Presence Of Pulmonary Hypertension Research Articles

Легенева гіпертензія при сепсисі в дітей та її корекція за допомогою донатора оксиду азоту L-аргініну

У статті обговорюється проблема гемодинамічних розладів у малому колі кровообігу при сепсисі, тяжкому сепсисі та септичному шоку в дітей. Продемонстровано наявність легеневої гіпертензії, що виявляється при дослідженні середнього артеріального тиску в легеневій артерії методом допплерометрії за Kitabatake. Також показано, що легенева гіпертензія поєднується з розладами системної гемодинаміки та тканинною гіпоксією, які існують, незважаючи на збільшення рівня NO у крові. Наведено результати впливу внутрішньовенної інфузії аргініну гідрохлориду на показники вмісту оксиду азоту в сироватці та показники центральної та легеневої гемодинаміки при легеневій гіпертензії на тлі септичних станів у дітей. Продемонстровано здатність внутрішньовенної інфузії аргініну гідрохлориду в дозі 5 мл/кг/добу протягом 24–48 годин вірогідно збільшувати вміст NO у крові та знижувати тиск у легеневій артерії й легеневий судинний опір при септичних станах у дітей.

Clinical impact of pulmonary hypertension on the outcomes of acute myocardial infarction patients with or without chronic obstructive pulmonary disease.

Acute myocardial infarction (AMI) and chronic obstructive pulmonary disease (COPD) are leading global causes of morbidity and mortality. In patients with both of these conditions, the presence of pulmonary hypertension (PH) can further worsen their prognosis. We examined the outcomes of AMI patients with COPD (AMI+COPD) and without COPD (AMI−COPD), depending on the presence or absence of PH.A total of 318 AMI patients with COPD (AMI+COPD cohort) (n = 109) or without COPD (AMI-COPD cohort) (n = 209) were included in this study and were subdivided into 2 groups according to right ventricular systolic pressure (RVSP) level (PH group [RVSP ≥35 mm Hg] and no PH group [RVSP <35 mm Hg]).We investigated characteristics and clinical outcomes in both the AMI-COPD and AMI+COPD cohorts. When investigating in-hospital clinical outcomes, the PH group had a higher proportion of new-onset heart failure (HF) in both cohorts. In the AMI+COPD cohort, however, the PH group had a higher incidence of cardiogenic shock than the no PH group, which was consistent with the result of the post-inverse probability of treatment weighting (IPTW) analysis. When investigating 1-year clinical outcomes, the PH group had higher incidences of a major adverse cardiac event and all-cause mortality in both cohorts. This finding was mainly driven by cardiac death in the AMI-COPD cohort, whereas it was mainly driven by non-cardiac death in the AMI+COPD cohort. After IPTW adjustment, these differences were statistically attenuated such that all variables were similar between both groups.PH may be associated with the development of new-onset HF (in all patients) and cardiogenic shock (in the AMI+COPD cohort). In addition, PH may be also associated with all-cause mortality, although it was statistically attenuated after IPTW adjustment.

The impact of pulmonary hypertension on outcomes of transcatheter mitral valve replacement in mitral annular calcification.

To assess the impact of pulmonary hypertension (PH) on outcomes of patients with severe mitral annular calcification (MAC) undergoing transcatheter mitral valve replacement (TMVR). PH is associated with poor outcomes after mitral valve surgery. Whether the presence of PH in patients with MAC undergoing (TMVR) is associated with poor outcomes, is unknown. Retrospective evaluation of 116 patients from 51 centers in 11 countries who underwent TMVR with valve in mitral annular calcification (ViMAC) using balloon-expandable aortic transcatheter valves (THVs) from September 2012 to March 2017. Pulmonary artery systolic blood pressure (PASP) by echocardiogram was available in 90 patients. The subjects were stratified based on PASP: No PH = PASP ≤35 mmHg (n = 11); mild to moderate PH = PASP 36-49 mmHg (n = 21) and severe PH = PASP ≥50 mmHg (n = 58). Clinical, procedural, and echocardiographic outcomes were assessed. Mean age was 72.7 (±12.8) years, 59 (65.6%) were female, Society of Thoracic Surgeons score was 15.8 + 11.8% and 90.0% where in New York Heart Association (NYHA) class III-IV. There was no significant difference in all-cause mortality at 30 days (no PH = 27.3%, mild-moderate PH = 19.0%, severe PH = 31.6%; p = 0.55) or at 1 year (no PH = 54.5%, mild-moderate PH = 38.1%, severe PH = 56.1%; p = 0.36). No difference in adverse events, NYHA class or amount of residual mitral regurgitation at 1 year were observed between the groups. This study suggests that the presence of PH in patients with predominantly mitral stenosis with MAC undergoing TMVR does not impact mortality or adverse events. Further studies are needed to fully understand the effect of PH in this group of patients.

