Measurement Of Mean Pulmonary Artery Pressure Research Articles

Association of pulmonary hypertension with the outcome in patients undergoing edge-to-edge mitral valve repair

ObjectivesThe association of pulmonary hypertension (PH) with the outcome after mitral transcatheter edge-to-edge repair (M-TEER) focusing on the new ESC/ERS guidelines definition for PH.BackgroundPH is frequently found in patients with...

Fixed-Dose Ultrasound-Assisted Catheter-Directed Thrombolysis for Acute Pulmonary Embolism Associated with COVID-19.

Background. Fixed-dose ultrasound-assisted catheter-directed thrombolysis (USAT) rapidly improves hemodynamic parameters and reverses right ventricular dysfunction caused by acute pulmonary embolism (PE). The effectiveness of USAT for acute PE associated with coronavirus disease 2019 (COVID-19) is unknown. Methods and results. The study population of this cohort study consisted of 36 patients with an intermediate-high- or high-risk acute PE treated with a fixed low-dose USAT protocol (r-tPA 10–20 mg/15 h). Of these, 9 patients tested positive for COVID-19 and were age–sex-matched to 27 patients without COVID-19. The USAT protocol included, beyond the infusion of recombinant tissue plasminogen activator, anti-Xa-activity-adjusted unfractionated heparin therapy (target 0.3–0.7 U/mL). The study outcomes were the invasively measured mean pulmonary arterial pressure (mPAP) before and at completion of USAT, and the National Early Warning Score (NEWS), according to which more points indicate more severe hemodynamic impairment. Twenty-four (66.7%) patients were men; the mean age was 67 ± 14 years. Mean ± standard deviation mPAP decreased from 32.3 ± 8.3 to 22.4 ± 7.0 mmHg among COVID-19 patients and from 35.4 ± 9.7 to 24.6 ± 7.0 mmHg among unexposed, with no difference in the relative improvement between groups (p = 0.84). Within 12 h of USAT start, the median NEWS decreased from six (Q1–Q3: 4–8) to three (Q1–Q3: 2–4) points among COVID-19 patients and from four (Q1–Q3: 2–6) to two (Q1–Q3: 2–3) points among unexposed (p = 0.29). One COVID-19 patient died due to COVID-19-related complications 14 days after acute PE. No major bleeding events occurred. Conclusions. Among patients with COVID-19-associated acute PE, mPAP rapidly decreased during USAT with a concomitant progressive improvement of the NEWS. The magnitude of mPAP reduction was similar in patients with and without COVID-19.

Differential Expression Profile of microRNAs and Tight Junction in the Lung Tissues of Rat With Mitomycin-C-Induced Pulmonary Veno-Occlusive Disease.

BackgroundPulmonary veno-occlusive disease (PVOD) is characterized by increased pulmonary vascular resistance. Currently, there is a lack of effective treatment. It is of great significance to explore molecular targets for treatment. This study investigated the differential expression profile of miRNAs and tight junction in the lung tissues of rats with mitomycin-C (MMC)-induced PVOD.MethodsA total of 14 rats were divided into the control group and he PVOD group. We measured mean pulmonary arterial pressure (mPAP) and right ventricular hypertrophy index (RVHI). Pathological changes including those in lung tissues, pulmonary venules, and capillary were detected by H&E and orcein staining. Western blot was used to detect GCN2, ZO-1, occludin, and claudin-5 expression. We analyzed the miRNAs profile in the rat lung tissues by high-throughput sequencing. The top differentially expressed miRNAs were validated by using real-time polymerase chain reaction (RT-PCR).ResultsThere were severe pulmonary artery hypertrophy/hyperplasia, thickening, and occlusion in the small pulmonary veins, pulmonary edema, and dilated capillaries in MMC-induced rats with PVOD. In addition, mPAP and RVHI were significantly increased (P < 0.05). The expression of GCN2 was significantly decreased (P < 0.05). A total of 106 differentially expressed miRNAs were identified. According to the fold changes, the top ten upregulated miRNAs were miRNA-543-3p, miRNA-802-5p, miRNA-493-3p, miRNA-539-3p, miRNA-495, miRNA-380-5p, miRNA-214-5p, miRNA-539-5p, miRNA-190a-3p, and miRNA-431. The top 10 downregulated miRNAs were miRNA-201-3p, miRNA-141-3p, miRNA-1912-3p, miRNA-500-5p, miRNA-3585-5p, miRNA-448-3p, miRNA-509-5p, miRNA-3585-3p, miRNA-449c-5p, and miRNA-509-3p. RT-PCR confirmed that miRNA-214-5p was upregulated, while miRNA-141-3p was downregulated (P < 0.05). Functional analysis showed various signaling pathways and metabolic processes, such as fatty acid biosynthesis, tight junction, and the mTOR signaling pathway. In addition, the expression of the tight junction-related protein of ZO-1, occludin, and claudin-5 was significantly decreased in rats with PVOD (P < 0.05).ConclusionmiRNAs may be involved in the pathogenesis of PVOD. Furthermore, ZO-1, occludin, and claudin-5 verification confirmed that the tight junction may be involved in the development of the disease.

