Pulmonary Hypertension Guidelines Research Articles

Potential use of sPAP /PAAT ratio in Severe Functional Tricuspid Regurgitation with Pulmonary Hypertension.

Introduction To date, there is no specific evidence or criteria for the selection of patients with PH and severe tricuspid insufficiency that can be initiated into correction of tricuspid valvulopathy. Tricuspid regurgitation is a risk marker independent of mortality in patients with pulmonary hypertension. The critical factor for the success of the procedure is certainly to find the parameters to select patients so that they do not become just a futile act. Method From the initial group of 271 patients, were selected a final group of 123 patients, all diagnosed with precapillary PH confirmed by catheterization and with tricuspid regurgitation by echocardiography. Patients were in groups 1 and 2 according to the 2022 Pulmonary Hypertension Guidelines. Patients with right to left shunt were not excluded. Results In patients with severe precapillary PH the sPAP/PAAT ratio was close to 1 (0.89± 0.43), while in patients with mild precapillary PH or in the postcapillary group, the sPAP/PAAT ratio was considerably lower (0.47±0.20) p<0.001. The Average sPAP/PAAT of deceased patients was 0.76. 61.70% of deceased patients (42 out of 68) had severe tricuspid regurgitation. Conclusion In our study average sPAP/PAAT ratio of the deceased patients with severe FTR was 0.76 mmHg/ms, nevertheless, this knowledge could have a potential use but is not sufficient for full-informed qualification or disqualification for valve intervention which requires specific TTVR-related data.

The prognostic relevance of exercise pulmonary hypertension in cardiac and pulmonary diseases.

In this review, we provide an overview of the prognostic implications of exPH in patients with various common cardiac and pulmonary diseases. Exercise pulmonary hypertension (exPH) has been recently re-introduced in the current European Society of Cardiology/European Respiratory Society pulmonary hypertension guidelines. Accordingly, exPH is defined as a mean pulmonary arterial pressure (mPAP)/cardiac output ( CO ) slope greater than 3 mmHg/l/min. Key considerations for this re-introduction included increasing understanding on normal pulmonary hemodynamics during exercise and the broadly available evidence on the association of an abnormal mPAP/ CO slope with poor survival in the general population and in different disease entities. Exercise (patho-)physiology has opened a new field for clinical research facilitating recognition of cardiovascular and pulmonary vascular diseases in an early stage. Such early recognition with significant prognostic and possibly therapeutic relevance, but being undetectable at rest, makes exercise pulmonary hemodynamics particularly interesting for common diseases, such as valvular heart disease, left heart disease, and chronic pulmonary disease.

Novel Reference Equations for Pulmonary Artery Size and Pulsatility Using Echocardiography and Their Diagnostic Value in Pulmonary Hypertension

BackgroundAccording to the most recent pulmonary hypertension (PH) guidelines, a main pulmonary artery (MPA) diameter>25 mm on transthoracic echocardiography (TTE) supports the diagnosis of PH. However, the size of the pulmonary artery(PA) may vary according to body size, age, and cardiac phases. Research questions1)What are the reference limits for PA size on TTE, considering differences in body size, sex, and age? 2)What is the diagnostic value of PA size for classifying pulmonary hypertension? 3)How does the selection of different reference groups (healthy volunteers versus patients referred for right heart catheterization (RHC)) influence the diagnostic odds ratio (DOR)? Study Design and MethodsThe study included a reference cohort of 248 healthy individuals as controls, 693 PH patients proven by RHC, and 156 non-PH patients proven by RHC. In the PH cohort, 300 had group-1 PH, 207 had group-2 PH, and 186 with group-3 PH. MPA and right PA(RPA) diameters and areas were measured in the upper sternal short-axis and the suprasternal notch views. Reference limits (5th-95th percentile) were based on absolute values and height-indexed measures. Quantile regression analysis was used to derive median and 95th quantile reference equations for the PA measures. DORs and probability diagnostic plots for PH were then determined using healthy controls and non-PH cohorts. ResultsThe 95th percentile for indexed MPA diameter was 15mm/m in diastole and 19mm/m in systole in both sexes. Quantile regression analysis revealed a weak age effect (pseudo R2 of 0.08 to 0.10 for MPA diameters). Among measures, the MPA size in diastole had the highest DOR, 156.2(68.3-357.5), for detection of group-1 PH. Similarly, the DORs were also high for group-2 and 3 PH when compared to controls but significantly lower compared to non-PH cohort. InterpretationThe study presents novel reference limits for MPA based on height indexing and quantile regression.