Effect of pulmonary hypertension on exercise capacity and gas exchange in patients with chronic obstructive pulmonary disease living at high altitude.

Background: Pulmonary hypertension (PH) is associated with decreased exercise tolerance in chronic obstructive pulmonary disease (COPD) patients, but in the altitude the response to exercise in those patients is unknown. Our objective was to compare exercise capacity, gas exchange and ventilatory alterations between COPD patients with PH (COPD-PH) and without PH (COPD-nonPH) residents at high altitude (2640 m). Methods: One hundred thirty-two COPD-nonPH, 82 COPD-PH, and 47 controls were included. Dyspnea by Borg scale, oxygen consumption (VO2), work rate (WR), ventilatory equivalents (VE/VCO2), dead space to tidal volume ratio (VD/VT), alveolar-arterial oxygen tension gradient (AaPO2), and arterial-end-tidal carbon dioxide pressure gradient (Pa-ETCO2) were measurement during a cardiopulmonary exercise test. For comparison of variables between groups, Kruskal-Wallis or one-way ANOVA tests were used, and stepwise regression analysis to test the association between PH and exercise capacity. Results: All COPD patients had a lower exercise capacity and higher PaCO2, A-aPO2 and VD/VT than controls. The VO2 % predicted (61.3 ± 20.6 vs 75.3 ± 17.9; p < 0.001) and WR % predicted (65.3 ± 17.9 vs 75.3 ± 17.9; p < 0.001) were lower in COPD-PH than in COPD-nonPH. At peak exercise, dyspnea was higher in COPD-PH (p = 0.011). During exercise, in COPD-PH, the PaO2 was lower (p < 0.001), and AaPO2 (p < 0.001), Pa-ETCO2 (p = 0.033), VE/VCO2 (p = 0.019), and VD/VT (p = 0.007) were higher than in COPD-nonPH. In the multivariate analysis, PH was significantly associated with lower peak VO2 and WR (p < 0.001). Conclusion: In COPD patients residing at high altitude, the presence of PH was an independent factor related to the exercise capacity. Also, in COPD-PH patients there were more dyspnea and alterations in gas exchange during the exercise than in those without PH.

Effect of pulmonary hypertension on 5-year outcome of kidney transplantation.

Pulmonary hypertension affects about one in four patients with advanced chronic kidney disease and significantly increases the risk of death. Kidney transplantation is the recommended management option for patients with progressive or end‐stage kidney disease. However, the resource‐limited nature of kidney transplantation and its intensive peri‐operative and posttransplantation management motivates careful consideration of potential candidates’ medical conditions to optimally utilize available graft organs. Since pulmonary hypertension is known to increase peri‐operative morbidity and mortality among patients living with chronic kidney disease, we performed a retrospective cohort study to assess the impact of pretransplantation pulmonary hypertension on posttransplantation outcome. All patients who underwent single‐organ kidney transplantation at our center in calendar years 2010 and 2011 were identified and the presence of pulmonary hypertension was determined from pretransplantation echocardiography. Outcome was assessed at 5 years following kidney transplantation. Of 350 patients who were included, 117 (33%) had evidence of pulmonary hypertension. The risk of death, graft dysfunction, or graft failure at 5 years after kidney transplantation was higher among those with pulmonary hypertension, primarily owing to an increased risk of graft dysfunction. Importantly, in this institutional cohort of kidney transplant recipients, pretransplant pulmonary hypertension was not associated with a difference in posttransplant survival at 5 years. While institutional and regional differences in outcome can be expected, this report suggests that carefully selected patients with pulmonary hypertension receive similar long‐term benefits from kidney transplantation.

Clinical Application of 4D Flow MR Imaging to Pulmonary Hypertension.

Pulmonary hypertension (PH) is characterized by elevated pulmonary arterial pressure (PAP). Although right-heart catheterization is the gold standard method for the diagnosis of PH by definition, various less-invasive imaging tests have been used for screening, detection of underlying diseases-causing PH, and monitoring of diseases. Among them, 4D flow MRI is an emerging and unique imaging test that allows for comprehensive visualization of blood flow in the right heart and proximal pulmonary arteries. The characteristic blood flow pattern observed in patients with PH is vortical flow formation in the main pulmonary artery. Recent studies have proposed the use of these findings to determine not only the presence of PH but also estimate the mean PAP. Other applications of 4D flow MRI for PH include measurement of wall shear stress, helicity, and 3D flow balance in the pulmonary arteries. It is worth noting that 4D flow has also the potential for longitudinal follow-ups. In this review, the clinical definition of PH, summary of conventional imaging tests, characteristics of pulmonary arterial flow as shown by 4D flow MRI, and clinical application of 4D flow MRI in the management of patients with PH will be discussed.

