Association of uncertain significance genetic variants with myocardial mechanics and morphometrics in patients with nonischemic dilated cardiomyopathy.

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.

Left atrium volume and function changes during stress in patients with primary mitral regurgitation and preserved left ventricular ejection fraction.

INTRODUCTION: Patients with primary mitral regurgitation (MR) usually remain asymptomatic for a long time due to compensatory mechanisms and an adequate treatment could be delayed. Stress echocardiography and speckle-tracking analysis could help to evaluate impaired left atrium (LA) function before the manifestation of clinically significant myocardial changes in asymptomatic patients with primary MR and preserved left ventricular (LV) ejection fraction (EF). METHODS: This study prospectively enrolled 91 patients with preserved LV EF (≥60%) at rest, of which 60 patients had moderate-to-severe MR and 31 were healthy controls. Rest and stress (bicycle ergometry) echocardiography and speckle-tracking offline analysis were performed. RESULTS: In MR group LA volume indices were higher at rest and during stress, while LA reservoir, conduit, and contractile fractions were decreased (p < .005). LA deformation parameters at rest were similar in both groups. During maximum stress LA conduit, contractile fractions and reservoir strain were lower (p < .05) in patients with MR. Indices of LA volume were related to SPAP at rest and during stress. Higher NT-proBNP concentrations was associated with higher LA volume indices, decreased contractile and reservoir functions during peak stress (p < .05). LA volume indices, LA EF, and filling index at rest could predict exercise-induced pulmonary hypertension (EIPH) (p < .05). CONCLUSIONS: In patients with primary MR and preserved LV EF, LA parameters are related to SPAP and NT-pro-BNP concentration. LA volume indices, LA EF and LA filling index are predictors of EIPH.

Acquired Cardiovascular Diseases in Patients with Pulmonary Hypertension Due to Congenital Heart Disease: A Case Report.

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.

The Prognostic Value of One-Year Changes in Biventricular Mechanics for Three-Year Survival in Patients with Precapillary Pulmonary Hypertension: A Cardiovascular Magnetic Resonance Feature Tracking Study.

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.

The association of left ventricular histologically verified myocardial fibrosis with pulmonary hypertension in severe aortic stenosis.

OBJECTIVES: To evaluate the association between histologically verified left ventricular (LV) myocardial fibrosis (MF) and its bio- and functional markers with pulmonary hypertension (PH) in severe aortic stenosis (AS). METHODS: About 34 patients with isolated severe AS underwent 2D echocardiography, cardiac magnetic resonance (CMR) imaging, and plasma NT-proBNP evaluation before aortic valve replacement (AVR). LV measurements were analyzed by CMR and LV strain using feature tracking software (Medis Suite QStrain 2.0). Myocardial biopsy sampled at the time of AVR was assessed by a histomorphometric analysis. PH was defined as pulmonary artery systolic pressure (PASP) ⩾ 45 mm Hg. RESULTS: Patients with severe AS and PH (mean PASP 53 ± 3.7 mm Hg) had higher extent of diffuse MF versus patients without PH (12 (10.4-12.7)% vs 6.6 (4.6-8.2)% (p = 0.00)). The extent of diffuse MF correlated with LV dilatation (r = 0.7, p = 0.02), indices of LV dysfunction (lower ejection fraction (r = -0.6, p < 0.001), global longitudinal (r = -0.5, p = 0.02) and circumferential strain (r = -0.5, p = 0.05), elevated NT-proBNP (r = 0.5, p = 0.005) and elevated PASP (r = 0.6, p < 0.001)). Histological MF > 10% (AUC 94.9%), LV global longitudinal strain > -15.5% (AUC 86.3%), and NT-proBNP > 2090 ng/l (AUC 85.1%) were independent predictors of PH in severe AS. CONCLUSIONS: The extent of diffuse myocardial fibrosis in combination with reduced longitudinal left ventricular strain and increased plasma levels of NT-proBNP relates to pulmonary hypertension in severe aortic stenosis.

Surgical dilemma - spare or replace regurgitant aortic valve: Late comparative outcomes of two strategies.