Change in Invasively Measured Mean Pulmonary Artery Pressure After Transcatheter Mitral Valve Repair Is Associated With Heart Failure Readmission.

BackgroundPre-existing pulmonary hypertension is associated with poor outcomes after transcatheter mitral valve repair (TMVr) for mitral regurgitation (MR). However, the impact of an immediate change in mean pulmonary artery pressure (ΔmPAP) following TMVr on outcomes is unknown.MethodsPatients who underwent TMVr from December 2015 to February 18, 2020 at our institution for symptomatic 3-4+ MR and who had invasive hemodynamics measured immediately pre- and post-TMVR were included. Multivariate Cox regression analysis was performed to examine the association of ΔmPAP (post-TMVr - pre-TMVr mPAP) with the primary endpoint of heart failure (HF) readmission at 1 year. Secondary endpoints included all-cause mortality and the composite endpoint of HF readmission or all-cause mortality at 1 year.ResultsAmong 55 patients, 55% were men, mean age was 72 ± 14.2 years, and mean ΔmPAP was -1.4 ± 8.2 mm Hg. Overall, HF readmission occurred in 14 (25%), death in 10 (18%), and the composite endpoint in 20 (36%) patients. In multivariable analyses, higher ΔmPAP was significantly associated with HF readmission (hazard ratio (HR) = 1.10, 95% confidence interval (CI): 1.00 - 1.21; P = 0.04). ΔmPAP was not associated with death (HR = 1.04, 95% CI: 0.96 - 1.14; P = 0.33), though there was a numerical but statistically non-significant trend towards the composite endpoint (HR = 1.06, 95% CI: 1.00 - 1.13; P = 0.06) driven by HF readmission.ConclusionHigher ΔmPAP immediately following TMVr was associated with increased HF readmission at 1 year. Larger prospective studies are needed to validate these data and further explore the utility of ΔmPAP as a novel hemodynamic parameter to predict post-TMVR outcomes.

Regional Right Ventricular Abnormalities Implicate Distinct Pathophysiological Conditions in Patients With Chronic Thromboembolic Pulmonary Hypertension.

BackgroundRight ventricular (RV) dysfunction is a prognostic factor for cardiovascular disease. However, its mechanism and pathophysiology remain unknown. We investigated RV function using RV‐specific 3‐dimensional (3D)‐speckle‐tracking echocardiography (STE) in patients with chronic thromboembolic pulmonary hypertension. We also assessed regional wall motion abnormalities in the RV and chronological changes during balloon pulmonary angioplasty (BPA).Methods and ResultsTwenty‐nine patients with chronic thromboembolic pulmonary hypertension who underwent BPA were enrolled and underwent right heart catheterization and echocardiography before, immediately after, and 6 months after BPA. Echocardiographic assessment of RV function included both 2‐dimensional‐STE and RV‐specific 3D‐STE. Before BPA, global area change ratio measured by 3D‐STE was significantly associated with invasively measured mean pulmonary artery pressure and pulmonary vascular resistance (r=0.671 and r=0.700, respectively). Dividing the RV into the inlet, apex, and outlet, inlet area change ratio showed strong correlation with mean pulmonary artery pressure and pulmonary vascular resistance before BPA (r=0.573 and r=0.666, respectively). Only outlet area change ratio was significantly correlated with troponin T values at 6 months after BPA (r=0.470), and its improvement after BPA was delayed compared with the inlet and apex regions. Patients with poor outlet area change ratio were associated with a delay in RV reverse remodeling after treatment.ConclusionsRV‐specific 3D‐STE analysis revealed that 3D RV parameters were novel useful indicators for assessing RV function and hemodynamics in pulmonary hypertension and that each regional RV portion presents a unique response to hemodynamic changes during treatment, implicating that evaluation of RV regional functions might lead to a new guide for treatment strategies.

