Definition Of Pulmonary Hypertension Research Articles

The Management of Mild Pulmonary Hypertension in Clinical Practice.

The definition of pulmonary hypertension (PH) has been revised recently by lowering the mean pulmonary artery pressure (mPAP) threshold from ≥25 mmHg to >20mmHg, assessed by right heart catheterization (RHC). This change reflects the mPAP upper limit of normal and lower limit that is independently associated with adverse outcome. To improve the specificity of diagnosing pathogenic elevation in mPAP, however, classifying patients with precapillary PH now also includes pulmonary vascular resistance (PVR) >2.0WU (from >3.0WU). These changes are positioned to capture ~55% more patients with PH. Since all clinical trials showing benefit of pulmonary vasodilator therapy in precapillary PH used the classical hemodynamic definition, the approach to diagnosis and management of patients with mild PH (i.e., mPAP 21-24mmHg and PVR 2-3WU) requires particular consideration. Here, we use a question- answer format to discuss key areas in the management of mild PH, including practical information catered to clinicians without training in PH.

Impact of the 2022 pulmonary hypertension definition on haemodynamic classification and mortality in patients with aortic stenosis undergoing valve replacement.

With the 2022 pulmonary hypertension (PH) definition, the mean pulmonary artery pressure (mPAP) threshold for any PH was lowered from ≥25 to >20 mmHg, and the pulmonary vascular resistance (PVR) value to differentiate between isolated post-capillary PH (IpcPH) and combined pre- and post-capillary PH (CpcPH) was reduced from >3 Wood units (WU) to >2 WU. We assessed the impact of this change in the PH definition in aortic stenosis (AS) patients undergoing aortic valve replacement (AVR). Severe AS patients (n = 503) undergoing pre-AVR cardiac heart catheterization were classified according to both the 2015 and 2022 definitions. The post-AVR mortality [median follow-up 1348 (interquartile range 948-1885) days] was assessed. According to the 2015 definition, 219 (44% of the entire population) patients had PH: 63 (29%) CpcPH, 125 (57%) IpcPH, and 31 (14%) pre-capillary PH. According to the 2022 definition, 321 (+47%) patients were diagnosed with PH, and 156 patients (31%) were re-classified: 26 patients from no PH to IpcPH, 38 from no PH to pre-capillary PH, 38 from no PH to unclassified PH, 4 from pre-capillary PH to unclassified PH, and 50 from IpcPH to CpcPH (CpcPH: +79%). With both definitions, only the CpcPH patients displayed increased mortality (hazard ratios ≈ 4). Among the PH-defining haemodynamic components, PVR was the strongest predictor of death. In severe AS, the application of the 2022 PH definition results in a substantially higher number of patients with any PH as well as CpcPH. With either definition, CpcPH patients have a significantly increased post-AVR mortality.

Sarcoidosis-Associated Pulmonary Hypertension.

Sarcoidosis-associated pulmonary hypertension (SAPH) is a very severe complication of the disease, largely impacting its morbidity and being one of its strongest predictors of mortality. With the recent modifications of the hemodynamic definition of pulmonary hypertension (mean arterial pulmonary pressure >20 instead of <25 mmHg,) its prevalence is presently not precisely known, but it affects from 3 to 20% of sarcoid patients; mostly, although not exclusively, those with an advanced, fibrotic pulmonary disease. Its gold-standard diagnostic tool remains right heart catheterization (RHC). The decision to perform it relies on an expert decision after a non-invasive work-up, in which echocardiography remains the screening tool of choice. The mechanisms underlying SAPH, very often entangled, are crucial to define, as appropriate and personalized therapeutic strategies will aim at targeting the most significant ones. There are no recommendations so far as to the indications and modalities of the medical treatment of SAPH, which is based upon the opinion of a multidisciplinary team of sarcoidosis, pulmonary hypertension and sometimes lung transplant experts.

Updated Clinical Classification and Hemodynamic Definitions of Pulmonary Hypertension and Its Clinical Implications.