Pulmonary Arterial Hypertension (PAH) Group 1: Overview, Workup, Risk Stratification, and Current (and Future) Treatment Approaches

Abstract: Risk assessment (or risk stratification) and both current and future therapies for pulmonary arterial hypertension (PAH) will be discussed in part B. Risk assessment is key in the initial evaluation and follow-up of persons with PAH. Risk assessment provides information on disease severity and mortality, which, over time, have been incorporated into the application of PAH therapies. After the initial risk assessment, a 4-strata approach is recommended at subsequent follow- up evaluations by the 2022 ERS/ESC pulmonary hypertension (PH) guidelines as described initially in COMPERA 2.0. This method appears to have increased sensitivity to changes in risk from baseline to follow-up and to changes in long-term mortality risk. Current PAH therapies target the prostacyclin, endothelin, and nitric oxide pathways. A sequential approach to therapy has been recommended since publication of the 2009 guidelines and, in the most recent iteration incorporates the 4-strata approach at follow-up. Additional therapy is recommended when intermediate- high or high-risk status is present. New therapies are under active investigation that include targeting novel pathways. Sotatercept, a fusion protein that binds to and sequesters select transforming growth factor β superfamily ligands, is the most promising novel therapy at this time. A recent phase 3, randomized, double-blind, placebo-controlled study in group 1 PAH patients showed a statistically significant improvement in 6-minute walk distance and additional studies of this drug in PH populations are ongoing. Progress in phenotyping this heterogeneous disease is being made, and as PAH therapies continue to evolve, the use of personalized treatment regimens may be possible in the care of this complex, and highly morbid and mortal disease.

Is Pulmonary Capillary Wedge Pressure a Reliable Indicator of Postcapillary Pulmonary Hypertension?

Although current pulmonary hypertension (PH) guidelines recommend a pulmonary capillary wedge pressure (PCWP) above 15 mmHg for the detection of a post-capillary component, the rational of this recommendation may not be quite compatible with the peculiar hemodynamics of PH. We hypothesize that a high PCWP alone does not necessarily indicate left-sided disease and this diagnosis can be improved using LV transmural pressure difference (∆PTM). In this two-center, retrospective, observational study; we enrolled 1070 patients with PH who had undergone heart catheterization with the final study population comprising of 961 cases. ∆PTM was calculated as PCWP minus right atrial pressure. The patients with group II PH had significantly higher ∆PTM values (12.6 ± 6.6 mmHg) compared to the other groups (1.1 ± 4.8 in group I, 12.4 ± 6.6 in group II, 2.5 ± 6.4 in group III and 0.8 ± 8.0 in group IV, P<0.001) despite overlapping PCWP values. A ∆PTM cut-off of 7 mmHg identifies left-heart disease when PCWP>15 (area under curve, 0.825; 95% confidence interval, 0.784 to. 0.866; P<0.001). Five-year mortality was significantly higher in patients with high ∆PTM and PCWP subgroup compared to low ∆PTM plus high PCWP (26.1% vs. 18.5%, P=0.027), and low ∆PTM and PCWP subgroups (26.1% vs. 15.6%, P<0.001). ∆PTM has supplementary discriminatory power in distinguishing patients with and without post-capillary PH. In conclusion, a new approach utilizing ∆PTM may improve our understanding of PH pathophysiology and may identify a subpopulation who may potentially benefit from PH-specific treatments.

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

Agreement between direct Fick and bolus thermodilution measurements of cardiac output during right heart catheterisation and the impact on haemodynamic classifications

