and p = 0.017, respectively). Conclusions: Short-term CPAP treatment improves pulmonary ventilation, sleep efficiency, and sleep architecture. Significant alterations in both atrias’ GLS and RV end-diastolic volume were observed after 3 months of treatment. Longer-term follow-up and a larger patient sample are needed to confirm the reproducibility of our results.

Background and Objectives: The aim of this study was to clarify the tricuspid valve (TV) and right ventricular (RV) geometry and function characteristics using 3D echocardiography-based analysis and to identify echocardiographic predictors for severe tricuspid regurgitation (TR) in different etiologies of functional TR (fTR). Methods and Results: The prospective study included 128 patients (median age 64 years, 57% females): 109 patients with moderate or severe fTR (69-caused by dominant left-sided valvular pathology (LSVP), 40 due to precapillary pulmonary hypertension (PH)), and 19 healthy controls. The 2D and 3D-transthoracic echocardiography analysis included TV, right atrium, RV geometry, and functional parameters. All the RV geometry parameters as well as 3D TV parameters were increased in both fTR groups when compared to controls. Higher RV diameters, length, areas, volumes, and more impaired RV function were in PH group compared to LSVP group. PH was associated with larger leaflet tenting height, volume, and more increased indices of septal-lateral and major axis tricuspid annulus (TA) diameters. LVSP etiology was associated with higher anterior-posterior TA diameter and sphericity index. Univariate and multivariate logistic regression and ROC analyses revealed that different fTR etiologies were associated with various 2D and 3D echocardiographic parameters to predict severe TR: major axis TA diameter and TA perimeter, the leaflet tenting volume had the highest predictive value in PH group, septal-lateral systolic TA diameter-in LSVP group. The 3D TA analysis provided more reliable prediction for severe fTR. Conclusions: TV and RV geometry vary in different etiologies of functional TR. Precapillary PH is related to more severe RV remodeling and dysfunction and changes of TV geometry, when compared to LSVP group. The 3D echocardiography helps to determine echocardiographic predictors of severe TR in different fTR etiologies.

Background: Cardiovascular remodeling is essential in patients with obstructive sleep apnea (OSA), and continuous positive airway pressure (CPAP) therapy could improve these processes. Two-dimensional (2D) speckle-tracking (ST) echocardiography is a useful method for subclinical biventricular dysfunction diagnosis and thus might help as an earlier treatment for OSA patients. It is still not clear which blood serum biomarkers could be used to assess CPAP treatment efficacy. Objectives: To evaluate left heart geometry, function, deformation parameters, and blood serum biomarker (galectin-3, sST2, endothelin-1) levels in patients with OSA, as well as to assess changes after short-term CPAP treatment. Materials and Methods: Thirty-four patients diagnosed with moderate or severe OSA, as well as thirteen patients as a control group, were included in the study. All the subjects were obese (body mass index (BMI) > 30 kg/m2). Transthoracic 2D ST echocardiography was performed before and after 3 months of treatment with CPAP; for the control group, at baseline only. Peripheral blood samples for the testing of biomarkers were collected at the time of study enrolment before the initiation of CPAP therapy and after 3 months of CPAP treatment (blood samples were taken just for OSA group patients). Results: The left ventricle (LV) end-diastolic diameter and volume, as well as LV ejection fraction (EF), did not differ between groups, but an increased LV end-systolic volume and a reduced LV global longitudinal strain (GLS) were found in the OSA group patients (p = 0.015 and p = 0.035, respectively). Indexed by height, higher LV MMi in OSA patients (p = 0.007) and a higher prevalence of LV diastolic dysfunction (p = 0.023) were found in this group of patients. Although left atrium (LA) volume did not differ between groups, OSA group patients had significantly lower LA reservoir strain (p < 0.001). Conventional RV longitudinal and global function parameters (S′, fractional area change (FAC)) did not differ between groups; however, RV GLS was reduced in OSA patients (p = 0.026). OSA patients had a significantly higher right atrium (RA) diameter and mean pulmonary artery pressure (PAP) (p < 0.05). Galectin-3 and sST2 concentrations significantly decreased after 3 months of CPAP treatment. Conclusions: OSA is associated with the left heart remodeling process—increased LV myocardial mass index, LV diastolic dysfunction, reduced LV and RV longitudinal strain, and reduced LA reservoir function. A short-term, 3-months CPAP treatment improves LV global longitudinal strain and LA reservoir function and positively affects blood serum biomarkers. This new indexing system for LV myocardial mass by height helps to identify myocardial structural changes in obese patients with OSA.

