Mean Pulmonary Vascular Resistance Research Articles

The relation between socioeconomic status and invasive haemodynamics at evaluation for advanced heart failure.

Socioeconomic deprivation is a risk marker for worse prognosis in patients with heart failure (HF), and a potential barrier to referral for advanced HF evaluation. The relationship between socioeconomic status (SES) and invasive haemodynamics in patients undergoing evaluation for advanced HF therapies is unknown. We combined a consecutive clinical registry of patients evaluated for advanced HF with patient-level data on SES (household income, education, workforce status, cohabitant status and distance from home to tertiary HF centre) derived from nationwide registries. Using this information, the cohort was divided into groups of low-, medium- and high degree of socioeconomic deprivation. The associations between SES and invasive haemodynamics were explored with multiple linear regression adjusted for age and sex. A total of 631 patients were included. The median age was 53years, and 23% were women. Patients in the highest income quartile versus the lowest (Q4 vs. Q1) were older (median age 57 vs. 50years) and more often male (83% vs. 67%), both P<0.001. Increasing household income (per 100000 Danish kroner,1 EUR=7.4 DKK) was associated with lower pulmonary capillary wedge pressure (PCWP)[-0.18mmHg, 95% confidence interval (CI) -0.36 to -0.01, P=0.036] but not significantly associated with central venous pressure (CVP) (-0.07mmHg, 95% CI -0.21 to 0.06, P=0.27), cardiac index (-0.004L/min/m2, 95% CI -0.02 to 0.01, P=0.60), or pulmonary vascular resistance (PVR) (-0.003 Wood units, 95% CI -0.37 to 0.16, P=0.84). Comparing the most deprived with the least deprived group, adjusted mean PVR was higher (0.35 Wood units, 95% CI 0.02 to 0.68, P=0.04), but PCWP (0.66mmHg, 95% CI -1.49 to 2.82, P=0.55), CVP (-0.26mmHg, 95% CI -1.76 to 1.24, P=0.73) and cardiac index (-0.03L/min/m2, 95% CI -0.22 to 0.17, P=0.78) were similar. Most haemodynamic measurements were similar across layers of SES. Nevertheless, there were some indications of worse haemodynamics in patients with lower household income or a high accumulated burden of socioeconomic deprivation. Particular attention may be warranted in socioeconomically deprived patients to ensure timely referral for advanced HF evaluation.

BMP10 reflects pre-capillary pulmonary hemodynamics: association of biomarkers and hemodynamic parameters in pulmonary hypertension

Abstract Background and aims The role of biomarkers in diagnosing pulmonary hypertension (PH) and distinguishing between pre- and post-capillary PH remains poorly understood. We aimed to identify biomarkers with a strong association with mean pulmonary arterial pressure, mPAP (PH diagnosis) and pulmonary vascular resistance, PVR (pre-capillary component), but not with pulmonary arterial wedge pressure, PAWP (post-capillary component). Methods Blood samples were collected in patients undergoing right heart catheterization within a prospective cross-sectional study. Biomarkers measured included BMP10, NT-proBNP, ANG2, ESM1/endocan, FGF23, GDF15, IGFBP7, IL6, MyBPC3, proC3, and proC6/endotrophin. Primary outcomes were mPAP, PVR, and PAWP, while secondary outcomes included PH diagnosis (mPAP &gt; 20 mmHg) and elevated PVR (&gt; 2 Wood units). Multivariable linear and logistic regression models were used to assess the relationship between biomarkers and outcomes. Results Of the 127 patients included (age 66 ± 13 years, 54% female), 73% were diagnosed with PH. BMP10, NT-proBNP, ANG2, MyBPC3, and FGF23 showed a strong association with mPAP (p &lt; 0.001). BMP10 and NT-proBNP were strongly associated with PVR (p &lt; 0.001), while NT-proBNP and ANG2 were strongly associated with PAWP (p &lt; 0.001). NT-proBNP had the strongest association with the diagnosis of PH (area under the curve = 0.76). BMP10 was the only biomarker associated with elevated PVR (OR 1.60, 95%CI 1.01–2.54, p = 0.04) but not with PAWP (p = 0.86). Conclusions Several biomarkers were strongly associated with mPAP, PAWP, and PVR. BMP10 was the only biomarker strongly associated with mPAP and PVR, but not with PAWP, thus reflecting the pre-capillary PH component. Measurement of BMP10 along with NT-proBNP may aid in diagnosing PH. Graphical abstract