Abstract Background/Introduction When measuring cardiac output (CO) during right heart catheterisation (RHC), both direct Fick (DF) and bolus thermodilution (TD) methods are endorsed by pulmonary hypertension (PH) guidelines (1). Agreement between these techniques in contemporary practice is not well understood, nor are the diagnostic consequences of disagreement. Purpose To investigate the real-world agreement between DF and TD techniques for measuring CO during contemporary RHC, and evaluate the impact of disagreement on patients’ haemodynamic classification. Methods This was a single-centre study including 182 patients who had CO measured simultaneously by DF and TD methods during RHC. Oxygen consumption (VO2) was measured with an indirect calorimeter. The primary outcome was the agreement between DF and TD derived CO using Bland-Altman analysis. Secondary outcomes included the impact of specific clinical variables on CO agreement, and the frequency of disagreement in determining patients’ haemodynamic classification. Results Disagreement between DF and TD measures of CO was noted, with the DF technique tending to overestimate CO relative to the TD measurement (median DF-CO 5.42 L/min [interquartile range (IQR) 3.90 – 7.41] vs median TD-CO 4.10 L/min [IQR 3.47 – 5.10], p&amp;lt;0.001). For CO values between 3-7 L/min, the error between DF and TD measurements ranged from -4.75% to +44.5%, and limits of agreement were between -69.9% and +101% (Figure 1). Disagreement was increased by 19.2% in patients with at least moderate-to-severe tricuspid regurgitation, and by 16.0% in those with irregular heart rhythms (Table1). The agreement between DF and TD derived pulmonary vascular resistance (PVR) was equally poor. Nonetheless, in 86.3% of patients, the techniques agreed in classifying the PVR as abnormally elevated (&amp;gt; 2 Wood Units), with disagreement occurring more frequently in patients with less severe PH. Conclusions In this real-world study there was poor agreement between DF and TD derived CO, resulting in more than 10% of patients having discrepant haemodynamic classifications. This data highlight that both methods should be utilised in clinical practice, particularly in borderline cases or when the haemodynamic assessment influences treatment eligibility or operative risk assessment. The two techniques should not be used interchangeably for serial surveillance, and the impact of our findings on invasive assessments of valvular pathologies needs further investigation.

Toward the Implementation of Optimal Cardiac Magnetic Resonance Risk Stratification in Pulmonary Arterial Hypertension

The 2022 European Society of Cardiology/European Respiratory Society pulmonary hypertension (PH) guidelines incorporate cardiac magnetic resonance (CMR) imaging metrics in the risk stratification of patients with pulmonary arterial hypertension (PAH). Thresholds to identify patients at estimated 1-year mortality risks of< 5%, 5%to 20%, and > 20%are introduced. However, these cutoff values are mostly single center-based and require external validation. What are the discriminative prognostic properties of the current CMR risk thresholds stratifying patients with PAH? We analyzed data from incident, treatment-naïve patients with PAH from the Amsterdam University Medical Centres, Vrije Universiteit, The Netherlands. The discriminative properties of the proposed CMR three risk strata were tested at baseline and first reassessment, using the following PH guideline variables: right ventricular ejection fraction, indexed right ventricular end-systolic volume, and indexed left ventricular stroke volume. A total of 258 patients with PAH diagnosed between 2001 and 2022 fulfilled the study criteria and were included in this study. Of these, 172 had follow-up CMR imaging after 3months to 1.5 years. According to the CMR three risk strata, most patients were classified at intermediate risk (n= 115 [45%]) upon diagnosis. Only 29(11%) of patients with PAH were classified at low risk, and 114 (44%) were classified at high risk. Poor survival discrimination was seen between risk groups. Appropriate survival discrimination was seen at first reassessment. Risk stratifying patients with PAH with the recent proposed CMR cutoffs from the European Society of Cardiology/European Respiratory Society 2022 PH guidelines requires adjustment because post-processing consensus is lacking and general applicability is limited. Risk assessment at follow-up yielded better survival discrimination, emphasizing the importance of the individual treatment response.

Correlation of QRS/T angle with clinical, echocardiographic, and hemodynamic variables in chronic thromboembolic pulmonary hypertension patients