Atrial septal defect is one of the most common congenital heart diseases in adults. The defect often leads to volume overload in the right heart coupled with the potential risk of right heart failure and pulmonary arterial hypertension. These conditions lead to worsening in quality of life, decrease in physical capacity, and even to fatal outcomes. The main strategy for treatment of atrial septal defect is a transcatheter or surgical closure of the defect, but in patients with severe pulmonary arterial hypertension, it is recommended to manage pulmonary arterial hypertension and after that treat the defect invasively. This strategy is called “treat and repair” strategy. We present an illustrative case report of management and treatment of atrial septal defect, complicated with severe pulmonary arterial hypertension. In this case, surgical closure of the defect was contraindicated because of the high pulmonary vascular resistance. Therefore, the “treat and repair” strategy was approached. After specific medical treatment of pulmonary arterial hypertension, surgical closure of the defect was chosen and proven successful.

Cardiovascular abnormalities are well-known manifestations of a late form of syphilis – tertiary syphilis. Since the era of antibiotics, the incidence of late manifestations of syphilis has declined almost to a rare entity. The injury of aorta (the aortitis with a dilatation of aortic root and its associated complications) is the most common between all the cardiovascular lesions. A less common manifestation of syphilitic aortitis is coronary artery ostial narrowing related to aortic wall thickening. We present the case of a 37-year-old male who was treated for an acute myocardial infarction due to bilateral coronary artery ostial stenosis secondary to syphilitic aortitis. According to the multidisciplinary decision, surgical revascularization (coronary artery bypass grafting, CABG) was performed. According to dermatologist recommendation, patient postoperative cardiovascular treatment was supplemented with intramuscular doses of benzathine penicillin recommended for tertiary syphilis. Further follow-up visits were also planned to detect possible changes of the aortic wall, dynamics of aortic regurgitation or potential anastomotic restenosis due to progression of aortitis.

Eisenmenger syndrome (ES) is the most severe form of pulmonary arterial hypertension (PAH) associated with congenital heart disease. It is an extremely devastating condition with a serious impact on patients' life. Classical therapy of ES remains directed to avoid complications, such as erythrocytosis, treatment of congestive heart failure, prevention of infection, and secondary haematological abnormalities such as iron deficiency and coagulation disorders. However, the only effective treatment is heart–lung transplantation; still, morbidity and mortality after transplantation remain substantially high. Furthermore, waiting lists for heart–lung transplantation are long. Recent studies examining the use of advanced medical treatment in patients with ES have shown that it may have beneficial effects in patients with ES; however, additional studies need to be done to confirm its efficacy and appropriate clinical use. A 41-year-old female admitted to the Hospital of Lithuanian University of Health Sciences due to progressive dyspnea on minimal effort, heart failure symptoms leading to NYHA functional class III-IV. After clinical and instrumental investigations, ES secondary to unrepaired patent ductus arteriosus with severe PAH was diagnosed. Treatment with sildenafil was initiated together with the standard pharmacological therapy, and the patient was added to the waiting list for the heart and lung transplantation. After 24 months of stable condition, her clinical status deteriorated, and combination therapy (sildenafil and ambrisentan) was initiated. Clinical symptoms and exercise capacity improved, and she has been stable for 4 years thereafter. Our experience of the management of an adult patient with ES showed the benefits of treatment with advanced therapy with pulmonary vasodilators that improved the patient's quality of life and delayed the need for heart and lung transplantation.

