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The article presents a case study of a 63-year-old woman with Gaucher disease (GD1) at an advanced stage of the disease. Topics include pulmonary involvement being a rare life-threatening disorder and pulmonary hypertension (PH) showing the most serious pulmonary complication in GD1; and early menopause, hip fracture and related operation, and oxygen therapy for the last 2 years due to bronchiectasis.

Pulmonary arterial hypertension (PAH) is an uncommon disorder that may be hereditable, idiopathic or associated with conditions like drug exposure, connective tissue disease, HIV infection or congenital heart disease. Familial disease are usually due to mutations in the bone morphogenic protein receptor type 2 (BMPR2), activin-like kinase-type 1 (ALK1) and endoglin (ENG). Functional and structural changes in the pulmonary vasculature lead to increased pulmonary vascular resistance. Vascular remodeling involves endothelial dysfunction, activation of fibroblasts and smooth muscle cells and recruitment of circulating progenitor cells. Vasoconstriction has also been shown to affect the remodeling process. Genetics, cellular and molecular basis of PAH are discussed in the paper. [ABSTRACT FROM AUTHOR]

Background: Pulmonary hypertension guidelines recommend invasive right heart catheterization for diagnosis and clinical follow-up. Our aim was to compare non-invasive impedance cardiography with invasive techniques for cardiac index measurements and mortality prediction in patients with pulmonary hypertension. Methods: Between 2008 and 2018, 284 right heart catheterizations were performed for the diagnosis of pulmonary hypertension in 215 patients with mean pulmonary artery pressure >25 mm Hg, and at least 2 methods used for cardiac output measurement were included in the study retrospectively. Patients were evaluated with Pearson’s correlation in 3 groups: estimated Fick (eFick) method and thermodilution (group 1), eFick method and impedance cardiography (group 2), and thermodilution and impedance cardiography (group 3). We also compared the predictive power of cardiac index measured by different methods for 1-year overall mortality and hospitalizations. Results: There were strong and moderate positive correlations in groups 1 and 3, respectively (r=0.634, P < .001, r=0.534, P=.001), and the weakest correlation was in group 2 (r=0.390, P=.001). The mean difference (bias) between eFick method versus impedance cardiography, impedance cardiography vs. thermodilution, and eFick method vs. thermodilution was 0.6 mL/min, 0.47 mL/min, and −0.2 mL/min respectively, but limits of agreement were wide. In both groups, cardiac index <2.5 L/min/m² as measured by thermodilution significantly predicted 1-year mortality. Also, impedance cardiography was better than eFick method in predicting mortality (P=.02). Conclusions: Our single-center real-life data showed that for cardiac output and cardiac index measurements, impedance cardiography provides a moderate correlation with thermodilution and is fair with eFick method methods. Moreover, thermodilution appeared superior to both eFick method and impedance cardiography, while impedance cardiography was even better than eFick method in predicting 1-year adverse events, including total mortality and hospitalization, in patients with pulmonary hypertension. [ABSTRACT FROM AUTHOR]

Objective: The purpose of this study is to compare the analysis of right ventricular (RV) free wall strain via 2D speckle tracking echocardiography with conventional echocardiography and clinical parameters in patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) receiving specific treatment. This study also aims to describe the differences between patients with repaired and unrepaired defects. Methods: This prospective study included 44 adult patients with PAH-CHD who were receiving PAH-specific treatment in a single center. This study excluded patients with complex congenital heart disease. The authors studied the conventional echocardiographic parameters, such as RV fractional area change (FAC), tricuspid annular plane systolic excursion (TAPSE), right atrial (RA) area, Tricuspid S', and hemodynamic parameters, such as functional class, 6-minute walking distance (6MWD), and N-terminal pro-brain natriuretic peptide (NT-proBNP) levels. Results: The mean age of participants was 33.8±11.6 years, and 65.9% of participants were female. The mean RV free wall strain was -14.8±4.7%. Majority of the patients belonged to WHO functional class 2 (61.4%) with a mean NT-proBNP level of 619.2±778.4 and mean 6MWD of 400.2±86.9 meters. During the follow-up of 30.8±9.0 months, 6 patients (13.6%) developed clinical right heart failure, whereas 9 (20.5%) of them died. There was a positive and significant correlation between RV free wall strain and WHO functional class (r=0.320, p=0.03), whereas there was a negative correlation between RV free wall strain and FAC (r=-0.392, p=0.01), TAPSE (r=-0.577, p=0.0001), and Tricuspid S' (r=-0.489, p=0.001). There was no significant correlation of RV free wall strain with either RA area or 6MWD. Patients with repaired congenital heart defects had worse RV functional parameters and RV free wall strain than patients with unrepaired defects. Conclusion: The assessment of RV free wall strain via 2D speckle tracking echocardiography is a feasible method and correlates well with conventional echocardiography and clinical parameters in patients with PAH-CHD receiving specific treatment. [ABSTRACT FROM AUTHOR]