Pulmonary hypertension and lung transplantation waitlist outcomes for hypersensitivity pneumonitis

RationaleHypersensitivity pneumonitis is an interstitial lung disease that can progress to pulmonary hypertension which increases mortality. The European Society of Cardiology recently reduced the diagnostic criteria for pre-capillary pulmonary hypertension from mean pulmonary artery pressure ≥25mmHg to ≥20mmHg and pulmonary vascular resistance from ≥3Wu to ≥2Wu. ObjectiveWe investigated the effect of the new criteria on hypersensitivity pneumonitis lung transplantation waitlist outcomes. MethodsWe conducted a retrospective cohort study of the Scientific Registry of Transplant Recipients.1175 hypersensitivity pneumonitis patients listed for lung transplantation were divided into three groups based on the presence of pre-capillary pulmonary hypertension; those with pre-capillary pulmonary hypertension based on old criteria, new criteria, and those without pre-capillary pulmonary hypertension based on either criterion. The individual components of pre-capillary pulmonary hypertension were also assessed with three mean pulmonary artery pressure groups (≤20mmHg, >20mmHg to <25mmHg, ≥25 mmHg) and three pulmonary vascular resistance groups (≤2Wu, >2Wu to <3Wu, ≥3Wu). Survival analysis was performed. Measurements and Main ResultsKaplan-Meier analysis showed a difference in waitlist survival probability among the mean pulmonary artery pressure groups and among the pulmonary vascular resistance groups driven by the most severe groups. Multivariate Cox regression showed no difference between the various pre-capillary pulmonary hypertension groups. Pulmonary vascular resistance ≥3Wu was associated with increased waitlist mortality. ConclusionsThe new thresholds of mean pulmonary artery pressure and pulmonary vascular resistance do not appear to prognosticate waitlist outcomes when combined. PVR ≥3Wu is associated with worse waitlist outcomes and may be useful in prognostication.

Circulating Endothelin 1 but Not Transforming Growth Factor-β Levels Are Reduced after Pulmonary Endarterectomy in Subjects Affected by Chronic Thromboembolic Pulmonary Hypertension: A Prospective Cohort Study.

Background and objectives: Endothelin-1 (ET-1) and transforming growth factor-β (TGF-β) play a pivotal role in the pathophysiology and vascular remodeling of chronic thromboembolic pulmonary hypertension (CTEPH) which is an under-diagnosed complication of acute pulmonary embolism (PE). Currently, pulmonary endarterectomy (PEA) is still the treatment of choice for selected patients suffering from CTEPH. The aim of this study was to evaluate the preoperative and postoperative circulating levels of ET-1 and TGF-β in subjects affected by CTEPH undergoing successful surgical treatment by PEA. Methods: The data from patients diagnosed with CTEPH who underwent PEA at the Foundation IRCCS Policlinico San Matteo Hospital (Pavia, Italy) were prospectively recorded in the Institutional database. Circulating ET-1 and TGF-β levels were assessed by an ELISA commercial kit before PEA, at 3 months and 1 year after PEA. The demographic data, preoperatory mean pulmonary arterial pressure (mPAP), cardiac output (CO), and pulmonary vascular resistance (PVR) were also recorded. Univariate and multivariate analyses were performed. Results: The analysis included 340 patients with complete ET-1 measurements and 206 patients with complete TGF-β measurements. ET-1 significantly decreased both at 3 months (p < 0.001) and at 1 year (p = 0.009) after PEA. On the other hand, preoperatory TGF-β levels did not significantly change after PEA. Furthermore, ET-1, but not TGF-β, was a good predictor for increased mPAP in multivariate analyses (p < 0.05). Conclusions: ET-1 but not TGF β was significantly modulated by PEA in subjects affected by CTEPH up to 1 year after surgery. The mechanisms leading to prolonged elevated circulating TGF-β levels and their clinical significance have to be further elucidated.