Introduction. Turner syndrome (TS) is assigned to the rare diseases group. Morbidity and mortality of TS patients are high, particularly due to the cardiovascular disorders, so monitoring for cardiovascular complications must be ensured. The data demonstrate a strong correlation between 2-dimensional echocardiographic (2Decho) evaluation and magnetic resonance imaging (MRI); still, according to recent guidelines, MRI remains a gold standard. In this study, we aimed to compare aortic dimensions on MRI and 2Decho in TS patients. Methods. 50 TS patients (≥18 years) were enrolled into the cross-sectional study. 2Decho and MRI were performed. The measurements of the aorta were assessed in five standard positions on 2Decho and in 9 standard positions on MRI; ASI (aortic size index) of the ascending aorta was calculated since reduced adult height is observed in TS patients. Results. ASI on echocardiography strongly correlated with ASI on MRI in all positions of the ascending aorta, but significantly larger medians of ASI were found on 2Decho in all positions of the ascending aorta and arch when compared with MRI measurements. Still, the prevalence of aortic sinus dilation was significantly and more frequently (52% vs. 38%, p

Background and objectives. Mitral regurgitation (MR) is usually dynamic and increasing with exertion. Stress may provoke symptoms, cause the progression of pulmonary hypertension (PH) and unmask subclinical changes of the left and right ventricle function. The aim of this study was to evaluate changes of right ventricle (RV) functional parameters during stress and to find out determinants of RV function in patients with MR. Materials and methods. We performed a prospective study that included patients with asymptomatic primary moderate to severe MR and preserved left ventricular (LV) ejection fraction (EF) at rest (≥60%). Conventional 2D echocardiography at rest and during stress (bicycle ergometry) and offline speckle tracking analysis were performed. Results. 80 patients were included as MR (50) and control (30) groups. Conventional functional and myocardial deformation parameters of RV were similar in both groups at all stages of exercise (p > 0.05). The grade of MR (p = 0.004) and higher LV global longitudinal strain (p = 0.037) contributed significantly to the changes of tricuspid annular plane systolic excursion (TAPSE) from rest to peak stress. Changes of MR ERA from the rest to peak stress were related to RV free wall longitudinal strain (FWLS) and four chambers longitudinal stain (4CLS) at rest (p = 0.011; r = −0.459 and p = 0.001; r = −0.572, respectively). Significant correlations between LV EF, stroke volume, cardiac output and RV fractional area change, S′, TAPSE, FWLS, 4CLS were obtained. However, systolic pulmonary artery pressure and RV functional, deformation parameters were not related (p > 0.05). Conclusions. Functional parameters of LV during exercise and severity of MR were significant determinants of RV function while PH has no correlation with it in patients with primary asymptomatic moderate to severe MR.

Takotsubo cardiomyopathy is rapidly reversible heart failure syndrome that usually mimics the symptoms of acute myocardial infarction with the characteristic regional wall-motion abnormalities (classically with a virtual apical ballooning caused by hypokinetic or akinetic apical or midventricular myocardium and hypercontraction of the basal segments) and absence of obstructive coronary artery disease. TC is usually associated with identifiable emotional, psychological or physical stress event and most commonly appears in postmenopausal women. The certain pathophysiological mechanism remains unknown. However, the central hypothesis is supported by the excess of catecholamines and hyperactivity of nervous system. In the last decades the frequency of the TC diagnosis is increasing rapidly but at the initial presentation the diagnosis remains challenging due to the close similarities between TC and ST elevation myocardial infarction clinical presentations that consider TC as an important part of differential diagnosis in acute coronary syndrome.