Assessment of the role of computed tomography versus echocardiography in pulmonary hypertension

BackgroundThe era of diagnosing pulmonary arterial hypertension is rapidly evolving. There are changes in the definition, screening, diagnostic modalities, and disease staging.AimThis study aims to assess pulmonary hypertension using computed tomography (CT) and echocardiography.DesignThis is a cross-sectional study.Participants and methodsThis study included 30 cases diagnosed with pulmonary artery hypertension according to the inclusion and exclusion criteria. All patients were subjected to a careful assessment of history, a skillful clinical examination, and investigations: (a) complete blood picture, liver kidney functions, bleeding profile, and arterial blood gases. (b) Spirometry. (c) Echocardiogram. (d) CT chest.ResultsThe correlation between the ratio of main pulmonary artery and aorta to other study parameters was statistically significant. There was a negative statistically significant correlation between the mP/Ao ratio and oxygen saturation (P=0.001); however, we found a positive significant correlation between the mP/Ao ratio and ejection fraction (P=0.006), systolic pulmonary artery pressure (P<0.0001), and mean pulmonary artery pressure (P<0.0001). In contrast, the correlation was nonsignificant when the mP/Ao ratio was compared with other parameters (P>0.05), and a nonsignificant correlation was also found between systolic pulmonary artery pressure and duration of dyspnea (P>0.05).ConclusionThis study has shown that combining CT and echocardiography in the diagnosis of pulmonary hypertension can be a reliable technique to measure mean pulmonary artery pressure than if any of both tests done separately.

Validation of the Tricuspid Annular Plane Systolic Excursion/Systolic Pulmonary Artery Pressure Ratio for the Assessment of Right Ventricular-Arterial Coupling in Severe Pulmonary Hypertension.

The ratios of tricuspid annular plane systolic excursion (TAPSE)/echocardiographically measured systolic pulmonary artery pressure (PASP), fractional area change/invasively measured mean pulmonary artery pressure, right ventricular (RV) area change/end-systolic area, TAPSE/pulmonary artery acceleration time, and stroke volume/end-systolic area have been proposed as surrogates of RV-arterial coupling. The relationship of these surrogates with the gold standard measure of RV-arterial coupling (invasive pressure-volume loop-derived end-systolic/arterial elastance [Ees/Ea] ratio) and RV diastolic stiffness (end-diastolic elastance) in pulmonary hypertension remains incompletely understood. We evaluated the relationship of these surrogates with invasive pressure-volume loop-derived Ees/Ea and end-diastolic elastance in pulmonary hypertension. We performed right heart echocardiography and cardiac magnetic resonance imaging 1 day before invasive measurement of pulmonary hemodynamics and single-beat RV pressure-volume loops in 52 patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension. The relationships of the proposed surrogates with Ees/Ea and end-diastolic elastance were evaluated by Spearman correlation, multivariate logistic regression, and receiver operating characteristic analyses. Associations with prognosis were evaluated by Kaplan-Meier analysis. TAPSE/PASP, fractional area change/mean pulmonary artery pressure, RV area change/end-systolic area, and stroke volume/end-systolic area but not TAPSE/pulmonary artery acceleration time were correlated with Ees/Ea and end-diastolic elastance. Of the surrogates, only TAPSE/PASP emerged as an independent predictor of Ees/Ea (multivariate odds ratio: 18.6; 95% CI, 0.8-96.1; P=0.08). In receiver operating characteristic analysis, a TAPSE/PASP cutoff of 0.31 mm/mm Hg (sensitivity: 87.5% and specificity: 75.9%) discriminated RV-arterial uncoupling (Ees/Ea <0.805). Patients with TAPSE/PASP <0.31 mm/mm Hg had a significantly worse prognosis than those with higher TAPSE/PASP. Echocardiographically determined TAPSE/PASP is a straightforward noninvasive measure of RV-arterial coupling and is affected by RV diastolic stiffness in severe pulmonary hypertension. URL: https://www.clinicaltrials.gov. Unique identifier: NCT03403868.

Hemodynamic Definition of Pulmonary Hypertension: Commentaryon the Proposed Change by the 6th World Symposiumon Pulmonary Hypertension

The ESC/ERS guidelines (published at the end of 2015) and other international recommendations defined pulmonary hypertension (PH) by an invasively measured mean pulmonary arterial pressure (mPAP) ≥ 25 mmHg at rest. At the 6th World Symposium on Pulmonary Hypertension in Nice a modification of this hemodynamic definition in the sense of lowering the threshold to > 20 mmHg was proposed. A pulmonary vascular resistance (PVR) ≥ 3 Wood units (WU) is additionally required for the diagnosis of pre-capillary PH. This modification must be critically reviewed with regard to the underlying rationale and possible consequences. Therefore, a detailed explanation is required. In particular, it must be made clear that this change currently has no influence on the evidence-based and approval-compliant prescription of drugs for the targeted therapy of pulmonary arterial hypertension (PAH).