Objectives: In this study, we aimed to examine the relationships between frontal QRS-T (fQRS-T) angle prognostic risk factors outlined in the current pulmonary hypertension (PH) guidelines and to demonstrate whether the fQRS-T could detect patients with unfavorable echocardiographic and hemodynamic data. Patients and methods: Between July 2009 and February 2023, a total of 33 patients (8 males, 25 females; median age: 61 years; range, 55 to 70 years) with chronic thromboembolic pulmonary hypertension (CTEPH) who underwent electrocardiographic (ECG) examination were retrospectively analyzed. The fQRS-T angle was calculated from surface ECGs. Functional class, 6-min walk distance, and brain natriuretic peptide values were recorded. Two-dimensional echocardiographic data including comprehensive right ventricular (RV) functions, right atrial area (RAA), tricuspid annular systolic plane excursion (TAPSE), systolic pulmonary artery pressure (sPAP), and TAPSE/sPAP ratio were noted. Among invasive hemodynamic variables, sPAP, mean PAP (mPAP), pulmonary vascular resistance (PVR), and cardiac index (CI) were obtained. The correlations between clinical, echocardiographic, and hemodynamic variables were analyzed. Results: There was no significant correlation between clinical variables and fQRS-T angle. The TAPSE and TAPSE/sPAP ratio were negatively correlated with fQRS/T angle (r=-0.37, p=0.02, r=-0.35, and p=0.03, respectively), whereas RV Tei index and RAA were positively correlated with the fQRS-T angle (r=0.53, p=0.014, r=0.47, and p=0.007, respectively). The hemodynamic data including sPAP, mPAP, and PVR were positively correlated with the fQRS-T angle (r=0.32, p=0.048, r=0.34, p=0.034, r=0.35, and p=0.02, respectively) and CI was negatively correlated with the fQRS-T angle (r=-0.30, p=0.048). Conclusion: Our study results suggest that the fQRS/T angle is correlated with poor prognostic echocardiographic and hemodynamic variables in CTEPH patients.

Risk assessment in pulmonary arterial hypertension: A step towards clinical implementation based on the 2022 ESC/ERS pulmonary hypertension guidelines.

Risk assessment in pulmonary arterial hypertension: A step towards clinical implementation based on the 2022 ESC/ERS pulmonary hypertension guidelines.

Iron deficiency in pulmonary vascular disease: pathophysiological and clinical implications.

Iron deficiency is common in pulmonary hypertension, but its clinical significance and optimal definition remain unclear. Phenotypic data for 1028 patients enrolled in the Redefining Pulmonary Hypertension through Pulmonary Vascular Disease Phenomics study were analyzed. Iron deficiency was defined using the conventional heart failure definition and also based upon optimal cut-points associated with impaired peak oxygen consumption (peakVO2), 6-min walk test distance, and 36-Item Short Form Survey (SF-36) scores. The relationships between iron deficiency and cardiac and pulmonary vascular function and structure and outcomes were assessed. The heart failure definition of iron deficiency endorsed by pulmonary hypertension guidelines did not identify patients with reduced peakVO2, 6-min walk test, and SF-36 (P > 0.208 for all), but defining iron deficiency as transferrin saturation (TSAT) <21% did. Compared to those with TSAT ≥21%, patients with TSAT <21% demonstrated lower peakVO2 [absolute difference: -1.89 (-2.73 to -1.04) mL/kg/min], 6-min walk test distance [absolute difference: -34 (-51 to -17) m], and SF-36 physical component score [absolute difference: -2.5 (-1.3 to -3.8)] after adjusting for age, sex, and hemoglobin (all P < 0.001). Patients with a TSAT <21% had more right ventricular remodeling on cardiac magnetic resonance but similar pulmonary vascular resistance on catheterization. Transferrin saturation <21% was also associated with increased mortality risk (hazard ratio 1.63, 95% confidence interval 1.13-2.34; P = 0.009) after adjusting for sex, age, hemoglobin, and N-terminal pro-B-type natriuretic peptide. The definition of iron deficiency in the 2022 European Society of Cardiology (ESC)/European Respiratory Society (ERS) pulmonary hypertension guidelines does not identify patients with lower exercise capacity or functional status, while a definition of TSAT <21% identifies patients with lower exercise capacity, worse functional status, right heart remodeling, and adverse clinical outcomes.

Evaluation of the European Society of Cardiology/European Respiratory Society derived three- and four-strata risk stratification models in pulmonary arterial hypertension: introducing an internet-based risk stratification calculator.