Pulmonary hypertension (PH) refers to a pathologic elevation of the mean pulmonary artery pressure (mPAP) and is associated with increased morbidity and mortality in a wide range of medical conditions. These conditions are classified according to similarities in pathophysiology and management in addition to their invasive hemodynamic profiles. The 2022 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension present the newest clinical classification system and includes significant updates to the hemodynamic definitions. Pulmonary hypertension is now hemodynamically defined as an mPAP > 20 mmHg, reduced from the previous threshold of ≥25 mmHg, due to important insights from both normative and prognostic data. Pulmonary vascular resistance has been extended into the definition of pre-capillary pulmonary hypertension, with an updated threshold of >2 Wood Units (WU), to help differentiate pulmonary vascular disease from other causes of increased mPAP. Exercise pulmonary hypertension has been reintroduced into the hemodynamic definitions and is defined by an mPAP/cardiac output slope of >3 mmHg/L/min between rest and exercise. While these new hemodynamic thresholds will have a significant impact on the diagnosis of pulmonary hypertension, no evidence-based treatments are available for patients with mPAP between 21-24 mmHg and/or PVR between 2-3 WU or with exercise PH. This review highlights the evidence underlying these major changes and their implications on the diagnosis and management of patients with pulmonary hypertension.

Impact of the New Definition of Pulmonary Hypertension on the Prevalence of Primary Graft Dysfunction in Lung Transplant Recipients

Pulmonary hypertension (PH) secondary to lung disease (Group-3 PH) is the second leading cause of PH. The role of PH as a risk factor for primary graft dysfunction (PGD) following lung transplant (LT) is controversial. To assess the impact that the new definition of PH had on the prevalence of PH in patients with advanced lung disease-candidate for LT, and its association with the occurrence of PGD. A retrospective study was performed in all patients undergoing cardiac catheterisation referred for consideration as candidates to LT in a centre between 1 January 2017 and 31 December 2022. The baseline and haemodynamic characteristics of patients were analysed, along with the occurrence of PGD and post-transplant course in those who ultimately underwent transplantation. A total of 396 patients were included. Based on the new 2022 European Society of Cardiology/European Respiratory Society definitions, as many as 70.7% of patients met PH criteria. Since the introduction of the 2022 definition, a significant reduction was observed in the frequency of severe Group-3 PH (41.1% vs 10.3%; p<0.001), with respect to the 2015 definition. As many as 236 patients underwent transplantation. None of the variables associated with PH was identified as a risk factor for PGD. The new classification did not have any impact on the prevalence of PGD after transplantation. These results exclude that any significant differences exist in the baseline characteristics or post-transplant course of patients with Group-3 PH vs unclassified PH.

PULMONARY HYPERTENSION – A MINIREVIEW

Pulmonary hypertension (PH) is a rare disease characterized by considerable morbidity and mortality. Significant progress has been recently achieved in enhancing the identification, diagnosis, and treatment of the disease, as evidenced by the latest guideline. Several additional medical conditions can complicate the overall understanding of the patient's condition, making the diagnosis even more difficult. Genetic and molecular factors, certain toxic drugs (such as methamphetamines, desatinib, or anorexigens), systemic disorders, or other predisposing conditions lead to the remodeling of distal pulmonary arterioles, resulting in pulmonary hypertension. Non-invasive investigations are initially undertaken in suspected cases based on cardiac biomarkers, lung function, and echocardiograms. Nowadays, the definition of pulmonary hypertension (PH) has recently changed, now including patients with mean pulmonary artery pressure &gt;20 mmHg, and hemodynamic evaluation with right heart catheterization remains the diagnostic gold standard. Beyond new medically targeted therapies, there is a greater appreciation for the importance of supervised training in stable PH and the possible role of interventional therapies in select cases. The landscape of PH is in constant change, characterized by progress, innovation, and new medical opportunities.

Shape of Pulmonary Artery Doppler Flow Profile and Right Ventricular Hemodynamics in Neonates

To characterize pulmonary artery Doppler flow profile (PAFP) patterns among infants receiving care in neonatal intensive care units (NICUs) and to examine the association of PAFP patterns with pulmonary and right ventricular (RV) hemodynamics. This is a retrospective study at two tertiary NICUs over 4 years that included neonates who demonstrated a complete tricuspid regurgitation envelope on targeted neonatal echocardiography (TNE). Separate personnel reviewed TNEs to characterize PAFP patterns, divide cohort into PAFP groups, and measure quantitative indices of RV hemodynamics (RV systolic pressure [RVSP], pulmonary artery acceleration time [PAAT] and its ratio with RV ejection time [RVET/PAAT], tricuspid annular plane systolic excursion [TAPSE] and RV output [RVO]), for intergroup comparisons. We evaluated TNEs from 186 neonates with median (IQR) gestational age of 28.5 weeks (25.9-35.9) weeks. Four distinct PAFP patterns were identified: A. near-isosceles triangle (22%), B. right-angled triangle (29%), C. notching (40%), and D. low peak velocity (<0.4 m/s; 9%). Groups A-C demonstrated a stepwise worsening in all indices of PH, while pattern D was associated with lower TAPSE and RVO. Using common definitions of pulmonary hypertension (PH), pattern A performed best to rule out PH [sensitivity range 81-90%] and pattern C for diagnosing PH [specificity range 63-78%]. Inspection of PAFP is a simple bedside echocardiography measure that provides clinically meaningful information on underlying RV hemodynamics and may aid in screening and monitoring of patients for PH in NICUs.