Combined automated 3D volumetry by pulmonary CT angiography and echocardiography for detection of pulmonary hypertension.

To assess the diagnostic accuracy of automated 3D volumetry of central pulmonary arteries using computed tomography pulmonary angiography (CTPA) for suspected pulmonary hypertension alone and in combination with echocardiography. This retrospective diagnostic accuracy study included 70 patients (mean age 66.7, 48 female) assessed for pulmonary hypertension by CTPA and transthoracic echocardiography with estimation of the pulmonary arterial systolic pressure (PASP). Gold standard right heart catheterisation with measurement of the invasive mean pulmonary arterial pressure (invasive mPAP) served as the reference. Volumes of the main, right and left pulmonary arteries (MPA, RPA and LPA) were computed using automated 3D segmentation. For comparison, axial dimensions were manually measured. A linear regression model was established for prediction of mPAP (predicted mPAP). MPA, RPA and LPA volumes were significantly increased in patients with vs. without pulmonary hypertension (all p < 0.001). Of all measures, MPA volume demonstrated the strongest correlation with invasive mPAP (r = 0.76, p < 0.001). Predicted mPAP using MPA volume and echocardiographic PASP as covariates showed excellent correlation with invasive mPAP (r= 0.89, p < 0.001). Area under the curves for predicting pulmonary hypertension were 0.94 for predicted mPAP, compared to 0.90 for MPA volume and 0.92 for echocardiographic PASP alone. A predicted mPAP > 25.8mmHg identified pulmonary hypertension with sensitivity, specificity, positive and negative predictive values of 86%, 93%, 95% and 81%, respectively. Automated 3D volumetry of central pulmonary arteries based on CTPA may be used in conjunction with echocardiographic pressure estimates to noninvasively predict mPAP and pulmonary hypertension as confirmed by gold standard right heart catheterisation with higher diagnostic accuracy than either test alone. • This diagnostic accuracy study derived a regression model for noninvasive prediction of invasively measured mean pulmonary arterial pressure as assessed by gold standard right heart catheterisation. • This regression model using automated 3D volumetry of the central pulmonary arteries based on CT pulmonary angiography in conjunction with the echocardiographic pressure estimate predicted pulmonary arterial pressure and the presence of pulmonary hypertension with good diagnostic accuracy. • The combination of automated 3D volumetry and echocardiographic pressure estimate in the regression model provided superior diagnostic accuracy compared to each parameter alone.

Oesophageal or transgastric views for estimating mean pulmonary artery pressure with transoesophageal echocardiography: A prospective observational study.

Recent data suggest that in cardiac surgical patients, the pulmonary artery acceleration time (PAT) is useful for estimating mean pulmonary artery pressure (MPAP) noninvasively with transoesophageal echocardiography (TOE). The pulmonary valve can be visualised from multiple echocardiographic windows, but it is unclear which, if any, view correlates best with MPAP. To compare the PAT measured with TOE from oesophageal and transgastric views with MPAP obtained invasively with a pulmonary artery catheter. A prospective observational study. St. Vincent's Hospital, Melbourne, a university tertiary referral centre in Australia. Sixty-three patients having cardiac surgery were included in our study. All patients had insertion of both a TOE probe and pulmonary artery catheter; this is the routine standard of care in our centre. Nil. During a period of haemodynamic stability, the PAT was measured first from an oesophageal view and then immediately after from a transgastric view. The results were then compared with the invasively measured MPAP. Simultaneous measurements of MPAP and PAT were taken in 63 patients. In two patients, these measurements were not possible in the transgastric position due to an inability to visualise the right ventricular outflow tract and pulmonary valve. A Bland-Altman analysis of the PAT measured from the upper oesophageal and transgastric views showed a mean difference of 1 ms and limits of agreement of -18 to 16 ms. The area under the receiver operating curves for predicting pulmonary hypertension with PAT were upper oesophageal view 0.99 [95% confidence interval (CI), 0.98 to 1.00] and transgastric view 0.99 (95% CI, 0.97 to 1.00). The agreement between the results from these two views in the diagnosis of pulmonary hypertension (defined as PAT < 107 ms) was 93.4% with a kappa of 0.85 (95% CI, 0.59 to 1.00). There is an inverse curvilinear relationship between PAT and MPAP. Using a cut-off of 107 ms, the upper oesophageal view predicted pulmonary hypertension (defined as MPAP > 25 mmHg) with a sensitivity of 94.7% and specificity of 97.6%. The transgastric view predicted pulmonary hypertension with a sensitivity of 89.4% and specificity of 95.2%. Oesophageal and transgastric measurements of PAT have close agreement and a similar high ability to discriminate between people with and without pulmonary hypertension. The transgastric measurement was unobtainable in a small percentage of patients and required more probe manipulation. We would recommend PAT measurement in the upper oesophageal view.