Background and objective: Cardiovascular magnetic resonance (CMR) - based feature tracking (FT) can detect left ventricular (LV) strain abnormalities in pulmonary hypertension (PH) patients, but little is known about the prognostic value of LV function and mechanics in PH patients. The aim of this study was to evaluate LV systolic function by conventional CMR and LV global strains by CMR-based FT analysis in precapillary PH patients, thereby defining the prognostic value of LV function and mechanics. Methods: We prospectively enrolled 43 patients with precapillary PH (mean pulmonary artery pressure (mPAP) 55.91 ± 15.87 mmHg, pulmonary arterial wedge pressure (PAWP) ≤15 mmHg) referred to CMR for PH evaluation. Using FT software, the LV global longitudinal strain (GLS) and global circumferential strain (GCS), also right ventricular (RV) GLS were analyzed. Results: Patients were classified into two groups according to survival (survival/non-survival). LV GLS was significantly reduced in the non-survival group (−12.4% [−19.0–(−7.8)] vs. −18.4% [−22.5–(−15.5)], p = 0.009). By ROC curve analysis, LV GLS > −14.2% (CI: 3.229 to 37.301, p < 0.001) was found to be robust predictor of mortality in PH patients. Univariable analysis using the Cox model showed that severely reduced LV GLS > −14.2%, with good sensitivity (77.8%) and high specificity (93.5%) indicated an increase of the risk of death by 11-fold. LV GLS significantly correlated in PH patients with RV ESVI (r = 0.322, p = 0.035), RV EF (r = 0.444, p < 0.003). Conclusions: LV systolic function and LV global longitudinal strain measurements using CMR-FT correlates with RV dysfunction and is associated with poor clinical outcomes in precapillary PH patients.

Background and objectives: Chronic kidney disease (CKD) increases the risk of cardiovascular diseases even in its early stages and is associated with structural and functional cardiac abnormalities. The aim of this study was to use speckle-tracking echocardiography (STE) to evaluate left and right ventricle mechanics and function, markers of subclinical dysfunction in patients with end-stage renal disease (ESRD) undergoing haemodialysis. Methods: Patients with ESRD undergoing regular haemodialysis and with preserved left ventricle (LV) ejection fraction (EF) (n = 38) were enrolled in this retrospective study. The control group consisted of 32 age-matched persons with normal kidney function (glomerular filtration rate (GFR) >90 mL/min/1.73 m2 according to Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI)). Conventional 2D echocardiography and STE were performed in all patients. Results: 70 individuals, 31 (44.29%) males and 39 (55.71%) females, were included in the study. There were no significant differences in age, sex and body surface area between the groups. LV end diastolic diameter did not differ between the groups, while LV myocardial mass index was higher in the group of patients on haemodialysis (111.64 ± 27.99 versus 84.21 ± 16.99, p < 0.001) and LV diastolic dysfunction (LVDD) was found in 31 (81.6%) patients of this group. LV global longitudinal strain (GLS) (−22.43 ± 2.71 versus −24.73 ± 2.03, p < 0.001) and LV global circumferential strain (GCS) at the mitral valve and papillary muscles levels (−18.73 ± 3.49 versus −21.67 ± 2.22, p < 0.001; −18.64 ± 2.75 versus −20.42 ± 2.38, p = 0.005, respectively) were significantly lower in haemodialysis group patients. The parameters of the right ventricle (RV) free wall longitudinal function including RV GLS (−22.63 ± 3.04 versus −25.45 ± 2.48, p < 0.001), were reduced in haemodialysis patients compared with the controls. However, RV fractional area change (FAC) did not differ between the groups (p = 0.19). Conclusion: Patients with ESRD and preserved LV ejection fraction undergoing haemodialysis had a higher prevalence of LVDD and impaired LV longitudinal and circumferential deformation indices, as well as reduced RV longitudinal function and deformation parameters compared with the age-matched healthy controls. STE helps to detect subclinical LV and RV dysfunction in chronic haemodialysis patients.

Background: Severe aortic stenosis (AS) complicated by pulmonary hypertension (PH) is associated with poor outcomes after surgical aortic valve replacement (AVR). There is still scarce information about predictors of secondary PH in this group of patients. Objectives: The aim of this study was to investigate the prognostic impact of biomarkers together with conventional Doppler echocardiographic parameters of left ventricular diastolic function on elevated pulmonary systolic pressure (PSP) in severe AS patients before surgical AVR. Methods: Sixty patients with severe isolated AS (aortic valve area 14 (OR 6.00; 95% CI, 1.41–25.48; p = 0.009), MV DT ≤ 177.5 ms (OR 9.31; 95% CI, 2.06–41.14; p = 0.001), LA volume > 100 mL (OR 9.70; 95% CI, 1.92–49.03; p = 0.002)) and biomarkers (NT-proBNP > 4060 ng/L (OR 12.54; 95% CI, 2.80–55.99; p < 0.001) and GDF-15 > 3393 pg/mL (OR 18.33; 95% CI, 2.39–140.39; p = 0.001)) were significantly associated with elevated PSP in severe AS. Conclusions: Left ventricular diastolic dysfunction and elevated biomarkers levels could predict the development of pulmonary hypertension in patients with severe aortic stenosis. Elevation of biomarkers paired with worsening of LV diastolic dysfunction could help to stratify patients for earlier surgical treatment before the development of pulmonary hypertension.