Prognostic value of pulmonary hypertension in patients with advanced heart failure evaluated fort heart replacement therapy in the light of new definition of pulmonary hypertension

Abstract Background Pulmonary hypertension (PH) due to left heart disease is a common complication in heart failure (HF) and is associated with increased mortality. The 2022 ESC Guidelines updated the hemodynamic definition of PH lowering the threshold to mean pulmonary artery pressure (mPAP) &amp;gt;20 mmHg. The relevance of this classification has not been fully explored in the setting of advanced heart failure. Purpose The aim of this study was to investigate epidemiology and clinical outcomes of PH according the 2022 definition in patients with advanced HF candidate to heart replacement therapy (HRT) defined as heart transplantation (Htx) or left ventricular assist device (LVAD) implantation. Methods This study is a retrospective analysis of all adult (&amp;gt; 18 years old) patients that underwent HRT evaluation with an available right heart catheterization from 2003 to 2018. Patients were selected based on HLA typing as a proxy for HRT evaluation. Patients were divided into groups based on the presence of PH with mPAP &amp;lt; 20 mmHg, 21-24 mmHg or &amp;gt; 25 mmHg) to define the impact of the revised classification. Updated definition was then applied to investigate association with prognosis. Study outcome was the composite occurrence of death, urgent HTx or LVAD implantation. Patients were stratified into 3 different eras to account for the extent of the study period. Univariate and multivariate analysis was used to detect risk factors correlated to outcome. Results 625 patients were screened, of whom 619 had an available RHC and were included in the study. Median follow-up was 15 months (IQR 5-39). With the threshold of mPAP &amp;gt; 20 mmHg, 450 patients (73%) had PH as compared to 366 (59%) with the previous definition. Survival from endpoint was significantly lower in both patients with mPAP 21-24 [1 year: 87%; 5-years 53%; OR 1,7 (1,3-3,8); p = 0.005] and &amp;gt; 25 mmHg [1 year 81%, 5 years 50%; OR 2,7 (1,8-4,2); p&amp;lt; 0,001], as compared to those without PH (1 year 91%, 5 years 78%). No difference was found between the groups of increased mPAP. Applying the 2022 definition, 122 (19%) patients had isolated isolated pC-PH, 320 (52%) combined pC-PH and 8 a form of pre-capillary PH. Isolated pC PH and combined pC PH both correlated with worse outcome (OR respectively 2,3; p = 0.0017 and 2,7 p&amp;lt;0.001). In patients with combined PH, PVR &amp;gt; 3 WU was associated with worse survival (88% vs 76% at 1t year). Conclusion In this study of patients with advanced HF evaluated for HRT, the 2022 hemodynamic definition of PH identified an additional 13% of patients. Survival was lower in patients with mPAP &amp;gt; 20 mmHg and these finding support the revised threshold and its association with prognosis in this population. In patients with combined PH, PVR &amp;gt; 3 WU is associated with worse outcomes. In advanced HF, this threshold may retain better prognostic value.

Pulmonary hypertension in patients with heart failure with reduced ejection fraction: impact of the new definition of pulmonary hypertension