Abstract 10064: Pulmonary Hypertension in Patients with Connective Tissue Disease-associated Interstitial Lung Disease and Vascular Changes on Computed Tomography

Introduction: Pulmonary hypertension (PH) is a complication in patients with connective-tissue disease-associated interstitial lung disease (CTD-ILD) associated with mortality. In Idiopathic Pulmonary Fibrosis, increased vascular volume has been reported to be associated with poor outcomes. We sought to elicit whether similar changes could be observed in patients with CTD-ILD and pulmonary hypertension. Methods: We identified patients with a known diagnosis of CTD-ILD who had undergone chest CT, transthoracic echocardiography (TTE), and right heart catherization (RHC) using the Mass General Brigham Research Patient Data Repository (IRB #2018P000419). The electronic health records (EHR) were reviewed to confirm the diagnosis of CTD-ILD. A 3D reconstruction of the pulmonary vasculature using the Chest Imaging Platform ( www.chestimagingplatform.com ) to compute the total vascular volume was performed on thin sliced CT scans closest to the RHC. Vessels were separated into arterial and venous components using a deep learning approach. Statistical analysis was performed with R 4.01. Values are shown as medians and interquartile ranges and were compared using Wilcoxon Rank-Sum tests. Results: A total of 104 patients with CTD-ILD were identified: Pre-capillary PH (n=43), Isolated Post-capillary PH (n=9), Combined Pre- and Post-capillary PH (n=8), and PH Absent (n=44). There was no difference in patient age or lung volume by CT scan. On TTE, RVSP was elevated in PH (52 mmHg, IQR 41-64 mmHg vs 34 mm Hg, IQR 27-49 mmHg, p &lt; 0.0001), as was the arterial total vascular volume (118 mL, IQR 85-152 ml vs 100 mL, IQR 77-126 ml, p = 0.009) with no trend for significance for venous total vascular volume. Addition of total arterial volume remained a statistically significant predictor of presence of PH (p = 0.01) when added to logistic regression models predicting PH by TTE RVSP, with improvement in model AIC (106.1 to 97.9) and AUC (0.75 to 0.80) of the model. Conclusions: In this retrospective study of patients with CTD-ILD and available imaging and hemodynamics, patients with PH have increased arterial vascular volume compared to those without PH. Information derived from CT images routinely acquired in this population could be used to improve screening and phenotyping.

Risk stratification and survival in patients after liver transplantation: the role of right heart function and dimension

Abstract Background Liver transplantation (LTx) is often the only treatment strategy in patients with advanced liver disease. Since the donor pool is limited, careful preoperative examination of the cardiac functional status is crucial. Especially the presence of pulmonary hypertension (PH) either coincidental or as part of porto-pulmonary hypertension (PoPHT) is known to play a prognostic role in post-transplant survival and graft durability. However, right heart function itself may play a role even in the absence of PH. Purpose We retrospectively evaluated right heart function data acquired during transthoracic echocardiography (TTE) in the evaluation process for LTx listing to identify patient populations at risk for poor long-term prognosis. Methods We performed a single-center retrospective cohort study of patients that underwent LTx between 2011 and 2020. All patients ultimately underwent LTx; Survival data were assessed at 90 days, 1 year and 3 years. The waiting period from time of cardiac assessment to the actual operation was blinded. We evaluated right heart end-diastolic diameter (RVEDd), tricuspid annular plane systolic excursion (TAPSE), as well as right atrial area (RAA). We pre-specified cut-offs for abnormal values with RVEDd above 39 mm, TAPSE below 18 mm and RAA above 16 cm2. Results We evaluated 351 patients who underwent LTx. 247 data sets were complete and could be included in the primary analysis (mean age 56.3±10.3 years, 75.7% male). Patients had a median Lab-MELD-Score of 14 points (IQR: 10–21 points). 23.8% of patients received a living donor transplant. The left ventricular ejection fraction was not compromised in any of the subjects (mean LVEF: 68.6±9.0%). The median waiting time in days between TTE and LTx was 142 days (IQR: 37–434 days) The primary end-point of mortality occurred in 44 or 17.8% of patients over a Period of three years. In univariate Cox-regression the pre specified cut-offs for TAPSE and RAA were not associated with a statistically significant survival (HR 0.663, CI: 0.33–1.33, p: 0.25). However, univariate analysis revealed that increased RVEDd was associated with post-LTx survival (HR 1.98, CI: 1.09–3.58, p=0.025) as well as Log-Rank (Mantel-Cox) in the Kaplan-Meier-estimate (Chi2: 5.25, p:0.022). In multivariable analysis including several laboratory values as well as the Lab-Meld-Score (Figure 2), RVEDd group remained the only significant parameter predicting mortality (HR 2.12, CI: 1.02–4.41, p=0.04). The Kaplan Meier analysis showed a significant difference in survival at 3 years of follow-up. Combining right ventricle parameters in a secondary analysis did not provide any additional benefit in predicting survival Conclusion Analysis of right heart function and especially dilatation was able to provide additional information concerning long-term outcome post-LTx in this retrospective analysis even when taking into consideration that moderate to severe PH or PoPHT was ruled out beforehand. Funding Acknowledgement Type of funding sources: None.