Non-invasive methods for estimating mPAP in COPD using cardiovascular magnetic resonance imaging

PurposePulmonary hypertension (PH) is associated with a poor outcome in chronic obstructive pulmonary disease (COPD) and is diagnosed invasively. We aimed to assess the diagnostic accuracy and prognostic value of non-invasive cardiovascular magnetic resonance (CMR) models.MethodsPatients with COPD and suspected PH, who underwent CMR and right heart catheter (RHC) were identified. Three candidate models were assessed: 1, CMR-RV model, based on right ventricular (RV) mass and interventricular septal angle; 2, CMR PA/RV includes RV mass, septal angle and pulmonary artery (PA) measurements; 3, the Alpha index, based on RV ejection fraction and PA size.ResultsOf 102 COPD patients, 87 had PH. The CMR-PA/RV model had the strongest diagnostic accuracy (sensitivity 92%, specificity 80%, positive predictive value 96% and negative predictive value 63%, AUC 0.93, p<0.0001). Splitting RHC-mPAP, CMR-RV and CMR-PA/RV models by 35mmHg gave a significant difference in survival, with log-rank chi-squared 5.03, 5.47 and 7.10. RV mass and PA relative area change were the independent predictors of mortality at multivariate Cox regression (p=0.002 and 0.030).ConclusionCMR provides diagnostic and prognostic information in PH-COPD. The CMR-PA/RV model is useful for diagnosis, the RV mass index and PA relative area change are useful to assess prognosis.Key Points• Pulmonary hypertension is a marker of poor outcome in COPD.• MRI can predict invasively measured mean pulmonary artery pressure.• Cardiac MRI allows for estimation of survival in COPD.• Cardiac MRI may be useful for follow up or future trials.• MRI is potentially useful to assess pulmonary hypertension in patients with COPD.

Novel formula to measure mean pulmonary artery pressure

Novel formula to measure mean pulmonary artery pressure

New Formulae for Calculating Left Heart Pressure in Pulmonary Artery Hypertension Patients at the Bedside

Objective: To compare new empirical formulae in HeartSmart, to calculate the mean pulmonary artery and mean pulmonary artery occlusion (wedge) pressures to that of the Pulmonary Artery Catheter Thermodilution Method. Method: 20 adult patients with pulmonary artery hypertension admitted on to Bradford Royal Infirmary NHS Trust Hospital UK Intensive Care Unit, where haemodynamic monitoring is routinely performed as part of the treatment using the thermodilution method compared to the HeartSmart to guide interventional fluid resuscitation etc. Each subject has six paired values of each left heart pressure and cardiac indexes to test the robustness of HeartSmart against the perceived ‘gold standard’ thermodilution method. This was a double blind study the haemodynamic variables of each technology were close enough to each other. Results: Bland-Altman plot comparing the measured mean pulmonary artery pressure (MPAP) mmHg to the Heartsmart calculated pulmonary artery pressures (CPAP) mmHg: For mean of the differences between MPAP mmHg and CPAP mmHg = - 1.08 mmHg, Legend 1 . Comparing the measured mean pulmonary artery occlusion (wedge) pressure (MPWP) mmHg to the HeartSmart calculated pulmonary artery occlusion (wedge) pressure (CPWP) mmHg: For mean of the differences between MPWP mmHg and CPWP mmHg = - 0.56 mmHg, Legend 2 . Comparing the measured mean cardiac index in litres/min/m 2 MCI to the HeartSmart calculated mean cardiac index in litres/min/m 2 CCI: For mean of the differences between MCI litres/min/m 2 and CCI litres/min/m 2 = -0.03 litres/min/m 2 ; Legend 5 . Conclusion: The results demonstrate HeartSmart TM software compares favourably with the Pulmonary Artery Catheter thermodilution method at the bedside and could be used interchangeably or replace PACTD. Background: The incidence of pulmonary artery hypetension is on the increase in the United Kingdom. Primary pulmonary arterial hypertension is a rare disease that is characterized by increased pulmonary-artery pressure in the absence of common secondary causes of pulmonary hypertension, such as viruses auto immune system, chronic heart, lung, or thromboembolic disease. Before the advent of new and some novel therapies, patients with idiopathic or familial pulmonary arterial hypertension average survival rates were less than 7 years. Despite the progress in diagnostic techniques and treatment, pulmonary arterial hypertension is becoming more common although remaining a progressive, debilitating fatal disease. The clinical signs and symptoms can be nonspecific, patients often receive a diagnosis too late as the condition advances, deteriorates and of course treatment is less effective. The use of the (PAC) right heart floatation pulmonary artery catheter (Swan-Ganz) to measure the mean pulmonary artery and mean pulmonary artery occlusion (capillary wedge) pressures is one means of confirming the diagnosis of pulmonary artery hypertension, although its use is not appropriate in neonates or paediatric cases.