Abstract Background Prevalence, haemodynamic character, and associated factors of pulmonary hypertension (PH) in heart failure with reduced left ventricular ejection fraction (HFrEF) according to the 2022 European Society of Cardiology (ESC) PH definition remain unclear. Purpose To describe the prevalence of PH and its phenotypes in patients with advanced HFrEF according to the 2022 PH definition and analyse characteristics of the respective PH groups. Methods We retrospectively analysed 175 consecutive patients (pts) (136 men) with advanced HFrEF referred to a single centre. All pts underwent right heart catheterization (RHC). We identified pts with post-capillary PH which was defined as mean pulmonary artery pressure (mPAP) &amp;gt; 20 mm Hg and pulmonary artery wedge pressure (PAWP) &amp;gt; 15 mm Hg. Pts with mPAP ≤ 20 mm Hg and/or PAWP ≤ 15 mm Hg were labelled as pts without post-capillary PH. According to pulmonary vascular resistance (PVR), PH was further classified as isolated post-capillary (IPC PH) (PVR ≤ 2 Wood units) or combined post- and pre-capillary PH (CPC PH) (PVR ≤ 2 Wood units). We compared selected demographic, clinical, echocardiographic, laboratory, and haemodynamic parameters between the individual groups. Results Median age of the pts was 54 (18-69) years and mean left ventricular ejection fraction (LVEF) 21.9 ± 6.7 %. Out of 175 pts included, 146 (83 %) pts had post-capillary PH. Twenty-nine (17%) pts did not meet the post-capillary PH criteria. Thirty-five (20%) pts had IPC PH and 111 (63%) pts CPC PH. In comparison with the pts without post-capillary PH, pts with post-capillary PH had longer history of HFrEF (6.8 ± 5.6 vs 3.8 ± 4.6 years, p&amp;lt;0.01), lower systolic blood pressure (108 ± 13 vs 120 ± 20 mmHg, p&amp;lt;0.01), lower LVEF (21 ± 6 vs 26 % ± 8 p&amp;lt;0.001), larger left atrial diameter (51 ± 7 vs 45 ± 7 mm, p&amp;lt;0.001), right ventricular diameter (39 ± 9 vs 34 ± 8 mm, p&amp;lt;0.01), higher prevalence of renal insufficiency (29 vs 59%, p&amp;lt;0.05) and atrial fibrillation (12 vs 0 %, p&amp;lt;0.05), and higher natriuretic peptide levels (4565 ± 4087 vs 1976 ± 2376 pg/mL, p&amp;lt;0.001). Patients with CPC PH were older (54 ± 10 vs 47 ± 13 years, p &amp;lt; 0.001), had lower 6-minute walking distance (390 ± 126 vs 439 ± 74 years, p&amp;lt;0.05), higher prevalence of renal insufficiency (33 vs 14 %, p&amp;lt;0.05), and diabetes mellitus (32 vs 11 % p&amp;lt;0.05) than pts with IPC PH. Alteration in pulmonary haemodynamics was more pronounced in CPC PH than IPC PH, with higher mPAP (40 ± 9 vs 29 ± 5 mm Hg, p&amp;lt;0.001), PAWP (27 ± 6 vs 22 ± 5 mm Hg, p&amp;lt;0.001), and lower cardiac index (1.9 ± 0.4 vs 2.3 ± 0.5 L/min/m2, p&amp;lt;0.001). Conclusion Post-capillary PH according to 2022 definition is frequent in pts with advanced HFrEF, with CPC PH prevalence three times as high as in ICP PH. Despite lowering both the threshold for presence of PH and pre-capillary component in the recent definition, PH and CPC PH still seem to reflect a more advanced stage of HFrEF.

Comprehensive echocardiographic evaluation in CTEPH: the impact of TAPSE/sPAP and RVOT acceleration time on the diagnostic performance of peak tricuspid regurgitation velocity

Abstract Introduction The hemodynamic definition of pulmonary hypertension (PH) has been updated, with lowering of the mean pulmonary arterial pressure (mPAP) threshold from 25 to 20 mmHg according to the new 2022 ESC/ERS Guidelines. Peak tricuspid regurgitation velocity (PTRV) remains the key variable for assigning the echocardiographic probability of PH, but a comprehensive echocardiographic evaluation also includes detecting additional signs suggestive of PH. Since the cut-off for PTRV remained the same (&amp;gt;2.8m/s), the potential impact of other echocardiographic variables in the diagnosis of PH according to the new criteria has not been extensively evaluated. Objectives To evaluate the screening power of the standard echocardiographic parameters to detect PH according to the new guidelines and to establish the sensitivity and specificity of adding tricuspid annular plane systolic excursion/systolic pulmonary arterial pressure ratio (TAPSE/sPAP) and right ventricular outflow tract acceleration time (RVOT AT) to PTRV in the diagnosis of PH. Methods Prospective registry of consecutive intermediate-high- and high-risk PE pts submitted to CDT in a single tertiary center. 3 months after the procedure, patients were submitted to a right heart catheterization and echocardiogram to screen chronic thromboembolic pulmonary hypertension (CTEPH). According to new PH criteria, patients were divided in two groups, and echocardiographic parameters were analyzed. ROC curve was performed to evaluate the sensitivity and specificity of each variable. Results 17 pts (60% women, mean age 59 ± 16 years) were included. Among these, 7 pts (41,2%) were diagnosed with pre-capillary PH by RHC at 3 months of follow-up. Among echocardiographic parameters, PTRV (p 0.015), presence of tricuspid regurgitation (0.034) and TAPSE/sPAP (p 0.041) were significantly different between groups. Other parameters did not show significant differences (Fig 1). Regarding PTRV, the cut-off of 2.8 m/s achieved a low sensitivity of 28.6%, although 100% of specificity (AUC 0.643) for the diagnosis of PH. Adding TAPSE/sPAP &amp;lt; 0,55 mm/mmHg (recommended cut-off) to the screening, increased the sensitivity to 42,9% without losing specificity (AUC 0,714). When adding RVOT AT &amp;lt; 105 ms (recommended cut-off) to the analysis, meaning, using three echocardiographic variables combined, it achieved a sensitivity of 85,7%, although the specificity was lower – 80% (AUC 0,829) (Fig 2). Conclusion PTRV and TAPSE/sPAP ratio are the only conventional echocardiographic parameters to be significantly different when comparing patients with and without PH. However, with the recent update in PH criteria, the use of the PTRV recommended cut-off leads to a significant underdiagnosis in our population. Although PTRV did not seem to predict accurately the presence of PH when used alone, when combined with TAPSE/sPAP and RVOT AT, our data suggests a growth in the screening power, without a major loss in specificity.Figure 1Figure 2

