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.

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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 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.

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.

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.

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.

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.