BACKGROUND: To evaluate different aortic root surgery techniques and their contemporary clinical outcomes in patients with regurgitant aortic valve and aortic root aneurysm. METHODS: The study consisted of 141 adult patients who underwent aortic valve reimplantation (David group = 73) or aortic valve replacement surgery (Bentall group = 68) for aortic valve regurgitation (AR) and dilatation of the aortic root at our institution within the same period (April 2004-October 2016). Kaplan-Meier method was used to estimate survival and other clinically relevant outcomes between the groups. RESULTS: The completeness of clinical follow-up was 100%, with a mean time of 8.0 ± 3.8 years. Thirty-day (in-hospital) mortality rates were equivalent between groups (1.3 and 1.5%, p = 1.0). The overall survival rates at 10 years were significantly better for the David group patients comparing to Bentall group patients (95.3 ± 2.6% vs 79.7 ± 6.8%; p = 0.04) with similar freedom from AV related reoperation (94.4 ± 2.7% vs 98.5 ± 1.5%; p = 0.2). Freedom from bleeding events at 10 years was 90.7 ± 3.6% for Bentall group patients and none were observed among David group patients (p = 0.01). CONCLUSIONS: Aortic valve and root surgery can be performed with equivalent safety and efficacy using either valve-sparing (David procedure) or valve-replacing (Bentall procedure) techniques in selected patients. Furthermore, patients after the David procedure demonstrated significantly improved survival and low risk of bleeding in comparison to the Bentall procedure with an acceptable risk of reoperation at 10 years follow-up.

Effect of Short-Term Treatment with Continuous Positive Airway Pressure on Cardiopulmonary Exercise Tolerance, Pulmonary and Cardiac Function in Patients with Obstructive Sleep Apnea.

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.

Impact of Carpal Tunnel Syndrome Surgery on Early Diagnosis and Treatment of Transthyretin Cardiac Amyloidosis.

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.

Fatal pulmonary tumour thrombotic microangiopathy in patient with ovarian adenocarcinoma: review and a case report.

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.

Echocardiographic predictors of exercise induced pulmonary hypertension in patients with asymptomatic moderate to severe mitral regurgitation and preserved left ventricular ejection fraction.

BACKGROUND: The significant role of mitral regurgitation (MR) in development of pulmonary hypertension (PH) has been proved in previous studies. Experts suggest systolic pulmonary arterial pressure (SPAP) ⩾60 mmHg during exercise as a significant threshold of negative prognostic value in patients with MR. PURPOSE: The aim of this study was to evaluate the changes of SPAP and to ascertain the determinants of exercise induced pulmonary hypertension (EIPH) in patients with asymptomatic primary MR. METHODS: We performed a prospective study that included 50 patients with asymptomatic primary moderate to severe MR with preserved left ventricular ejection fraction (LV EF ⩾60%) at rest. They were divided into two groups according to the presence (PH group; n = 13) or absence (non-PH group; n = 37) of EIPH. Rest and stress (bicycle ergometry) echocardiography and speckle-tracking offline analysis were performed. RESULTS: An increment of SPAP from rest to peak stress was higher in PH group (p < 0.001). Multivariate regression analysis showed that MR effective regurgitation orifice area (EROA; p = 0.008) and regurgitant volume (RVol; p = 0.006) contributed significantly to SPAP at rest. Higher increment of MR EROA during stress and worse parameters of LV diastolic function at rest (E, A, E/e') correlated significantly with higher SPAP during peak stress and they had a major role in predicting EIPH according to univariate logistic regression analysis. In ROC analysis SPAP >33.1 mmHg at rest could predict EIPH with 84.6% sensitivity and 87.1% specificity (95%CI 0.849-1.000; p < 0.001). CONCLUSIONS: Parameters of MR severity (EROA and RVol) were significant determinants of SPAP at rest, while the increment of MR EROA during stress and parameters of resting LV diastolic function were the best predictors of significant EIPH.

The Treatment Strategy for the Atrial Septal Defect in the Presence of Severe Pulmonary Hypertension.

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.

Different Causes of Functional Tricuspid Valve Regurgitation Are Linked to Differences in Tricuspid Valve and Right-Sided Heart Geometry and Function: 3D Echocardiography Study.