By using the new hemodynamic definition of pulmonary hypertension in patients with left heart disease the prognostic value of different phenotypes might be lost

Abstract Background and aims In the 2022 ECS/ERS pulmonary hypertension (PH) guidelines the threshold for increased pulmonary vascular resistance (PVR) was lowered from 3 to 2 Wood Units (WU). Consequently, in patients with PH due to left heart disease, the proportions of patients with combined pre- and postcapillary PH (CpcPH) will increase while the proportion with isolated post-capillary PH (IpcPH) will decrease. In the present study we investigate in patients with left heart disease the impact of this revision in terms of hemodynamic profile, right ventricular (RV) function and prognostication. Methods. Two-hundred and two patients consecutively referred for heart transplant or heart failure work-up were included with right heart catheterization and echocardiography within 48 hours. RV dysfunction was graded using a novel echocardiographic score allocating one point each for (1) systolic pulmonary artery pressure (PAP) &amp;gt;40 mmHg, (2) reduced tricuspid annular plane systolic excursion (TAPSE&amp;lt;17 mm), (3) RV area dilatation, (4) moderate/severe tricuspid regurgitation and (5) reduced inferior cava collapsibility. For assessment of prognostication, we studied the end-point of all-cause mortality. Results. One-hundred and seventy-three patients (86%) had left ventricular ejection fraction &amp;lt;40%. Eighty-nine patients (44%) underwent heart transplantation and the median (25%; 75% percentile) follow-up time (years) was 1.8 (0.35; 6.7). Sixty patients (30%) had mean PAP&amp;lt;20 mmHg, thus no PH. Ninety-six patients (48%) had IpcPH using PVR 3 WU as threshold. Lowering the threshold to PVR 2 WU decreased the corresponding proportion to 24%. The degree of backward failure (PA wedge pressure), forward failure (cardiac index) or RV dysfunction (RVD score) did not significantly differ by the change in hemodynamic definition (Table). With the previous hemodynamic definition, the Kaplan-Meier curves demonstrated increased mortality in CpcPH patients compared with IpcPH (Figure B). Using the new definition this difference was not present (Figure B). Conclusions. The revised hemodynamic definition of PH significantly increases the proportion with CpcPH, but does not significantly alter the hemodynamic or RV function profiles in left heart disease patients with IpcPH or CpcPH phenotype. However, our findings indicate that by using the new hemodynamic definition of IpcPH and CpcPH phenotype the prognostic value of this hemodynamic classification might be lost.Table

Diagnosing post-capillary hypertension in patients with left heart disease: impact of new guidelines.

In 2022, the definition of pulmonary hypertension (PH) in the presence of left heart disease was updated according to the new joint guidelines of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS). The impact of the new ESC/ERS definition on the prevalence of post-capillary PH (pc-PH) and its subgroups of isolated post-capillary (Ipc-PH) and combined pre- and post-capillary PH (Cpc-PH) in patients with left heart disease is unclear. We retrospectively identified N = 242 patients with left heart disease with available data on right heart catheterisation (RHC) and cardiac magnetic resonance imaging (CMR). The proportion of pc-PH and its subgroups was calculated according to the old and new ESC/ERS PH definition. As the old definition did not allow the exact allocation of all patients with pc-PH into a respective subgroup, unclassifiable patients (Upc-PH) were regarded separately. Seventy-six out of 242 patients had pc-PH according to the new ESC/ERS definitions, with 72 of these patients also meeting the criteria of the old definition. Using the old definition, 50 patients were diagnosed with Ipc-PH, 4 with Cpc-PH, and 18 with Upc-PH. Applying the new definition, Ipc-PH was diagnosed in 35 patients (4 newly), and Cpc-PH in 41 patients. No CMR parameter allowed differentiating between Ipc-PH and Cpc-PH, regardless of which guideline version was used. Applying the new ESC/ERS 2022 guideline definitions mildly increased the proportion of patients diagnosed with pc-PH (+ 5.5%) but markedly increased Cpc-PH diagnoses. This effect was driven by the allocation of patients with formerly unclassifiable forms of post-capillary PH to the Cpc-PH subgroup and a significant shift of patients from the Ipc-PH to the Cpc-PH subgroup. Distribution of post-capillary pulmonary hypertension (pc-PH) subgroups according to the European Society of Cardiology/European Respiratory Society (ESC/ERS) PH guidelines from 2015 and 2022 in N = 242 patients with left heart disease.