Estimation of prognosis in pulmonary arterial hypertension (PAH) has been influenced by that various risk stratification models use different numbers of prognostic parameters, as well as the lack of a comprehensive and time-saving risk assessment calculator. We therefore evaluated the various European Society of Cardiology (ESC)-/European Respiratory Society (ERS)-based three- and four-strata risk stratification models and established a comprehensive internet-based calculator to facilitate risk assessment. Between 1 January 2000 and 26 July 2021, 773 clinical assessments on 169 incident PAH patients were evaluated at diagnosis and follow-ups. Risk scores were calculated using the original Swedish Pulmonary Arterial Hypertension Registry (SPAHR)/Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA) three-strata model, the updated SPAHR three-strata model with divided intermediate risk, and the simplified three-parameter COMPERA 2.0 four-strata model. The original SPAHR/COMPERA and the updated SPAHR models were tested for both 3-6 and 7-11 available parameters, respectively. Prognostic accuracy [area under the receiver operating characteristic (ROC) curve (AUC)] and Uno's cumulative/time-dependent C-statistics (uAUC) were calculated for 1-, 3-, and 5-year mortality. At baseline, both the original SPAHR/COMPERA and the updated SPAHR models, using up to six parameters, provided the highest accuracy (uAUC = 0.73 for both models) in predicting 1-, 3-, and 5-year mortality. At follow-ups, the updated SPAHR model with divided intermediate risk (7-11 parameters) provided the highest accuracy for 1-, 3-, and 5-year mortality (uAUC = 0.90), followed by the original SPAHR/COMPERA model (7-11 parameters) (uAUC = 0.88) and the COMPERA 2.0 model (uAUC = 0.85). The present study facilitates risk assessment in PAH by introducing a comprehensive internet-based risk score calculator (https://www.svefph.se/risk-stratification). At baseline, utilizing the original or the updated SPAHR models using up to six parameters was favourable, the latter model additionally offering sub-characterization of the intermediate risk group. Our findings support the 2022 ESC/ERS pulmonary hypertension guidelines' strategy for risk stratification suggesting the utilization of a three-strata model at baseline and a simplified four-strata model at follow-ups. Our findings furthermore support the utility of the updated SPAHR model with divided intermediate risk, when a more comprehensive assessment is needed at follow-ups, complementing the three-parameter COMPERA 2.0 model. Larger multi-centre studies are encouraged to validate the utility of the updated SPAHR model. By introducing an internet-based risk score calculator (https://www.svefph.se/risk-stratification), risk assessment is facilitated. Our results support the 2022 ESC/ERS pulmonary hypertension guidelines' risk stratification strategy, additionally suggesting the updated SPAHR three-strata model with divided intermediate risk, as a promising complement to the new simplified three-parameter COMPERA 2.0 four-strata strategy, when a more comprehensive overview is needed.

Left Ventricular Filling Pressure in Chronic Thromboembolic Pulmonary Hypertension

BackgroundChronic thromboembolic pulmonary hypertension (CTEPH) is characterized by obstruction of major pulmonary arteries with organized thrombi. Clinical risk factors for pulmonary hypertension due to left heart disease including metabolic syndrome, left-sided valvular heart disease, and ischemic heart disease are common in CTEPH patients. ObjectivesThe authors sought to investigate prevalence and prognostic implications of elevated left ventricular filling pressures (LVFP) in CTEPH. MethodsA total of 593 consecutive CTEPH patients undergoing a first diagnostic right and left heart catheterization were included in this study. Mean pulmonary arterial wedge pressure (mPAWP) and left ventricular end-diastolic pressure (LVEDP) were utilized for assessment of LVFP. Two cutoffs were applied to identify patients with elevated LVFP: 1) for the primary analysis mPAWP and/or LVEDP >15 mm Hg, as recommended by the current pulmonary hypertension guidelines; and 2) for the secondary analysis mPAWP and/or LVEDP >11 mm Hg, representing the upper limit of normal. Clinical and echocardiographic features, and long-term mortality were assessed. ResultsLVFP was >15 mm Hg in 63 (10.6%) and >11 mm Hg in 222 patients (37.4%). Univariable logistic regression analysis identified age, systemic hypertension, diabetes, atrial fibrillation, calcific aortic valve stenosis, mitral regurgitation, and left atrial volume as significant predictors of elevated LVFP. Atrial fibrillation, calcific aortic valve stenosis, mitral regurgitation, and left atrial volume remained independent determinants of LVFP in adjusted analysis. At follow-up, higher LVFPs were measured in patients who had meanwhile undergone pulmonary endarterectomy (P = 0.002). LVFP >15 mm Hg (P = 0.021) and >11 mm Hg (P = 0.006) were both associated with worse long-term survival. ConclusionsElevated LVFP is common, appears to be due to comorbid left heart disease, and predicts prognosis in CTEPH.