Animal models of pulmonary arterial hypertension associated with atrial septal defect

Pulmonary arterial hypertension (PAH) is a well-known complication of congenital heart disease (CHD). The lack of a satisfactory animal model for PAH associated with CHD (PAH-CHD) has limited progress in understanding the pathogenesis of PAH and the development of therapeutic agents. The development of a rat model for PAH associated with atrial septal defect (ASD) was achieved through atrial septal puncture and thermal ablation. Two and 4 weeks after modeling, hematoxylin and eosin staining showed that the vascular thickness, vascular thickness index, vascular area, and vascular area index in pulmonary arteries with an outer diameter of 50–300 μm in the PAH-ASD 2 and 4 weeks group were higher than those in the sham group (all P < 0.05). Alpha-smooth muscle actin (ɑ-SMA) staining showed that the medial thickness, medial thickness index, medial area, and medial area index in pulmonary arteries with an outer diameter of 50–300 µm at 2 and 4 weeks after modeling were significantly higher than those in the sham group (all P < 0.05). Additionally, mean pulmonary arterial pressure (mPAP) and pulmonary vascular resistance (PVR) in the PAH-ASD 2 and 4 weeks groups were significantly higher than those in the sham group (both P < 0.05). Elastin van Gieson staining showed that the vascular obstruction score in the PAH-ASD 2 and 4 weeks group was significantly higher than that in the sham group (both P < 0.05). The PAH-ASD rats were successfully generated. These findings suggest that our model would be useful for further research into the pathogenesis, prevention, and treatment of PAH-ASD.

Post-capillary pulmonary hypertension in heart failure: impact of current definition in the PH-HF multicentre study.

Based on retrospective studies, the 2022 European guidelines changed the definition of post-capillary pulmonary hypertension (pcPH) in heart failure (HF) by lowering the level of mean pulmonary artery pressure (mPAP) and pulmonary vascular resistance (PVR). However, the impact of this definition and its prognostic value has never been evaluated prospectively. Stable left HF patients with the need for right heart catheterization were enrolled from 2010 to 2018 and prospectively followed up in this multicentre study. The impact of the successive pcPH definitions on pcPH prevalence and subgroup [i.e. isolated (IpcPH) vs. combined pcPH (CpcPH)] was evaluated. Multivariable Cox regression analysis was used to assess the prognostic value of mPAP and PVR on all-cause death or hospitalization for HF (primary outcome). Included were 662 HF patients were (median age 63 years, 60% male). Lowering mPAP from 25 to 20 mmHg resulted in +10% increase in pcPH prevalence, whereas lowering PVR from 3 to 2 resulted in +60% increase in CpcPH prevalence (with significant net reclassification improvement for the primary outcome). In multivariable analysis, both mPAP and PVR remained associated with the primary outcome [hazard ratio (HR) 1.02, 95% confidence interval (CI) 1.00-1.03, P = .01; HR 1.07, 95% CI 1.00-1.14, P = .03]. The best PVR threshold associated with the primary outcome was around 2.2 WU. Using the 2022 definition, pcPH patients had worse survival compared with HF patients without pcPH (log-rank, P = .02) as well as CpcPH compared with IpcPH (log-rank, P = .003). This study is the first emphasizing the impact of the new pcPH definition on CpcPH prevalence and validating the prognostic value of mPAP > 20 mmHg and PVR > 2 WU among HF patients.

Pulmonary Flow Management by Combination Therapy of Hemostatic Clipping and Balloon Angioplasty for Right Ventricular-Pulmonary Artery Shunt in Hypoplastic Left Heart Syndrome.

Controlling pulmonary blood flow in patients who have undergone Norwood palliation, especially early postoperatively, is challenging due to a change in the balance of systemic and pulmonary vascular resistance. We applied a combination therapy of clipping and balloon angioplasty for right ventricle-pulmonary artery (RV-PA) shunt to control pulmonary blood flow, but the influence of the combination therapy on the PA condition is uncertain. Retrospectively analysis was conducted of all infants with hypoplastic left heart syndrome who had undergone Norwood palliation with RV-PA shunt at Okayama University Hospital from January 2008 to September 2022. A total of 50 consecutive patients underwent Norwood palliation with RV-PA shunt in this study period. Of them, 29 patients underwent RV-PA shunt flow clipping, and the remaining 21 had unclipped RV-PA shunt. Twenty-three patients underwent balloon angioplasty for RV-PA shunt with clips. After balloon angioplasty, oxygen saturation significantly increased from 69 (59-76)% to 80 (72-86)% (p < 0.001), and the narrowest portion of the clipped conduit significantly improved from 2.8 (1.8-3.4) to 3.8 (2.9-4.6) mm (p < 0.001). In cardiac catheterizations prior to Bidirectional cavo-pulmonary shunt (BCPS), there were no significant differences in pulmonary-to-systemic flow ratio (Qp/Qs), ventricular end-diastolic pressure, Nakata index, arterial saturation, mean pulmonary artery pressure and pulmonary vascular resistance index. On the other hand, in Cardiac catheterizations prior to Fontan, Nakata index was larger in the clipped group (p = 0.02). There was no statistically significant difference in the 5-year survival between the two groups (clipped group 96%, unclipped group 74%, log-rank test: p = 0.13). At least, our combination therapy of clipping and balloon angioplasty for RV-PA shunt did not negatively impact PA growth. Although there is a trend toward better but not statistically significant difference in outcomes in the clipped group compared to the non-clipped group, this treatment strategy may play an important role in improving outcomes in hypoplastic left heart syndrome.