Circulating proteomic biomarkers related to the development of chronic thromboembolic pulmonary hypertension in patients with acute pulmonary embolism

Abstract Background Chronic thromboembolic pulmonary hypertension (CTEPH), known as a complication of acute pulmonary embolism (PE), can be attributed to both persistent organized thrombi and small-vessel disease. It has been reported that patients with acute PE are more likely to develop CTEPH or have a worse prognosis in follow-up if they have pulmonary hypertension (PH) or right ventricular dysfunction in their acute phase; in acute PE, increased pulmonary arterial pressure can be induced by a combination of mechanical obstruction and pulmonary vasoconstriction. However, it remains unknown whether contributors to the increase pulmonary arterial pressure in acute phase would persistently influence the pulmonary vasculature, which could further lead to the development and progression of CTEPH. Purpose To investigate underlying contributors related to pulmonary hypertension in acute PE that would persistently influence the pulmonary vasculature in the development of CTEPH, and to explore potential biomarkers predictive of CTEPH in acute PE. Methods Plasma from patients with acute PE were collected and classified into Group 1 (with PH) and Group 2 (without PH) according to the presence of PH, which was defined by echocardiographic estimated “high probability” of PH as recommended in the 2015 ESC/ERS Guidelines for the diagnosis and treatment of PH. In addition, plasma from patients with acute PE who had not developed PH both in their acute and follow-up phase but had persistent thrombi (Group 3) were also studied and compared to that of CTEPH patients (Group 4). Differential expression proteins (DEPs) between 4 groups were identified in terms of data-independent acquisition-based quantitative proteomic analysis. Results Twenty-two DEPs were identified between Group1 and Group 2, and 105 DEPs were identified between Group 3 and Group 4. Among two groups of DEPs, 8 proteins were in common: antithrombin-III, osteopontin, properdin, desmoplakin, C1QA (complement C1q subcomponent subunit A), LDHA (L-lactate dehydrogenase A chain), IGKV1D-33 (immunoglobulin kappa variable 1D-33) and PEPD (Xaa-Pro dipeptidase). The level of LDHA, antithrombin-III, osteopontin and properdin were decreased both in acute PE patients with PH and CTEPH patients, while the level of desmoplakin, C1QA and IGKV1D-33 were increased in the above two groups. Regarding PEPD, its level was lower in acute patients with PH but was higher in CTEPH. Based on underlying pathogenic mechanism and the percentage of missing value, osteopontin, properdin, desmoplakin, C1QA, LDHA and PEPD were further validated as potential biomarkers predictive of CTEPH in acute PE. Conclusions Contributors to the increase pulmonary arterial pressure in acute PE can be further involved in the development of CTEPH, and these proteins can be potential biomarkers to help guide the management and follow-up strategy in acute PE. Funding Acknowledgement Type of funding sources: Public grant(s) – National budget only. Main funding source(s): Chinese Academy of Medical Sciences Innovation Fund for Medical Sciences (CIFMS; No. 2017-12M-1-009); National Key Research and Development Program of China (No: 2016YFC1304400) Differential expression proteinsRelative intensity of proteins

Predictive association of pulmonary hypertension with risks of adverse renal outcomes in type 2 diabetes