Updated definition of pulmonary hypertension and outcome after transcatheter aortic valve implantation

ObjectiveThe European Society of Cardiology guidelines have recently defined new cut-offs for pulmonary hypertension (PH) and pulmonary vasculature resistance (PVR; median pulmonary artery pressure (mPAP) >20 instead of 25 mm...

Analysis of Electrocardiographic Criteria of Right Ventricular Hypertrophy in Patients with Chronic Thromboembolic Pulmonary Hypertension before and after Balloon Pulmonary Angioplasty.

Chronic thromboembolic pulmonary hypertension (CTEPH) may lead to typical electrocardiographic changes that can be reversed by balloon pulmonary angioplasty (BPA). The aim of this study was to investigate the significance of rarely used electrocardiogram (ECG) parameters, possible electrocardiographic differences between residual and significantly improved CTEPH and the role of electrocardiographic parameters in low mPAP (mean pulmonary arterial pressure) ranges since the mPAP threshold for the definition of pulmonary hypertension has recently been adjusted (≥25 mmHg to >20 mmHg). Between March 2014 and October 2020, 140 patients with CTEPH and 10 with CTEPD (chronic thromboembolic pulmonary disease) without pulmonary hypertension (PH) were retrospectively enrolled (12-lead ECG and right heart catheterization before and 6 months after BPA). The ECG parameters of right heart strain validated by studies and clinical experience were evaluated. Special attention was paid to six specific ECG parameters. After BPA, the cohort was divided into subgroups to investigate possible electrocardiographic differences with regard to the haemodynamic result. The present study confirmed that the typical electrocardiographic signs of CTEPH can be found on an ECG, can regress after BPA and partially correlate well with haemodynamic parameters. "R V1, V2 + S I, aVL - S V1" was a parameter of particular note. BPA reduced its frequency (47% vs. 29%) statistically significantly after Bonferroni correction (p < 0.001). Moreover, it showed a good correlation with mPAP and PVR (r-values: 0.372-0.519, p-values: < 0.001). Exceeding its cut-off value before therapy was associated with more severe CTEPH before therapy (higher mPAP, PVR, NT-pro-BNP and troponin and lower TAPSE) and an increased risk of death. Exceeding its cut-off value before and after therapy was associated with more severe CTEPH after therapy (higher RAP, mPAP, PVR, NT-pro-BNP and NYHA class) and an increased risk of death. Men tend to be affected more frequently. After subgrouping, it was observed that a higher median mPAP was associated with a higher right atrial pressure (RAP), a higher pulmonary vascular resistance (PVR) and a lower cardiac output (CO) before and after BPA. In addition, under these conditions, more and more severe electrocardiographic pathologies were detected before and after BPA. Some patients with low mPAP also continued to show mild ECG changes after BPA. In some cases, very few to no pathological ECG changes were detected, and the ECG could present as mostly normal in some patients (5% before BPA and 13% after BPA). "R V1, V2 + S I, aVL - S V1" seems to be able to support the diagnosis of CTEPH, indicate therapeutic improvement and estimate haemodynamics. It also seems capable of predicting a (persistent) severe disease with probably increased need for therapy and increased mortality. Mild PH has been observed to have either no or few mild ECG changes. This might complicate the (early) detection of PH.

New 2022 ESC/ERS definition of pulmonary hypertension: can we rely on the same non-invasive echocardiographic parameters?