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.

Changes in Left Heart Geometry, Function, and Blood Serum Biomarkers in Patients with Obstructive Sleep Apnea after Treatment with Continuous Positive Airway Pressure.

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 process­increased 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.

Global and regional right ventricular mechanics in repaired tetralogy of Fallot with chronic severe pulmonary regurgitation: a three-dimensional echocardiography study.

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 < 0.0001), rToF patients demonstrated lower absolute values of LEF (17 ± 4 vs 28 ± 4), REF (20 ± 5 vs 25 ± 4) and AEF (17 ± 5 vs 21 ± 4) than controls (p < 0.01). However, only the relative contribution of LEF to global RVEF (0.39 ± 0.09 vs 0.52 ± 0.05, p < 0.0001) was significantly decreased in rToF, whereas the contribution of REF (0.45 ± 0.08 vs 0.46 ± 0.04, 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 < 0.0001) and CS (-19.79 ± 3.3 vs -22.81 ± 3.5, p < 0.01) than controls. However, looking at the regional RV deformation, the 3D CS was lower in rToF than in controls only in the basal RV free-wall segment (p < 0.01). 3D RV LS was reduced in all RV free-wall segments in rToF (p < 0.0001), but similar to controls in the septum (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.

Changes of biventricular function after CABG surgery: does cardioplegia type matter?

AIM: we choose to evaluate, whether type of cardioplegia is an important predictor to determine biventricular function changes after CABG. METHODS: 81 patients who underwent CABG surgery and matched inclusion criteria were enrolled in this study. The exclusion criteria were acute MI within 30 days, impaired systolic left ventricle function (LVEF ⩽35%), atrial fibrillation.TTE was performed for all patients and echocardiographic parameters of biventricular geometry and function were assessed before CABG surgery, first postoperative day and 6 months after surgery. Cardioplegia type was randomly chosen. First group consisted of 49 patients (60.5%) when CC was performed and the others 32 patients (39.5%) formed the second - BC group. RESULTS: Patients' demographic characteristics were similar in both groups, except the lower rates of AH and BSA in BC group (p = 0.015, p = 0.001 respectively). Longer duration of XCT and CBP time was observed in BC group (p = 0.019 and p = 0.028). BC group patients showed more efficient right heart chambers size reduction (p = 0.001 for RV diameter; p = 0.015 for RA diameter) and better improvement of longitudinal RV function (p = 0.02 for TAPSE; p = 0.001 for RV S') 6 months after surgery when compared with CC group patients. RV global systolic function diminished in both groups postoperatively, but the reduction was higher in CC group, although the difference was significant in comparing early postoperative measurements with the late after CABG surgery (p = 0.03). Changes of LV systolic function as well as diameter of LA did not differ between groups (p = 0.165 and p = 0.279, respectively), while diastolic function improved significantly in BC group patients at the late follow-up period: E/e' decreased (p < 0.001) and e' velocity of interventricular septum augmented significantly (p < 0.001). CONCLUSION: BC is associated with better RV reverse remodelling and improvement of longitudinal RV function, as well as LV diastolic function improvement after CABG surgery.

Predictors of early mortality after surgical treatment of infective endocarditis: a single-center experience.