Pretransplant NT-proBNP levels are associated with mortality among lung transplant recipients.

The prognostic significance of pretransplant N-terminal pro-brain (B)-type natriuretic peptide (NT-proBNP) level has not been investigated in lung transplant recipients. The electronic files of 173 patients with chronic lung disease who underwent lung transplantation in 2018-2022 at a tertiary medical center were retrospectively reviewed. Right heart catheterization (RHC) and NT-proBNP determination were performed preoperatively in all cases. Pretransplant demographic, clinical, and laboratory data were compared between posttransplant survivors and nonsurvivors. Correlations of NT-proBNP values with lung function and RHC parameters and all-cause mortality were analyzed. NT-proBNP level correlated positively with mean pulmonary artery pressure (R = 0.51, p < 0.001) and pulmonary vascular resistance (PVR) (R = 0.45, p = 0.0013), and negatively with diffusing lung capacity for carbon monoxide (R = -0.25, p = 0.0017), cardiac index (R = -0.26, p = 0.001), and cardiac output (R = -0.23, p = 0.004). Over a median follow-up time of 23.22 months, 74 patients died. On univariate analysis, mortality was significantly associated with higher log-NT-proBNP (hazard ratio [HR] = 0.54, 95% confidence interval [CI] 1.15-2.05, p = 0.016), older age at transplant registration (HR = 1.033, 95% CI 1.009-1.058, p = 0.0068), higher PVR (HR 1.15, 95% CI 1.07-1.23, p = 0.015), and lower cardiac output (HR = 0.62, 95% CI 0.42-0.92, p = 0.045). On multivariate analysis adjusted for age, sex, and body mass index, mortality significance was maintained only for higher log-NT-proBNP (HR = 1.54, 95% CI 1.12-2.11, p = 0.007). Among lung transplant recipients, pretransplant NT-proBNP levels correlated well with RHC parameters and were strongly associated with posttransplantation mortality. Assessment of NT-proBNP may improve risk stratification of lung transplant candidates.

Better efficacy of sequential combination with balloon pulmonary angioplasty after long-term riociguat for patients with inoperable chronic thromboembolic pulmonary hypertension.

The present study aimed to evaluate the efficacy of long-term riociguat sequentially combined with balloon pulmonary angioplasty (BPA) for patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH). Eight inoperable CTEPH patients were enrolled in this study, who have been administrated riociguat 2.5 mg three times daily for about 8 years, then underwent several sessions of BPA procedures. Data are prospectively collected to evaluate clinical outcomes, hemodynamics, exercise capacity, and right heart size and function by echocardiography at baseline, 8 years after riociguat, and 3 months after the final BPA. Eight patients (mean age 54.9 ± 11.4 years) were treated with riociguat 2.5 mg three times daily for 95.0 ± 10.7 months. Cardiac index (CI) (1.5 ± 0.5 L/min/m2 to 2.4 ± 0.6 L/min/m2, p = 0.005), 6 min walking distance (6MWD) (329.6 ± 87.5 m to 418.1 ± 75.8 m, p = 0.016), and pulmonary vascular resistance (PVR) (1336.9 ± 320.2 dyn·s·cm-5 to 815.4 ± 195.6 dyn·s·cm-5, p = 0.008) were significant improvement after riociguat treatment. Mean 4.1 ± 1.6 additional combinational BPA sessions and mean 18.8 ± 8.1 balloon dilations were performed. Mean pulmonary artery pressure (54.1 ± 11.1 mmHg to 33.6 ± 7.7 mmHg, p = 0.002) and PVR (815.4 ± 195.6 dyn·s·cm-5 to 428.3 ± 151.2 dyn·s·cm-5, p＜0.001) were further decreased. CI (2.4 ± 0.6 L/min/m2 to 2.7 ± 0.7 L/min/m2, p = 0.028) and 6MWD (418.1 ± 75.8 m to 455.7 ± 100.0 m, p = 0.038) were increased significantly. After long-term riociguat treatment, sequential combination with BPA delivered considerably incremental benefits on exercise capacity and pulmonary hemodynamics, as well as right heart size and function of technically inoperable CTEPH patients.