COUNTERPOINT: Pulmonary Vascular Resistance 2.0— Shedding Light or Casting Shadows?

COUNTERPOINT: Pulmonary Vascular Resistance 2.0— Shedding Light or Casting Shadows?

Could Impedance Cardiography be a Non-Invasive Alternative Method of Measuring Cardiac Output in Patients with Pulmonary Hypertension?

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. 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. 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). 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.

Reappraisal of the Transthoracic Echocardiographic Algorithm in Predicting Pulmonary Hypertension Redefined by Updated Pulmonary Artery Mean Pressure Threshold.

Although an adopted echocardiography algorithm based on tricuspid regurgitation jet peak velocity and suggestive findings for pulmonary hypertension has been utilized in the non-invasive prediction of pulmonary hypertension probability, the reliability of this approach for the updated hemodynamic definition of pulmonary hypertension remains to be determined. In this study, for the first time, we aimed to evaluate the tricuspid regurgitation jet peak velocity and suggestive findings in predicting the probability of pulmonary hypertension as defined by mean pulmonary arterial pressure > 20 mm Hg and > 25 mm Hg, respectively. Our study group was comprised of the retrospectively evaluated 1300 patients (age 53.1 ± 18.8 years, female 62.1%) who underwent right heart catheterization with different indications between 2006 and 2018. All echocardiographic and right heart catheterization assessments were performed in accordance with the European Society of Cardiology/European Respiratory Society 2015 Pulmonary Hypertension Guidelines. Although tricuspid regurgitation jet peak velocity showed a significant relation with mean pulmonary arterial pressure in both definitions, suggestive findings offered a significant contribution only in predicting mean pulmonary arterial pressure ≥ 25 mm Hg but not for mean pulmonary arterial pressure > 20 mm Hg. In predicting the mean pulmonary arterial pressure > 20 mm Hg, tricuspid regurgitation jet peak velocity and suggestive findings showed an odds ratio of 2.57 (1.59-4.14, P <.001) and 1.25 (0.86-1.82, P =.16), respectively. In predicting the mean pulmonary arterial pressure ≥ 25 mm Hg, tricuspid regurgitation jet peak velocity, and suggestive findings showed an odds ratio of 2.33 (1.80-3.04, P <.001) and 1.54 (1.15-2.08, P <.001), respectively. The tricuspid regurgitation jet peak velocity > 2.8 m/s and tricuspid regurgitation jet peak velocity > 3.4 m/s were associated with 70% and 84% probability of mean pulmonary arterial pressure > 20 mm Hg and 60% and 76% probability of mean pulmonary arterial pressure ≥ 25 mm Hg, respectively. In contrast to those in predicting the mean pulmonary arterial pressure ≥ 25 mm Hg, suggestive findings did not provide a significant contribution to the probability of mean pulmonary arterial pressure > 20 mm Hg predicted by tricuspid regurgitation jet peak velocity solely. The impact of the novel mean pulmonary arterial pressure threshold on the echocardiographic prediction of pulmonary hypertension remains to be clarified by future studies.

Where we came from and where we are going: a perspective on the practice changing recommendations from the 2022 ESC/ERS pulmonary hypertension guidelines.

Where we came from and where we are going: a perspective on the practice changing recommendations from the 2022 ESC/ERS pulmonary hypertension guidelines.

Using a knowledge translation program to facilitate guideline-and evidence-based patient management: thePAH-QuERI Extension Program.

The Pulmonary Arterial Hypertension‐Quality Enhancement Research Initiative Extension Program was designed to support physicians’ adherence to pulmonary arterial hypertension (PAH) guidelines. Guidelines were followed in >95% of patients with functional class (FC) II/III, but for only 28.6% of FC IV patients (Month 36). Low adherence was driven by FC IV patients’ preference to avoid parenteral treatment.