Abstract Background Diabetic kidney disease is a major diabetic complication which contributes to the end-stage renal disease and renal deaths in diabetic patients. The aim of our study was to investigate the role of pulmonary hypertension (PHT) in the development of adverse renal outcomes in patients with type 2 diabetes (T2DM). Methods A total of 1479 diabetic patients (mean age 67±13 years; women, 44.7%) were recruited. Pulmonary artery systolic pressure (PASP) was estimated by transthoracic echocardiography at baseline. Adverse renal outcomes were defined as a composite endpoint of a sustained 40% decline in eGFR, end-stage renal disease requiring renal replacement therapy, kidney transplantation or renal deaths. Cox regression hazard model was used to assess the association between PHT and incident adverse renal outcomes. Results Patients were divided into three groups based on their baseline PASP: no PHT (n=1204, 75.1%), mild PHT (321, 20.0%) and significant PHT (78, 4.9%). A total of 474 adverse renal outcomes occurred during a median follow-up of 53 months, Kaplan-Meier survival curve showed that patients with significant PHT had highest risk of adverse renal outcomes. Multivariate cox regression demonstrated both mild PHT (HR=1.66, 95% CI 1.33, 2.06, P&amp;lt;0.001) and significant PHT (HR=2.09, 95% CI 1.45, 3.02, P&amp;lt;0.001) were independently associated with the incident adverse renal outcomes. Conclusion The presence of PHT was associated with adverse renal outcomes in patients with T2DM. Funding Acknowledgement Type of funding sources: None.

Preoperative evaluation of pulmonary hypertension in lung transplant candidates: echocardiography versus right heart catheterization

Abstract Background Right heart catheterization (RHC) and echocardiography are both routinely used for pulmonary hypertension (PH) assessment in LT candidates, although this is not mandatory according to guidelines. We aim to describe the correlation between the pulmonary artery systolic pressure (PASP) measured by echocardiography to that measured by RHC in this population. Methods From a retrospective registry of 461 LT candidates, undergoing RHC between 2015–2019, 393 consecutive patients were assessed for the presence of pulmonary hypertension according to two methods – echocardiography and RHC. The primary outcome was the correlation between the estimated PASP measured by echocardiography to that measured by RHC. Secondary outcome was the accuracy of the echocardiographic assessment of PH. Results Patients were predominantly males (63.6%) with a mean age of 61.46±8.33y. The two most common etiologies for lung failure were interstitial lung disease or pulmonary fibrosis (52.2%) and chronic obstructive pulmonary disease (30.5%). Estimated PASP as measured by echocardiography was available in 89.31% of the patients, with a mean value of 49.5±20.02 mmHg. Mean PASP measured by RHC was 42.47±17.96 mmHg. The correlation between the two measurements was moderate (Pearson's correlation: r=0.609, p&amp;lt;0.01). The accuracy of the echocardiographic estimation of PASP was poor with &amp;gt;10 mmHg differences between the two values in 79.9% of the patients. Conclusions In the pre-surgical evaluation of LT candidates, echocardiographic estimation of PASP has moderate correlation with the PASP measured by RHC and relatively poor accuracy. Funding Acknowledgement Type of funding sources: None.

PH-ILD: Identification, Evaluation, and Monitoring: A Diagnostic View From Both Sides

PH-ILD: Identification, Evaluation, and Monitoring: A Diagnostic View From Both Sides

Intrapulmonary artery Doppler to predict mortality and morbidity in fetuses with mild or moderate left-sided congenital diaphragmatic hernia.

In fetuses with isolated left-sided congenital diaphragmatic hernia (LCDH), prenatal detection of severe pulmonary hypoplasia is important, as fetal therapy can improve survival. Cases with mild or moderate lung hypoplasia still carry a considerable risk of mortality and morbidity, but there has been less interest in the accurate prediction of outcome in these cases. In this study of fetuses with mild or moderate isolated LCDH, we aimed to investigate: (1) the association between intrapulmonary artery (IPA) Doppler findings and mortality at discharge; (2) whether adding IPA Doppler findings improves the prediction of mortality based on lung size and liver herniation; and (3) the association between IPA Doppler findings and early neonatal morbidity. This was a retrospective study of all consecutive fetuses assessed at the BCNatal and UZ Leuven hospitals between 2008 and 2020 with a prenatal diagnosis of isolated, non-severe LCDH, defined as observed-to-expected lung-to-head ratio (o/e-LHR) > 25%, that were managed expectantly during pregnancy followed by standardized neonatal management. An additional inclusion criterion was the availability of IPA Doppler measurements. The primary outcome was the association between IPA Doppler findings and mortality at discharge. Other predictors included o/e-LHR, liver herniation and gestational age at birth. Secondary outcomes were the association between IPA Doppler findings and the presence of pulmonary hypertension (PHT), need for supplemental oxygen at discharge and need for extracorporeal membrane oxygenation. IPA pulsatility index (PI) values were converted into Z-scores. Logistic regression analysis was performed to investigate the associations between predictor variables and outcome, and the best model was chosen based on the Nagelkerke R2 . Observations for 70 non-severe LCDH cases were available. Fifty-four (77%) fetuses survived until discharge. On logistic regression analysis, higher IPA-PI was associated with an increased risk of mortality (odds ratio (OR), 3.96 (95% CI, 1.62-9.70)), independently of o/e-LHR (OR, 0.87 (95% CI, 0.79-0.97)). An IPA-PI Z-score cut-off of 1.8 predicted mortality with a detection rate of 69% and specificity of 93%. Adding IPA-PI to o/e-LHR improved significantly the model's performance (Nagelkerke R2 , 46% for o/e-LHR + IPA-PI vs 28% for o/e-LHR (P < 0.002)), with a detection rate of 81% at a 10% false-positive rate. IPA-PI was associated with PHT (OR, 2.20 (95% CI, 1.01-4.59)) and need for oxygen supplementation at discharge (OR, 1.90 (95% CI, 1.10-3.40)), independently of lung size. In fetuses with mild or moderate LCDH, IPA-PI was associated with mortality and morbidity, independently of lung size. A model combining o/e-LHR with IPA-PI identified up to four in five cases that eventually died, despite being considered to have non-severe pulmonary hypoplasia. © 2021 International Society of Ultrasound in Obstetrics and Gynecology.