Background/aim: Pulmonary arterial hypertension (PAH) is characterized by maladaptation of pulmonary vasculature which is leading to right ventricular hypertrophy and heart failure. miRNAs play a crucial role in the regulation of many diseases such as viral infection, cancer, cardiovascular diseases, and pulmonary hypertension (PH). In this study, we aimed to investigate the expression pattern of eight human plasma miRNAs (hsa-miR-21-3p, hsa-miR-143- 3p, hsa-miR-138-5p, hsa-miR-145-3p, hsa-miR-190a, hsa-miR-204-3p, hsamiR-206, hsa-miR-210-3p) in mild-to-severe PH patients and healthy controls. Materials and methods: miRNAs were extracted from the peripheral plasma of the PH patients (n: 44) and healthy individuals (n: 30) by using the miRNA Isolation Kit. cDNA was synthesized using All in-One First strand cDNA Synthesis Kit. Expression of the human plasma hsa-miR- 21-3p, hsa-miR-143-3p, hsa-miR-138-5p, hsa-miR-145-3p, hsa-miR-190a, hsa-miR-204- 3p, hsa-miR-206, hsa-miR210-3p, and miRNAs were analyzed by qRT-PCR. Results: According to our results, in PH patients hsa-miR-21-3p and hsa-miR-143-3p expression levels were decreased by 4.7 and 2.3 times, respectively. No significant changes were detected in hsa-miR-138-5p, hsa-miR-145-3p, hsa-miR-190a, hsa-miR-204-3p, hsamiR-206, and hsa-miR-210-3p expression levels between PH and control groups. In addition, considering the severity of the disease, it was observed that the decrease in miR-138, miR-143, miR-145, miR-190, mir-204, mir-206 and miR-208 expressions was significant in patients with severe PH. Conclusion: In the early diagnosis of PAH, hsa-miR-21-3p and especially hsa-miR-143-3p in peripheral plasma can be considered as potential biomarkers. [ABSTRACT FROM AUTHOR]

Pulmonary hypertension ( PH) is considered as a risk factor for morbidity and mortality in patients undergoing heart transplantation. Recently, left ventricular assist device ( LVAD) implantation has been increasingly used in reducing pulmonary artery pressure ( PAP) in patients with PH unresponsive to medical therapy. Herein, we aimed to compare the efficacy of continuous-flow and pulsatile-flow blood pumps on the improvement of PH in mechanical circulatory support patients. Twenty-seven patients with end-stage heart failure who underwent LVAD implantation surgery were enrolled. Fifteen of them (55.6%) had continuous-flow pump (HeartWare Ventricular Assist System, HeartWare, Inc., Miramar, FL, USA), and 12 of them (44.4%) had pulsatile pump (Berlin Heart EXCOR ventricular assist device, Berlin Heart AG, Berlin, Germany). The efficacy of LVADs on the improvement of PH was compared between continuous-flow and pulsatile pumps by the evaluation of systolic PAP, tricuspid annular plane systolic excursion ( TAPSE), right ventricular systolic motion ( RVSM), right ventricular ejection fraction ( RVEF), and grade of tricuspid insufficiency ( TI) for each of the study participants. All of the 15 patients who underwent continuous-flow blood pump implantation surgery (Group 1) were male with a mean age of 46.9 ± 11.7 years, and in pulsatile-flow blood pump implanted participants (Group 2), the mean age was 40.6 ± 16.8 years, all of whom were also male ( P = 0.259). Mean follow-up was 313.7 ± 241.3 days in Group 1 and 448.7 ± 120.7 days in Group 2 ( P = 0.139). In Group 1, mean preoperative and postoperative systolic PAP were measured as 51.7 ± 12.2 mm Hg and 22.2 ± 3.4 mm Hg, respectively, while those in Group 2 were 54.5 ± 7.5 mm Hg and 33.9 ± 6.4 mm Hg, respectively. A significantly greater decrease in systolic PAP was noticed in patients with continuous-flow blood pumps ( P = 0.023); however, no statistically significant difference was found when we considered the change in TAPSE between study groups ( P = 0.112). A statistical significance in the alteration of RVEF, RVSM, and the grade of TI during study visits was not found between the study groups ( P = 0.472, P = 0.887, and P = 0.237, respectively). Although the two studied types of LVADs were found to be effective in reducing PAP in heart transplantation candidates with PH, lesser postoperative systolic PAP values were achieved in patients who underwent continuous-flow pump implantation surgery. [ABSTRACT FROM AUTHOR]