OBJECTIVE: Surgical management of infective endocarditis continues to be challenging and is associated with significant morbidity and mortality. The objective of our study was to determine the risk factors and conditions associated with poor early infective endocarditis surgical treatment outcomes-30-day postoperative mortality. METHODS: A total of 124 patients who underwent surgery for infective endocarditis at the Hospital of Lithuanian University of Health Sciences Kaunas Clinics from January 2010 to December 2017 were retrospectively included in this study. The primary endpoints were 30-day postoperative mortality and identification of risk factors associated with it. Secondary endpoints were early postoperative outcomes and complication rates. RESULTS: During the study period, 124 patients with infective endocarditis underwent cardiac surgery, presenting an overall 30-day postoperative mortality rate of 10.48%. Mean age was 58 ± 14.4 years with 95 (76.61%) males. Independent predictive factors of early mortality were age >63 years (odds ratio = 6.4, 95% confidence interval = 1.66-24.66, p = 0.003), body mass index >30 kg/m² (odds ratio = 7.74, 95% confidence interval = 2.20-27.27, p = 0.003), and ischemic heart disease (odds ratio, 6.6, 95% confidence interval = 1.62-26.90, p = 0.003), as well as intraoperative parameters-prolonged aortic cross-clamp >84.5 minutes (odds ratio = 3.79, 95% confidence interval = 1.10-13.08, p = 0.03) and cardiopulmonary bypass time >107.5 minutes (odds ratio = 10.0, 95% confidence interval = 1.26-79.58, p = 0.023). Staphylococcus aureus infection (odds ratio = 5.04, 95% confidence interval = 1.29-19.64, p = 0.012), infective endocarditis-related intracardiac complication such as paravalvular abscess detected by transesophageal echocardiography (odds ratio = 4.32, 95% confidence interval = 1.31-14.25, p = 0.01), and infective endocarditis complicated by septic or cardiogenic shock (odds ratio, 18.43, 95% confidence interval = 4.59-73.98, p = 0.001) were statistically significant factors for increased risk of 30-day postoperative mortality. CONCLUSION: Surgical treatment of infective endocarditis showed good results in our center. The independent predictors of 30-day postoperative mortality for patients who underwent cardiac surgery for infective endocarditis were age, body mass index, ischemic heart disease, prolonged aortic cross-clamp and cardiopulmonary bypass time, Staphylococcus aureus infection, paravalvular abscess, and septic or cardiogenic shock.

Right Ventricle Mechanics and Function during Stress in Patients with Asymptomatic Primary Moderate to Severe Mitral Regurgitation and Preserved Left Ventricular Ejection Fraction.

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.

Identification of Cardiac MRI and Bio-Marker Thresholds for One-Year Survival in Pre-Capillary Pulmonary Hypertension: Prospective Study.

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) <25.5%, left-ventricle global longitudinal strain (LV GLS) >-14.18%, and right pulmonary artery (RPA) relative area change (RAC) <19%, and severely increased NT-proBNP level >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.

Multidisciplinary approach to rare primary cardiac sarcoma: a case report and review.

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.

Early and long-term results of aortic valve sparing aortic root reimplantation surgery for bicuspid and tricuspid aortic valves.

INTRODUCTION: To evaluate early and long-term clinical outcomes following aortic valve sparing aortic root reimplantation surgery in patients with leaking bicuspid and tricuspid aortic valves. METHODS: The study consisted of 92 consecutive adult patients (tricuspid aortic valve group = 63 and bicuspid aortic valve group = 29) who underwent aortic valve sparing aortic root reimplantation surgery with or without aortic cusp repair for dilatation of the aortic root and/or aortic valve regurgitation at our institution from April 2004 to October 2016. Clinical outcomes were investigated using Kaplan-Meier and log-rank tests between groups. RESULTS: The follow-up was 100% complete with a mean time of 5.3 ± 3.3 years. The 30-day in-hospital mortality was 3.1% in tricuspid aortic valve group and 3.4% in bicuspid aortic valve group patients. The overall survival rates at 10 years did not differ between bicuspid aortic valve and tricuspid aortic valve patient groups (96.6 ± 3.3% vs. 90.3 ± 4.2%, p = 0.3). Freedom from recurrent aortic valve regurgitation (>2+) at 10 years was 90.5 ± 4.1% in tricuspid aortic valve group and 75.7 ± 8.7% in bicuspid aortic valve group (p = 0.06). Freedom from aortic valve reoperation at 10 years was 100% in tricuspid aortic valve group and 83.9 ± 7.4% in bicuspid aortic valve group (p = 0.002). CONCLUSION: Aortic valve sparing aortic root reimplantation surgery is a safe and efficient technique, providing acceptable long-term survival with low rates of valve-related complications in both tricuspid aortic valve and bicuspid aortic valve patient groups. However, aortic valve reoperation rates at 10 years follow-up were higher in bicuspid aortic valve group patients compared to tricuspid aortic valve group patients.