Abstract Funding Acknowledgements Type of funding sources: None. Introduction The hemodynamic definition of pulmonary hypertension (PH) has been updated, with lowering of the mean pulmonary arterial pressure (mPAP) threshold from 25 to 20 mmHg according to the new 2022 ESC/ERS Guidelines. Although there is no single echocardiographic parameter that reliably informs about PH status, some of the echocardiographic parameters cut-offs remained the same, including a peak tricuspid regurgitation velocity (PTRV) &amp;gt;2.8m/s. The potential underdiagnosis of PH has not been evaluated. Objectives To evaluate the screening power of the standard echocardiographic parameters to detect PH according to the new guidelines and to establish new predictors. Methods A prospective registry of consecutive intermediate-high- and high-risk PE pts submitted to CDT in a single tertiary center was used. 3 months after the procedure, the patients were submitted to a right heart catheterization and echocardiogram to screen chronic thromboembolic pulmonary hypertension (CTEPH). According to new PH criteria, patients were divided in two groups, and echocardiographic parameters were analyzed regarding their predictive power. A ROC curve analysis was performed to evaluate optimal cut-offs in predicting PH according the new guidelines. Results 17 pts (60% women, mean age 59 ± 16 years) were included. Among these, 7 pts (41,2%) were diagnosed with pre-capillary PH by RHC at 3 months of follow-up. Among echocardiographic parameters, PTRV (p 0.015), presence of tricuspid regurgitation (0.034) and right ventricle-pulmonary artery (RV-PA) coupling (p 0.041) were significantly different between groups (Fig 1 A). Other parameters, such as right ventricle dilation (p 0.849), TAPSE (p 0.100), annular tricuspid s’ velocity (p 0.646), right ventricle outflow tract acceleration time (p 0.229) and the presence of pericardial effusion (p 0.849) did not show significant differences. Regarding the PTRV, a ROC curve analysis revealed an PTRV optimal cut-off of 2.6 m/s (pressure gradient 27mmHg) in our population (AUC 0.911, p 0.030, Sn 71.4%, Sp 100%), compared to the conventional cut-off of 2.8 m/s (pressure gradient 31 mmHg) (p 0.470, Sn 28.6%, Sp 100%). The use of PTRV &amp;gt; 2.6 m/s allows to reduce false negatives without losing specificity (Fig 1 B, C and D). Conclusion With the recent update in PH criteria, the use of the conventional PTRV cut-off leads to a significant underdiagnosis in our population. Lowering the PTRV threshold seems to increase sensitivity, without losing specificity. Other standard echocardiographic parameters did not seem to predict accurately the presence of PH.

Who Falls Through the Gap? Revisiting the Pulmonary Hypertension Nomenclature in Atrial Septal Defect Closure

Background: The incidence of atrial septal defect is 1.6 per 1,000 live births accounting for 8-10% of all congenital heart defects. For the majority of patients the clinical course is often benign. However, pulmonary arterial hypertension is known to complicate 9 to 35% of patients with a secundum type atrial septal defect. The purpose of the study is to apply the new definition of pulmonary hypertension to our cohort of atrial septal defect interventional closures. Objectives: We aim to see whether children with atrial septal defect who have a mean pulmonary artery pressure of <20 mmHg have differences in body habitus and outcomes compared with the children with mean pulmonary artery pressure of 21-25mmHg and children with mean pulmonary arterial pressure of >25 mmHg. Methods: The data collection took place from January to March 2022. A retrospective search was carried out at the archives of the hemodynamic laboratory of a large pediatric hospital in Athens, Greece, carrying out a large number of catheterizations for congenital heart disease. Results: In total, we retrieved 78 children, 30 boys and 48 girls with a diagnosis of atrial septal defect. In 17 cases the mean PAP was not documented so the remaining 61 children were stratified according to their mean PAP as mean PAP <20 mmHg, mean PAP >20mmHg but less than 25 mmHg and more than 25 mmHg. Conclusions: There was no difference in height, weight, BMI, BMI centile, sex age, hospital stay between the groups of no pulmonary hypertension, pulmonary hypertension and pulmonary pressures between 20-25 mmHg.

Natural history of chronic thromboembolic pulmonary disease with no or mild pulmonary hypertension