Cardiac acoustic biomarkers as surrogate markers to diagnose the phenotypes of pulmonary hypertension: an exploratory study

Pulmonary hypertension (PH) is commonly associated with left heart disease. In this retrospective study, using the database of a clinical study conducted between January 2008 and July 2008, the phenotypes of PH were classified using non-invasive cardiac acoustic biomarkers (CABs) and compared with classification by echocardiography. Records with same-day measurement of acoustic cardiography and right heart catheterization (RHC) parameters were included; cases with congenital heart disease were excluded. Using the RHC measurements, PH was classified as pre-capillary PH (Prec-PH), isolated post-capillary PH (Ipc-PH), and combined pre-capillary and post-capillary PH (Cpc-PH). The first, second, third, and fourth heart sounds (S1, S2, S3, and S4) were quantified as CABs (intensity, complexity, and strength). Forty subjects were selected: 5 had Prec-PH, 5 had Ipc-PH, 8 had Cpc-PH, and 22 had No-PH. CABs were significantly correlated with RHC measurements, with significant differences among phenotypes. Phenotype classification was performed using various CABs, and the diagnostic performance as assessed by the area under the receiver operating characteristic curve was 0.674–0.720 for Prec-PH, 0.657–0.807 for Ipc-PH, and 0.742 for Cpc-PH. High negative and low positive predictive values for phenotype identification were observed. CABs may provide an ambulatory measurement method with home-monitoring friendliness which is more convenient than standard examinations to identify presence of PH and its phenotypes.

Risk factors for acute kidney injury after pediatric cardiac surgery: a meta-analysis.

Cardiac surgery-associated acute kidney injury (AKI) is associated with increased morbidity and mortality in both adults and children. This study aimed to identify clinical risk factors for AKI following cardiac surgery in the pediatric population. PubMed/MEDLINE, Embase, Scopus, and reference lists of relevant articles were searched for studies published by August 2020. Studies were included if (1) the population consisted of pediatric patients (< 18years old), (2) patients underwent cardiac surgery, (3) risk factors were compared between patients who developed AKI and those who did not, and (4) studies were prospective or retrospective observational studies or randomized controlled trials. Children undergoing pediatric cardiac surgery. Random-effects meta-analysis was performed, comparing potential risk factors between pediatric patients who developed CS-AKI and those who did not. Sixty-one publications including a total of 19,680 participants (AKI: 7257 participants; no AKI: 12,423 participants) were included from studies published between 2008 and 2020. The pooled estimated incidence of AKI was 34.3% (95% confidence interval 30.0-38.8%, I2 = 96.8%). Binary risk factors that were significantly and consistently associated with AKI were the presence of pulmonary hypertension, cyanotic heart disease, univentricular heart, risk adjustment for congenital heart surgery 1 (RACHS-1) score ≥ 3, vasopressor use, cardiopulmonary bypass use, reoperation, and sepsis. Significant continuous risk factors included younger age, lower body weight, lower preoperative creatinine, higher preoperative estimated glomerular filtration rate (eGFR), higher RACHS-1 score, longer surgery time, longer cardiopulmonary bypass time, longer aortic cross-clamp time, and higher red blood cell transfusion volume. Results are limited by heterogeneity and potential residual confounding. Our meta-analysis identified clinical risk factors that are associated with AKI in children undergoing cardiac surgery. This might help clinicians anticipate and manage more carefully this population and implement standardized preventive strategies. CRD42021262699. A higher resolution version of the Graphical abstract is available as Supplementary information.

