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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 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: 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.

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.