Training and clinical testing of artificial intelligence derived right atrial cardiovascular magnetic resonance measurements

BackgroundRight atrial (RA) area predicts mortality in patients with pulmonary hypertension, and is recommended by the European Society of Cardiology/European Respiratory Society pulmonary hypertension guidelines. The advent of deep learning may allow more reliable measurement of RA areas to improve clinical assessments. The aim of this study was to automate cardiovascular magnetic resonance (CMR) RA area measurements and evaluate the clinical utility by assessing repeatability, correlation with invasive haemodynamics and prognostic value.MethodsA deep learning RA area CMR contouring model was trained in a multicentre cohort of 365 patients with pulmonary hypertension, left ventricular pathology and healthy subjects. Inter-study repeatability (intraclass correlation coefficient (ICC)) and agreement of contours (DICE similarity coefficient (DSC)) were assessed in a prospective cohort (n = 36). Clinical testing and mortality prediction was performed in n = 400 patients that were not used in the training nor prospective cohort, and the correlation of automatic and manual RA measurements with invasive haemodynamics assessed in n = 212/400. Radiologist quality control (QC) was performed in the ASPIRE registry, n = 3795 patients. The primary QC observer evaluated all the segmentations and recorded them as satisfactory, suboptimal or failure. A second QC observer analysed a random subcohort to assess QC agreement (n = 1018).ResultsAll deep learning RA measurements showed higher interstudy repeatability (ICC 0.91 to 0.95) compared to manual RA measurements (1st observer ICC 0.82 to 0.88, 2nd observer ICC 0.88 to 0.91). DSC showed high agreement comparing automatic artificial intelligence and manual CMR readers. Maximal RA area mean and standard deviation (SD) DSC metric for observer 1 vs observer 2, automatic measurements vs observer 1 and automatic measurements vs observer 2 is 92.4 ± 3.5 cm2, 91.2 ± 4.5 cm2 and 93.2 ± 3.2 cm2, respectively. Minimal RA area mean and SD DSC metric for observer 1 vs observer 2, automatic measurements vs observer 1 and automatic measurements vs observer 2 was 89.8 ± 3.9 cm2, 87.0 ± 5.8 cm2 and 91.8 ± 4.8 cm2. Automatic RA area measurements all showed moderate correlation with invasive parameters (r = 0.45 to 0.66), manual (r = 0.36 to 0.57). Maximal RA area could accurately predict elevated mean RA pressure low and high-risk thresholds (area under the receiver operating characteristic curve artificial intelligence = 0.82/0.87 vs manual = 0.78/0.83), and predicted mortality similar to manual measurements, both p < 0.01. In the QC evaluation, artificial intelligence segmentations were suboptimal at 108/3795 and a low failure rate of 16/3795. In a subcohort (n = 1018), agreement by two QC observers was excellent, kappa 0.84.ConclusionAutomatic artificial intelligence CMR derived RA size and function are accurate, have excellent repeatability, moderate associations with invasive haemodynamics and predict mortality.

CURATIVE BENEFIT OF SUBMUCOPERICHONDRIAL RESECTION OPERATION ON PULMONARY AR

Objective: We aimed at establishing a correlation between deviated nasal septum and pulmonary artery hypertension in patients reporting in Otorhinolaryngology (ENT) clinic at Combined Military Hospital Quetta, and evaluating the beneficial impact of sub mucoperichondrial resection (SMR) operation on right heart myocardial function, primarily related to Pulmonary Arterial Pressures of these patients of longstanding nasal septal deviation.&#x0D; Study Design: Descriptive; hospital-based cross-sectional survey.&#x0D; Place and Duration of Study: Departments of Otorhinolaryngology &amp; Cardiology, Combined Military Hospital, Quetta, from Sep 2019 to Apr 2021.&#x0D; Methodology: Electrocardiographic and 2-Dimensional Echocardiographic parameters of 87 randomized patients suffering from symptomatic longstanding deviated nasal septum (DNS) who had consented to undergo sub mucoperichondrial resection (SMR) operation were compared and studied for any change in status of probability of pulmonary arterial hypertension before and two months after their surgery. Probability of pulmonary hypertension was estimated using probability criteria from updated European Society of Cardiology Pulmonary Hypertension Guidelines 2019. Patients were classified into low, intermediate and high probability depending upon the number of criteria fulfilled by echocardiographic parameters.&#x0D; Results: Two (2.3%) patients suffering from deviated nasal septum presented with p-pulmonale. Twelve (13.8%) reported with right bundle branch block, and 7 (8%) patients demonstrated right axis deviation on electrocardiography. Overall high probability to develop pulmonary hypertension was discovered in 2 (2.3%) patients. We observed a significant improvement in pulmonary artery pressures in patients suffering from long term upper airway obstruction, two months following sub mucoperichondrial resection operation, in terms of maximum velocity and peak tricuspid regurgitation, right ventricle/left..........