Sequencing of mutations in the serine/threonine kinase domain of the bone morphogenetic protein receptor type 2 gene causing pulmonary arterial hypertension.

Objective:Germline mutations in the bone morphogenetic protein receptor type-2 (BMPR2) gene are considered to be a major risk factor for pulmonary arterial hypertension (PAH). BMPR2 mutations have been reported in 10%–20% of idiopathic PAH and in 80% of familial PAH cases. The aim of this study was to evaluate the frequency of mutations in the serine/threonine kinase domain of the BMPR2 gene in a group of patients from a single PAH referral center in Turkey.Methods:This cross-sectional study used a DNA-sequencing method to investigate BMPR2 mutations in the serine-threonine-kinase domain in 43 patients diagnosed with PAH [8 with idiopathic PAH and 35 with congenital heart disease (CHD)] from a single PAH referral center. Patients were included if they had a hemodynamically measured mean pulmonary arterial pressure of >25 mm Hg with a mean pulmonary capillary wedge pressure of ≤15 mm Hg. Patients with severe left heart disease and/or pulmonary disease that could cause pulmonary hypertension were excluded. Associations between categoric variables were determined using the chi-square test. Differences between idiopathic and CHD-associated PAH groups were compared with the unpaired Student’s t-test for continuous variables.Results:We detected a missense mutation, [p.C347Y (c.1040G>A)], in one patient with idiopathic PAH in exon 8 of the BMPR2 gene. The mutation was detected in a 27-year-old female with a remarkable family history for PAH. She had a favorable response to endothelin receptor antagonists. No mutations were detected in the exons 5–11 of the BMPR2 gene in the PAH-CHD group.Conclusion:A missense mutation was detected in only one of the eight patients with idiopathic PAH. The BMPR2 missense mutation rate of 12.5% in this cohort of Turkish patients with idiopathic PAH was similar to that seen in European registries. The index patient was a young female with a family history remarkable for PAH; she had a good long-term response to PAH-specific treatment, probably due to the early initiation of the treatment. Genetic screening of families affected by PAH might have great value in identifying the disease at an early stage.

Abstract 14798: Right Atrial Function as Prognostic factor in Patients with Pulmonary Hypertension

Background: The development of right ventricular (RV) dysfunction in patients with pulmonary hypertension (PH) has been associated with adverse outcomes. Right atrial (RA) function could be a prognostic factors as well as RV function, but non-invasive evaluation of RA function is limited. Our objective was thus to test the hypothesis that RA function was associated with hemodynamic parameters of RV performance in PH patients. Methods: Eighty PH patients with mean pulmonary artery pressure (PAP) of 40±11mmHg (all≥25mmHg) were recruited in this study. RA function was assessed by using two-dimensional speckle-tracking strain from RV-focused apical 4-chamber view. RA strain was calculated with the reference point set at the P wave, which enabled the recognition of peak negative (RAneg), positive strain (RAposi), and the sum of those values (RAtotal), corresponding to RA contractile, conduit, and reservoir function, respectively. All patients underwent right-heart catheterization for measurement of mean PAP and pulmonary vascular resistance (PVR). Results: RAneg (r=0.24 and p=0.03), RAposi (r=0.31 and p=0.01) and RAtotal (r=0.35 and p=0.001) were significantly correlated with mean PAP. In addition, RAposi (r=0.41 and p&lt;0.001) and RAtotal (r=0.44 and p&lt;0.001) were also correlated with PVR. Conclusions: Non-invasively assessed RA strains were associated with mean PAP and PVR. RA strain may be of a valuable additive factor for the management of PH patients, and thus have potential clinical applications.

Blood flow vortices along the main pulmonary artery measured with MR imaging for diagnosis of pulmonary hypertension.