Effect of sildenafil added to antifibrotic treatment in idiopathic pulmonary fibrosis

Sildenafil is a phosphodiesterase-5 inhibitor used to treat idiopathic pulmonary arterial hypertension; however, its benefits are unclear in patients with advanced idiopathic pulmonary fibrosis (IPF). We aimed to evaluate its effect as an add-on to antifibrotic agents on clinical outcomes of real-world IPF patients. Among a total of 607 IPF patients treated with antifibrotic agent, 66 concurrently received sildenafil. Propensity score matching was performed to adjust for differences in age, sex, body mass index, forced vital capacity (FVC), and diffusing capacity (DLCO) between the sildenafil and no-sildenafil groups. The outcomes of these groups in terms of FVC decline rate, all-cause mortality, hospitalization, and acute exacerbation were compared. Propensity score matching identified 51 matched pairs. The mean age of the patients was 69.5 years and 80.4% were male. Mean FVC and DLCO were 51.7% and 29.5% of the predicted values, respectively. The FVC decline rates did not differ significantly (p = 0.714) between the sildenafil (− 101 mL/year) and no-sildenafil (− 117 mL/year) groups. In multivariable analyses adjusted for comorbidities and presence of pulmonary hypertension, sildenafil had no significant impact on all-cause mortality, hospitalization, or acute exacerbation. Sildenafil add-on to antifibrotic treatment had no significant effects on the clinical outcomes of IPF patients.

Differences between Acute Exacerbations of Idiopathic Pulmonary Fibrosis and Other Interstitial Lung Diseases.

Interstitial lung diseases (ILDs) comprise a wide group of pulmonary parenchymal disorders. These patients may experience acute respiratory deteriorations of their respiratory condition, termed “acute exacerbation” (AE). The incidence of AE-ILD seems to be lower than idiopathic pulmonary fibrosis (IPF), but prognosis and prognostic factors are largely unrecognized. We retrospectively analyzed a cohort of 158 consecutive adult patients hospitalized for AE-ILD in two Italian university hospitals from 2009 to 2016. Patients included in the analysis were divided into two groups: non-IPF (62%) and IPF (38%). Among ILDs included in the non-IPF group, the most frequent diagnoses were non-specific interstitial pneumonia (NSIP) (42%) and connective tissue disease (CTD)-ILD (20%). Mortality during hospitalization was significantly different between the two groups: 19% in the non-IPF group and 43% in the IPF group. AEs of ILDs are difficult-to-predict events and are burdened by relevant mortality. Increased inflammatory markers, such as neutrophilia on the differential blood cell count (HR 1.02 (CI 1.01–1.04)), the presence of pulmonary hypertension (HR 1.85 (CI 1.17–2.92)), and the diagnosis of IPF (HR 2.31 (CI 1.55–3.46)), resulted in negative prognostic factors in our analysis. Otherwise, lymphocytosis on the differential count seemed to act as a protective prognostic factor (OR 0.938 (CI 0.884–0.995)). Further prospective, large-scale, real-world data are needed to support and confirm the impact of our findings.

Pulmonary hypertension in systemic sclerosis: diagnosis by systematic screening and prognosis after three years follow-up

BackgroundSystemic sclerosis (SSc) is a rare disease, and the presence of pulmonary hypertension can be a determining factor in prognosis. The aim of this study was to evaluate the diagnosis, profile, and prognosis of systemic sclerosis pulmonary hypertension (SSc-PH) diagnosed by systematic screening in a Brazilian population.MethodsA cohort of SSc patients underwent systematic screening for SSc-PH. Patients were referred for right heart catheterization (RHC) according to transthoracic echocardiogram or a combination of diagnostic tools. The clinical, immunological, and hemodynamic features and prognosis after 3 years were evaluated.ResultsTwenty patients underwent RHC. SSc pulmonary arterial hypertension (SSc-PAH) was the most common group of SSc-PH. These patients had long disease duration, high urate levels and highly elevated mean pulmonary arterial pressure (mPAP) and peripheral vascular resistance (PVR) on hemodynamics. Patients with mPAP > 20– < 25 mmHg had hemodynamic features of intermediate disease. Patients with SSc-PH associated to interstitial lung disease (SSc-ILD-PH) had signs of vasculopathy on hemodynamics. In patients with no-SSc-PH, the survival at 1, 2, and 3 years was 96%, 92% and 92%, respectively and in patients with SSc-PH it was 86.7%, 60% and 53.3%, respectively.ConclusionsPatients identified with SSc-PAH and SSc-ILD-PH in our screening had severe clinical and hemodynamic features. Mortality remains high in SSc-PH but was more related to Bo-PAH and SSc-ILD-PH, while in SSc-PAH, the prognosis was better. Trial registration: Current Controlled Trials ISRCTN 72968188, July 8th, 2021. Retrospectively registered.