Purpose: The aim of this study was to evaluate the associations of sonographic and sonoelastographic parameters with clinical cardiac parameters, as well as to assess their value in predicting survival in patients with pulmonary arterial hypertension (PAH). Methods: Thirty-six patients with PAH and normal liver function were prospectively enrolled in this prospective study along with 26 healthy controls, all of whom underwent ultrasound and point shear wave elastography examinations. Additionally, the portal vein pulsatility index (PVPI), inferior vena cava collapsibility index, and clinical cardiac variables were obtained in PAH patients. The values of hepatic (LVs) and splenic shear wave velocity (SVs) were compared between PAH patients and controls. The relationships between all sonographic and clinical parameters in the PAH patients were analyzed. Furthermore, their prognostic value in predicting survival was investigated. Results: LVs values in PAH patients (median, 1.62 m/s) were significantly higher than in controls (median, 0.99 m/s), while no significant difference was observed in SVs values. Patients with higher grades of tricuspid regurgitation (TR) had significantly different values of PVPI (P=0.010) and sonoelastographic parameters (P<0.001 for LVs and P=0.004 for SVs) compared to those with less severe TR. Tricuspid annular plane systolic excursion values were the only investigated parameter found to be associated with survival (hazard ratio, 0.814; 95% confidence interval, 0.694 to 0.954; P=0.011). Conclusion: Our results demonstrated a direct association between cardiac congestion (i.e., the severity of TR) and liver stiffness, which should be kept in mind during the assessment of fibrosis in patients with PAH. [ABSTRACT FROM AUTHOR]

Objective: The present study was designed to evaluate the characteristics of pulmonary hypertension (PH) and adult cardiology practice patterns for PH in our country. Methods: We evaluated preliminary survey data of 1501 patients with PH (females, 69%; age, 44.8±5.45) from 20 adult cardiology centers (AdCCs). Results: The average experience of AdCCs in diagnosing and treating patients with PH was 8.5±3.7 years. Pulmonary arterial hypertension (PAH) was the most frequent group (69%) followed by group 4 PH (19%), group 3 PH (8%), and combined pre- and post-capillary PH (4%). PAH associated with congenital heart disease (APAH-CHD) was the most frequent subgroup (47%) of PAH. Most of the patients' functional class (FC) at the time of diagnosis was III. The right heart catheterization (RHC) rate was 11.9±11.6 per month. Most frequently used vasoreactivity agent was intravenous adenosine (60%). All patients under targeted treatments were periodically for FC, six-minute walking test, and echo measures at 3-month intervals. AdCCs repeated RHC in case of clinical worsening (CW). The annual rate of hospitalization was 14.9±19.5. In-hospital use of intravenous iloprost reported from 16 AdCCs in CWs. Bosentan and ambrisentan, as monotreatment or combination treatment (CT), were noted in 845 and 28 patients, respectively, and inhaled iloprost, subcutaneous treprostinil, and intravenous epoprostenol were noted in 283, 30, and four patients, respectively. Bosentan was the first agent used for CT in all AdCCs and iloprost was the second. Routine use of antiaggregant, anticoagulant, and pneumococcal and influenza prophylaxis were restricted in only two AdCCs. Conclusion: Our nationwide data illustrate the current status of PH regarding clinical characteristics and practice patterns. [ABSTRACT FROM AUTHOR]