To approximate the functional relationship between invasively measured mean pulmonary arterial pressure (mPAP) and the phase-contrast magnetic resonance (MR) imaging-derived duration of vortical blood flow along the main pulmonary artery and to analyze its applicability for noninvasive diagnosis of pulmonary hypertension (PH) and borderline mPAP. The local ethics review board approved this prospective study of 145 patients suspected of having PH (69 patients with PH, 19 patients with borderline mPAP, and 57 patients with normal mPAP) who underwent right heart catheterization (RHC) and three-directional phase-contrast MR imaging of the main pulmonary artery. Velocity fields were viewed with dedicated software and evaluated for the duration of vortical blood flow in the main pulmonary artery (tvortex, the percentage of cardiac phases with vortex present). The relationship between mPAP at RHC and tvortex was assessed by means of a segmented linear regression model, and by Bland-Altman and receiver operating characteristic curve analyses. The relationship between mPAP and tvortex was described adequately (R(2) = 0.95) as linearly increasing, from tvortex of 0% (mPAP ≤ 16.0 mm Hg) with a slope of 1.59% per millimeter of mercury. The standard deviation between mPAP values derived from RHC and those estimated by using tvortex was 3.9 mm Hg. The area under the curve for tvortex-based diagnosis of PH was 0.994 (95% confidence interval [CI]: 0.982, 0.998), and the calculated PH cut-off value (tvortex ≥ 14.3%) resulted in sensitivity of 0.97 (95% CI: 0.90, 0.99) and specificity of 0.96 (95% CI: 0.89, 0.99). Vortical blood flow with tvortex less than 14.3% was specific for borderline mPAP. Duration of vortical blood flow in the main pulmonary artery that is determined by using phase-contrast MR imaging allows accurate estimation of elevated mPAP and diagnosis of PH. Clinical trial registration no. NCT00575692.

Congenital heart disease‐associated pulmonary arterial hypertension: preliminary results from a novel registry

Pulmonary arterial hypertension (PAH) frequently accompanies childhood congenital heart disease (CHD) and may persist into adult life. The advent of specific PAH therapies for PAH prompted formation of a national Australian and New Zealand registry in 2010 to document the incidence, demographics, presentation and outcomes for these patients. This multicentre, prospective, web-based registry enrols patients with CHD-associated PAH being followed in a tertiary centre. The inclusion criteria stipulated patient age ≥16 years, a measured mean pulmonary arterial pressure >25 mmHg at rest or echocardiographical evidence of PAH or a diagnosis of Eisenmenger syndrome, and followed since 1 January 2000. A single observer collected standardised data during a series of site visits. Of the first 50 patients enrolled, 30 (60%) were female. The mean age (standard deviation (SD)) at the time of PAH diagnosis or confirmation in an adult centre was 27.23 (10.07) years, and 32 (64%) patients are currently aged >30 years. Fourteen (28%) patients were in World Health Organization Functional Class II and 36 (72%) in Class III at the time of diagnosis. Forty-seven of 50 (94%) had congenital systemic-pulmonary shunts, and 36 (72%) never underwent intervention. Thirteen (26%) had Down syndrome. Confirmation of PAH by recent cardiac catheterisation was available in 30 (60%) subjects. During follow up, a total of 32 (64%) patients received a PAH-specific therapy. CHD associated with PAH in adult life has resulted in a new population with unique needs. This registry will allow documentation of clinical course and long-term outcomes for these patients.

Prospective Multicentric Survey On Pulmonary Hypertension (PH) in Adults with Sickle Cell Disease.