Abstract Background Pulmonary tumour thrombotic microangiopathy (PTTM) is a fatal disease in which tumour cells embolize to the pulmonary vasculature leading to pulmonary hypertension and right heart failure. Early diagnosis is essential for timely treatment which can reduce intimal pulmonary vascular proliferation and prolong survival, improve the symptoms. Due to rare occurrences and no clear diagnostic guidelines the disorder usually is found post-mortem. We present a review of this rare disease and a case of post-mortem diagnosed pulmonary tumour thrombotic microangiopathy in a young female. Case presentation 51 years old woman presented with progressively worsening dyspnea, right ventricular failure signs and symptoms. Computerized tomography denied pulmonary embolism. 2D transthoracic echocardiography demonstrated right ventricle dilatation and dysfunction, severely increased systolic pulmonary pressure. Right heart catheterization revealed pre-capillary pulmonary hypertension with mean pulmonary artery pressure of 78 mmHg, pulmonary wedge pressure of 15 mmHg, reduced cardiac output to 1.78 L/min with a calculated pulmonary vascular resistance of 35 Wood units, and extremely low oxygen saturation (26%) in pulmonary artery. Because of worsening ascites, pelvic magnetic resonance imaging was performed, tumours in both ovaries were diagnosed. Due to the high operative risk, detailed tumour diagnosis surgically was not established. The patient developed progressive cardiorespiratory failure, unresponsive to optimal heart failure drug treatment. A postmortem morphology analyses revealed tumorous masses in pre-capillary lung vessels, right ventricle hypertrophy, ovary adenocarcinoma. Conclusions An early diagnosis of PTTM is essential. Most cases are lethal due to respiratory failure progressing rapidly. Patients with a history of malignancy, symptoms and signs implying of PH should be considered of having PTTM. If detected early enough, combination of chemotherapy with specific PH therapy is believed to be beneficial in reducing intimal proliferation and prolonging survival, along with improving the symptoms.

Abstract Background Data about the right ventricular (RV) mechanics adaptation to volume overload in patients with repaired tetralogy of Fallot (rToF) are limited. Accordingly, we sought to assess the mechanics of the functional remodeling occurring in the RV of rToF with severe pulmonary regurgitation. Methods We used three-dimensional transthoracic echocardiography (3DTE) to obtain RV data sets from 33 rToF patients and 30 age- and sex- matched controls. A 3D mesh model of the RV was generated, and RV global and regional longitudinal (LS) and circumferential (CS) strain components, and the relative contribution of longitudinal (LEF), radial (REF) and anteroposterior (AEF) wall motion to global RV ejection fraction (RVEF) were computed using the ReVISION method. Results Corresponding to decreased global RVEF (45 ± 6% vs 55 ± 5%, p 0.05) and AEF (0.38 ± 0.09 vs 0.39 ± 0.04, p > 0.05) to global RVEF was similar to controls. Accordingly, rToF patients showed lower 3D RV global LS (-16.94 ± 2.9 vs -23.22 ± 2.9, p 0.05). Conclusions 3DTE allows a quantitative evaluation of the mechanics of global RVEF. In rToF with chronic volume overload, the relative contribution of the longitudinal shortening to global RVEF is affected more than either the radial or the anteroposterior components.