B-PO03-145 POST LVAD ATRIAL ARRHYTHMIAS IN PATIENTS WITH NO PRIOR HISTORY OF ATRIAL ARRHYTHMIAS

Data on incidence of post LVAD atrial arrhythmias (AA) in patients with no prior history of AA remain sparse. We sought to assess the incidence and predictors of de novo AA in continuous flow LVAD patients. A multi-center LVAD arrhythmia registry was queried for patients with no history of AA before LVAD implant. AA was defined as atrial tachycardia, flutter, or fibrillation lasting >6 hours or >1% burden. Univariate demographic and pre-operative risk factors were evaluated for association with post-LVAD AA. A multivariate regression model was used to identify independent factors associated with incidence of post-LVAD AA. A total of 298 patients (56±14 years, 73% male) with no history of AA underwent LVAD implantation. Patients with post LVAD AA (31%, n=92) were older (61 vs 58 years). There was no statistically significant difference between post LVAD AA and No AA groups in terms of gender (female 44% v 39%, p=.5), COPD (42 v 41%, p=.8), sleep apnea (43 v 40%, p=.6) or diabetes (42 v 51%, p=.2). Patients with higher INTERMACS profiles had a higher incidence of post LVAD AA (for class 1, 2, 3, 4+ respectively 33%, 28%, 35%, 56%, p=.04) whereas those with pulmonary hypertension (PHTN) were less likely to have post LVAD AA (33% v 50%, p=.01). The regression model identified higher INTERMACS class as an independent predictor of the post LVAD AA (OR=1.36, p=.02), Table 1. Patients with no history of AA were more likely to have de novo AA post LVAD with increasing INTERMACS profile and age whereas presence of PHTN was associated with lesser incidence of post LVAD AA. Gender, BMI, presence of COPD or sleep apnea were not significant predictors of post LVAD AA.

Gender features of structural and functional changes of the heart and levels of copeptin and NTproBNP in patients with acute Q-myocardial infarction in the presence of pulmonary hypertension

The aim. To evaluate the gender features of structural and functional changes in the heart and levels of copeptin and NTproBNP in patients with acute myocardial infarction (AMI) with concomitant pulmonary hypertension (PH). Materials and methods. 74 patients with AMI and concomitant PH who were treated in the intensive care unit and emergency cardiology for patients with myocardial infarction of the Municipal Non-Profit Enterprise “City Hospital of Emergency and Ambulance” of Zaporizhzhia City Council were examined. The patients were divided into two groups: the first group consisted of 42 male patients (mean age 71.06 ± 2.21 years) and 32 female patients (mean age 76.41 ± 2.32 years). All the patients were examined in the first three days by two-dimensional echocardiography on a device MyLab50 (“Esaote”, Italy). The serum levels of kopeptin and NTproBNP were determined by enzyme-linked immunosorbent assay using a set of reagents Elabscience (USA). Results. There was a trend towards a predominantly anterior AMI in the group of men and lower AMI – in the group of women. Individuals with functional class III and IV heart failure predominated (76.1 %) among the men with AMI and PH. At the same time, there were significantly more persons with III and IV functional class in the group of men than in the group of women (P &lt; 0.05). There was a significant diastolic left ventricular posterior wall thickening in women compared to men (8.4 %; P &lt; 0.05) and a tendency to diastolic interventricular septal thickening in females. There was an upward trend in end-systolic and end-diastolic sizes in the group of men compared to women; however, the differences did not reach the required level of significance. Evaluation of serum copeptin and NTproBNP in patients with AMI and PH revealed significantly higher levels of these indicators in the group of men compared to women (P &lt; 0.05). Conclusions. Men with AMI and PH had more severe heart failure compared to women due to the predominance of patients with functional classes III and IV. Women with AMI and PH demonstrated predominantly concentric type of remodeling in the form of significantly thickened LV posterior wall and the tendency to increase interventricular septum wall thickness, and eccentric hypertrophy in the form of LV dilatation prevailed in men. Men with AMI and PH had significantly higher serum copeptin and NTproBNP levels compared to women.