Abstract 572 Introduction :Pulmonary hypertension (PH) has emerged as life threatening complication in patients with sickle cell disease. It is usually diagnosed at echocardiography by estimating systolic Pulmonary Artery Pressure (PAP) through the measurement of a velocity of tricuspid regurgitation (TRV) > 2.5 m/s. Pulmonary Hypertension diagnosed as so could affect up to 30% of patients with sickle cell disease (SCD). However, according to most guidelines, PH is rather defined by measurement of mean PAP ≥ 25 mmHg at right heart catheterism. Whether measurement of TRV ≥ 2.5 m/s at echocardiography accurately identifies patients with PH in patients with SCD, has never been evaluated.We conducted a prospective multicentre study in 403 consecutive adult out-patients in stable clinical condition, recruited after informed consent, during 2 years (from Feb.2007 to March 2009) from 2 SCD referral centers in France (1 in Paris and 1 in Martinique). Follow -up was planned for 3 years. All patients had Doppler echocardiography, pulmonary function tests and 6 min walk test. RHC was systematically performed in case of TRV3 2.5m/sec. Results.Of the 385/403 patients (SS in 379, 6 Sb0thalassemia) with all data availabel, sex ratio 1.5 , mean age 34±10 yrs, 96 had TRV 3 2.5m/s (25 %) and underwent RHC. There was no PH in 72/96 with mPAp=19 ±3mmHg (false positive of echocardiography=75%). In 24 patients, m PAP was elevated (30 ± 0.4m/s). Mechanism of PH was: post-capillary PH in 13 patients (elevated pulmonary capillary wedge pressure) - hyperkinetic state in 5 patients (high cardiac output, normal pulmonary vascular resistance) - pre-capillary pulmonary arterial hypertension (PAH) in 6 patients (normal capillary wedge pressure, increased vascular resistance). Patients with a confirmed PH at RHC were significantly older (45 vs 33 yrs), had higher LDH, NT-Pro BNP, and a lower 6 min walk distance. There was no influence of long term treatment (hydroxyurea, chronic transfusion) on PH.During follow-up (mean=11 months), 3 patients died, all in the group of patients with TRV32.5 m/sec and confirmed PH at RHC (mPAP 3 25 mmHg). The 24 patients having confirmed PH with RHC display similar biological and hemodynamic profile to the 18/42 with TRV3 2.5m/sec among the 195 patients (132 SS,35SC,23S-Thalassemia b0or b+) of the Gladwin study(1), consenting to have RHC. The use of a higher cut-off value of TRV, 2.8 m/sec, decreases the false positive rate from 75% to 50%. However, 29% of patients with pulmonary hypertension (7/24) would have been undiagnosed. ConclusionPH confirmed by gold-standard technique (RHC) is relatively rare among SCD patients, with a prevalence of 24/385 (6%). PAH was present in only 6 patients (1.6%). In this population, a TRV 2.5m/s on Doppler echocardiography is inadequate to diagnose PH. The loose relationship found between TRV and pulmonary hypertension in patients with sickle cell disease does not support its use as a routine screening tool, whatever the cut-off used. Although PH on RHC was generally modest, PH seems to be an important prognostic factor. The large difference on mortality compared to previous studies will be discussed. Disclosures:No relevant conflicts of interest to declare.

Measurement of cardiac output and pulmonary transit time for assessment of pulmonary vascular resistance in domestic piglets

The degree of elevated pulmonary vascular resistance (PVR) is a crucial clinical parameter. Cardiac output (CO) and pulmonary transit time (PTT) can be ascertained by a number of radiological methods. A close functional relationship between CO, PTT and PVR would facilitate non-invasive PVR measurements. One-hundred and fifty-one measurements were made in six piglets. Pressures in the pulmonary and systemic circulation were measured invasively. Cardic output was determined by the use of a Doppler flow probe around the truncus pulmonalis. Temperature sensors were placed in the pulmonary truncus and left atrium. Elevated PVR was produced by repeated air embolism. After injection of ice-cold saline, the time span between the minimal temperature in the truncus pulmonalis and the left atrium was taken as PTT. The CO and PTT were inserted into a new formula derived from the Hagen-Poiseuille law for the calculation of the PVR model. Numerical constants of the formula were calculated by the robust method of minimization. The PVR values, as calculated from invasively measured mean pulmonary artery pressure, mean left atrial pressure and CO, served as reference. In the six piglets, the PVR model and PVR reference showed a strong linear correlation with r = 0.923. The Bland-Altman plot revealed a standard deviation of -0.64/+0.67 Wood units. Cardiac output, PTT and PVR showed a close functional relationship. With a correction for blood viscosity and body size, this relationship could be used for non-invasive clinical measurements of PVR.

Pulmonary venous wedge pressure provides a safe and accurate estimate of pulmonary arterial pressure in children with shunt-dependent pulmonary blood flow

To compare two methods of pulmonary arterial pressure measurement in children with shunt-dependent pulmonary blood flow. In children with shunt-dependent pulmonary blood flow, direct assessment of pulmonary arterial pressure requires passage of a catheter across the shunt. This can be technically difficult and dangerous. Use of the pulmonary venous wedge pressure offers an alternative but has not been validated in this patient population. We prospectively studied 18 children with shunt-dependent pulmonary blood flow. Pulmonary venous wedge pressure and directly measured pulmonary arterial pressures were independently assessed by two blinded cardiologists. Directly measured mean pulmonary arterial pressure and pulmonary venous wedge pressure are closely correlated (R2 = 0.80, P < 0.01). Agreement between the two measures is improved at lower mean pressures with greater differences at higher pressures. For 20 of 24 ipsilateral measurements, pulmonary venous wedge pressure was > or = directly measured pulmonary arterial pressure. Pulmonary venous wedge pressure never underestimated pulmonary arterial pressure by more than 3 mm Hg. Pulmonary venous wedge pressure provides a safe and accurate means of estimating pulmonary arterial pressure in children with shunt-dependent pulmonary blood flow. The slightly lower pressures seen on direct measurement compared with the reverse pulmonary vein may reflect impairment of flow across the shunt by the catheter.