Background and Objectives: Cardiac amyloidosis is an infiltrative, progressive, and restrictive cardiomyopathy that leads to heart failure, reduces life quality, and causes death. This is a multisystem disorder caused by mutations of the transthyretin protein and is associated not only with cardiac diseases or carpal tunnel syndrome but also with nerve, liver, lung, gastrointestinal tract, kidney, or eye pathologies. Carpal tunnel syndrome is an early red-flag symptom of transthyretin (TTR) cardiac amyloidosis; therefore, screening for unsuspected cardiac amyloidosis can be performed through histological testing of flexor retinaculum specimens gathered during carpal tunnel release surgery. Our case highlights that early detection and accurate diagnosis of a disease are important factors for improving clinical outcomes in patients with TTR amyloidosis. Case Summary: We report the case of a 71-year-old man who presented with bilateral carpal tunnel syndrome. Amyloid deposits were detected after carpal tunnel release surgery through histological testing of the synovial tissue. The patient was sent for a cardiological evaluation. Physical examination, laboratory tests, and the ECG revealed no significant changes. The diagnosis of amyloidosis was confirmed with multimodality imaging in the early stage, which helped to start specific medicamental treatment with the transthyretin stabilizer tafamidis. Conclusions: Our objective is to highlight the early recognition and specific medicamental treatment of cardiac amyloidosis for better patient prognosis and outcomes.

Abstract Background Aortic valve sparing surgery (AVS), in combination with aortic cusp repair (ACR), still raises many questions about the increased surgical complexity and applicability for patients with pure aortic valve regurgitation (AR). The aim of this study was to investigate our long-term outcomes and predictors of recurrent AR (> 2+) after AVS and reconstructive cusp surgery. Methods We reviewed data of 81 patients who underwent AVS (a reimplantation technique) with concomitant ACR for AR and or dilatation of the aortic root at our institution during the period from April 2004 to October 2016. On preoperative echocardiography, the majority of the patients, 70 (86.4%) presented with severe AR grade (> 3+) and 28 (34.5%) of the patients had the bicuspid phenotype. Time to event analysis (long-term survival, freedom from reoperation, and recurrence of AR > 2+) was performed with the Kaplan–Meier method. Multivariate Cox regression risk analysis was performed to identify independent predictors of recurrent AR (> 2+). The mean follow-up was 5.3 ± 3.3 years and 100% complete. Results The in-hospital (30-day) mortality rate after elective surgery was 1.2%. The overall actuarial survival rates were 92.9 ± 3.1% and 90.4 ± 3.9% at five and 10 years, respectively. Actuarial freedom from recurrent AR (> 2+) was 83.7 ± 4.5% within the cohort at five and 10 years. The cumulative freedom from all causes of cardiac reoperation was 94.2 ± 2.8% within the cohort at 10 years. Neither bleeding nor thromboembolic or permanent neurologic events were reported during follow-up. By multivariate analysis, independent predictors of reccurent AR (> 2+) were an effective height lower than 9 mm (p= 0.02) and intraoperative residual mild AR (p= 0.0001). Conclusions AVS with ACR, combined in a systematic fashion, is a safe and reproducible option with low risk of long-term valve related events and normal life expectancy for patients with pure aortic regurgitation. The competent aortic valve and effective height, not lower than 9 mm intraoperatively, are mandatory to achieve long-lasting AV competency.

Abstract Background Undifferentiated pleomorphic sarcoma is a very rare and aggressive type of primary cardiac tumors. Most cardiac sarcomas result in rapid growth and quick death. According to different sources the median survival is typically 6 to 12 months. We are presenting a case of primary cardiac sarcoma with 26 months disease free survival following cytoreductive surgery and chemotherapy. Case presentation A 48-year-old woman with progressing symptoms of dyspnea and palpitations for over 2 months was referred to a cardiologist. With the help of echocardiography and cardiovascular magnetic resonance cardiac sarcoma was suspected. Open biopsy and cytoreductive surgery were performed, complete resection of the tumor was not possible. Histology revealed undifferentiated pleomorphic sarcoma. Seven cycles of chemotherapy with Doxorubicine and Ifosfamide were completed. Cardiovascular magnetic resonance revealed a complete response – only signs of fibrosis without any signs of tumor were visible. Follow ups with echocardiography, cardiovascular magnetic resonance and chest, abdomen and pelvic computed tomography is performed every 3 months. Twenty-six months from initial diagnosis the patient is still free of recurrence of tumor with no compromises of the quality of life. Conclusion Standard chemotherapy together with cytoreductive surgery can have a complete response effect in undifferentiated pleomorphic sarcoma with unusual long-term survival.