We describe baseline characteristics, disease progression and mortality in chronic thromboembolic pulmonary disease patients as a function of mean pulmonary artery pressure (mPAP) according to new and previous definitions of pulmonary hypertension. All patients diagnosed with chronic thromboembolic pulmonary disease between January, 2015 and December, 2019 were dichotomized according to initial mPAP: ≤20mmHg ('normal') vs 21-24mmHg ('mildly-elevated'). Baseline features were compared between the groups, and pairwise analysis performed to determine changes in clinical endpoints at 1-year, excluding those who underwent pulmonary endarterectomy or did not attend follow-up. Mortality was assessed for the whole cohort over the entire study period. One hundred thirteen patients were included; 57 had mPAP ≤20mmHg and 56 had mPAP 21-24mmHg. Normal mPAP patients had lower pulmonary vascular resistance (1.6 vs 2.5WU, p<0.01) and right ventricular end-diastolic pressure (5.9 vs 7.8mmHg, p<0.01) at presentation. At 3 years, no major deterioration was seen in either group. No patients were treated with pulmonary artery vasodilators. Eight had undergone pulmonary endarterectomy. Over 37 months median follow-up, mortality was 7.0% in the normal mPAP group and 8.9% in the mildly-elevated mPAP group. Cause of death was malignancy in 62.5% of cases. Chronic thromboembolic pulmonary disease patients with mild pulmonary hypertension have statistically higher right ventricular end-diastolic pressure and pulmonary vascular resistance than those with mPAP ≤20mmHg. Baseline characteristics were otherwise similar. Neither group displayed disease progression on non-invasive tests up to 3 years. Mortality over 37 months follow-up is 8%, and mainly attributable to malignancy. Further prospective study is required to validate these findings.

Implication of the new definition of pulmonary hypertension in patients evaluated for heart transplantation

BackgroundThe 2018 World Symposium on Pulmonary Hypertension (WSPH) changed the definition of pulmonary hypertension (PH) with a new threshold of mean pulmonary artery pressure (mPAP) above 20 mmHg. ObjectiveTo evaluate the profile and prognosis of patients with chronic heart failure (HF) considered for heart transplantation with the new definition of PH. MethodsPatients with chronic HF considered for heart transplantation were classified as mPAP≤20mmHg, mPAP 20–25 mmHg, and mPAP≥25mmHg. Using a multivariate Cox model, we compared the mortality of patients with mPAP20–25mmHg, and mPAP≥25mmHg versus those with mPAP≤20mmHg. ResultsOf 693 patients with chronic HF considered for heart transplantation, 12.7%, 77.5% and 9.8% were classified as mPAP20–25mmHg, mPAP≥ 25mmHg and mPAP≤20mmHg. Patients of mPAP ≥ 25mmHg and mPAP 20–25 mmHg categories were older than mPAP ≤ 20 mmHg (56 versus 55 and 52 year-old, p = 0.02) with more frequent co-morbidities. Within 2.8 years, the mPAP20–25mmHg category displayed a higher risk of mortality compared with those of the mPAP≤20mmHg category (aHR 2.75, 95% CI 1.27–5.97, p = 0.01). Overall, the new PH definition using a threshold of mPAP >20 mmHg was associated with a higher risk of death (adj HR 2.71, 95% CI 1.26–5.80) than the previous definition (mPAP >25 mmHg, aHR: 1.35 95% CI 1.00–1.83, p = 0.05). ConclusionsOne out of 8 patients with severe HF are reclassified as having PH following the 2018 WSPH. Patients with mPAP20–25 evaluated for heart transplantation displayed significant co-morbidities and high mortality rates.

Revised Definition of Pulmonary Hypertension and Approach to Management: A Clinical Primer.

The definition of pulmonary hypertension (PH) has changed recently based, in part, on contemporary outcome data and to focus on early disease detection. Now, PH includes patients with mean pulmonary artery pressure >20 mm Hg measured by right heart catheterization. In contrast to the classical era, pulmonary vascular resistance >2.0 Wood units is also used for diagnosis and prognostication. These lowered thresholds aim to identify patients early in the disease course, which is important because delay to diagnosis of PH is common and linked to elevated morbidity and shortened lifespan. This clinical primer highlights key changes in diagnosis and approach to PH management, focusing on concepts that are likely to be encountered frequently in general practice. Specifically, this includes hemodynamic assessment of at-risk patients, pharmacotherapeutic management of pulmonary arterial hypertension, approach to PH in patients with heart failure with preserved ejection fraction, and newly established indications for early referral to PH centers to prompt comanagement of patients with pulmonary vascular disease experts.

How do we respond to the newly-revised haemodynamic definitions of pulmonary hypertension

In August 2022, the European Society of Cardiology (ESC) and the European Respiratory Society (ERS) jointly issued the 2022 ESC/ERS Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension, which updated the hemodynamic diagnostic criteria for pulmonary hypertension, causing widespread debate among experts. How should we respond to the updated diagnostic criteria for pulmonary hypertension? This paper analyzes the origin of the diagnostic criteria for pulmonary hypertension, how to understand the newly revised diagnostic criteria for hemodynamics, the definition of the new criteria for exercise pulmonary hypertension, and the important impact of the new diagnostic criteria on clinical practice, and puts forward countermeasures.