Background and objectives: Non-invasive imaging of the heart has an important place in the diagnosis and management of pulmonary arterial hypertension (PAH). The aim of this study was to establish the thresholds of cardiac magnetic resonance imaging (CMRI)-derived biventricular deformation, function parameters, and levels of N-terminal pro brain natriuretic peptide (NT-proBNP) for the prediction of survival of pre-capillary pulmonary hypertension (PHprecap) patients. Materials and Methods: In total, 64 incident PHprecap cases, who underwent CMRI, were consecutively enrolled in a prospective cohort study. Patients underwent a systemic evaluation, including measurement of NT-proBNP, two-dimensional (2D) echocardiography, six-minute walk test (6MWT), CMRI with feature tracking (FT), and right-heart catheterization (RHC). Patients were divided into two groups according to one-year survival (survival and non-survival groups). Survival analysis was performed. Results: One-year survival was 79.6%. The distribution between age, sex, mean pulmonary artery pressure (mPAP), New York Heart Association (NYHA) functional class, and 6MWT did not differ between the groups. Survival was significantly lower in the PAH group associated with connective tissue disease (CTD-PAH), where 44% (n = 4) of patients died during the first year. Univariate analysis revealed that severely reduced right-ventricle (RV) ejection fraction (EF) −14.18%, and right pulmonary artery (RPA) relative area change (RAC) 1738 (ng/L) indicate an increased risk of death in PHprecap patients. Conclusions: Impaired RV systolic function and LV global longitudinal strain, decrease of pulmonary artery distensibility, and CTD-PAH etiology, together with high NT-proBNP level, impair prognosis in pre-capillary PH patients. These findings are important for the risk stratification and management of pre-capillary pulmonary hypertension patients.

Background and Objectives: The influence of cardiac magnetic resonance (CMR) derived left ventricular (LV) parameters on the prognosis of patients with aortic stenosis (AS) was analyzed in several studies. However, the data on the relations between the LV parameters and the development of pulmonary hypertension (PH) in severe AS is lacking. Our objectives were to evaluate the CMR-derived changes of the LV size, morphology, and function in patients with isolated severe AS and PH, and to investigate the prognostic impact of these parameters on elevated systolic pulmonary artery pressure (sPAP). Materials and Methods: Thirty patients with isolated severe AS (aortic valve area ≤1 cm2) underwent a 2D-echocardiography (2D echo) and CMR before aortic valve replacement. Indices of the LV mass and volumes and ejection fraction were analyzed by CMR. The LV global longitudinal (LV LGS) and circumferential strain (LV CS) were calculated using CMR feature tracking (CMR-FT) software (Medis Suite QStrain 2.0, Medis Medical Imaging Systems B.V., Leiden, The Netherlands). The LV fibrosis expansion was assessed using a late gadolinium enhancement sequence. PH was defined as having an estimated sPAP of ≥45 mm Hg. The statistical analysis as performed using SPSS version 23.0 (SPSS, Chicago, IL, USA) Results: 30 patients with severe AS were included in the study, 23% with severe isolated AS had PH (mean sPAP 55 ± 6.6 mm Hg). More severe LV anatomical and functional abnormalities were observed in patients with PH when compared with patients without PH—a higher LV end-diastolic volume index (EDVi) (140 [120.0−160.0] vs. 90.0 mL/m² [82.5−103.0], p = 0.04), larger LV fibrosis area (7.8 [5.6−8.0] vs. 1.3% [1.2−1.5], p = 0.005), as well as lower LV global longitudinal strain (GLS; −14.0 [−14.9−(−8.9)] vs. −21.1% [−23.4−(−17.8)], p = 0.004). By receiver−operating characteristic (ROC) curve analysis, LV EDVi > 107.7 mL/m² (Area Under the Curve (AUC) 95.7%), LV GLS < −15.5% (AUC 86.3%), and LV fibrosis area >5% (AUC 89.3) were found to be robust predictors of PH in severe AS patients. Conclusions: In patients with severe aortic stenosis, a larger end-diastolic LV volume, impaired LV global longitudinal strain, and larger LV fibrosis extent can predict the development of pulmonary hypertension.