Sleep-disordered breathing and nocturnal hypoxemia in chronic thromboembolic pulmonary disease.

Sleep-disordered breathing (SDB) and nocturnal hypoxemia were known to be present in patients with chronic thromboembolic pulmonary hypertension (CTEPH), but the difference betweenSDB and nocturnal hypoxemia in patients who have chronic thromboembolic pulmonary disease (CTEPD) with or without pulmonary hypertension (PH) at rest remains unknown. Patients who had CTEPH (n = 80) or CTEPD without PH (n= 40) and who had undergone sleep studies from July 2020 to October 2022 at Shanghai Pulmonary Hospital were enrolled. Nocturnal mean SpO2 (Mean SpO2) <90% was defined as nocturnal hypoxemia, and the percentage of time with a saturation below 90% (T90%) exceeding 10% was used to evaluate the severity of nocturnal hypoxemia. Logistic and linear regression analyses were performed to investigate the difference and potential predictor of SDB or nocturnal hypoxemia between CTEPH and CTEPD without PH. SDB was similarly prevalent in CTEPH and CTEPD without PH (P = 0.104), both characterised by obstructive sleep apnoea (OSA). Twenty-two patients with CTEPH were diagnosed with nocturnal hypoxemia, whereas only three were diagnosed with CTEPD without PH (P = 0.021). T90% was positively associated with mean pulmonary arterial pressure (mPAP) and pulmonary vascular resistance in patients with CTEPH and CTEPD without PH (P < 0.001); T90% was also negatively related to cardiac output in these patients. Single-breath carbon monoxide diffusing capacity, sex and mPAP were all correlated with nocturnal hypoxemia in CTEPH and CTEPD without PH (all P < 0.05). Nocturnal hypoxemia was worse in CTEPD with PH; T90%, but not SDB, was independently correlated with the hemodynamics in CTEPD with or without PH.

Detrimental Impact of Comorbid Mental Disorders in Chronic Thromboembolic Pulmonary Hypertension - A Retrospective Observational Study.

Background: The relationship between the prognosis of patients with both chronic thromboembolic pulmonary hypertension (CTEPH) and a mental disorder (MD) remains unclear. Methods and Results: The study group comprised 157 patients with CTEPH who underwent right heart catheterization and were subdivided into 2 groups according to the presence of MDs: MD and non-MD. The patients with MDs were defined as those who had visited a psychiatrist and were under psychotropic drug treatment. The primary outcome was a composite of all-cause death and worsening of PH. The median follow-up period was 1,164 days. The incidence of the primary composite outcome was higher in the MD group than in the non-MD group (24.0% vs. 6.8%), whereas the all-cause mortality rate was comparable between groups (12.0% vs. 6.1%). The mean pulmonary arterial pressure, cardiac index, and pulmonary vascular resistance at baseline were all similar between groups. The Cox proportional hazards model indicated that MD was an independent risk factor for the primary composite outcome (hazard ratio, 2.990; 95% confidence interval, 1.034-8.642). Conclusions: In the present study, concomitant CTEPH and MD was significantly associated with a poor prognosis and such patients should be carefully followed.

Spontaneous hemodynamic improvement after balloon pulmonary angioplasty in patients with chronic thromboembolic pulmonary hypertension is observed within a short term after balloon pulmonary angioplasty.

Natural hemodynamic changesafter balloon pulmonary artery angioplasty (BPA) in a unified state without oxygen administration in patients with chronic thromboembolic pulmonary hypertension (CTEPH) remains undetermined. This study aimed to clarify the delayed changes in the hemodynamics after BPA in patients with CTEPH. We analyzed 73 consecutive patients with CTEPH who underwent BPA between July 2014 and December 2022. We extracted and evaluated hemodynamic data of the right heart catheter without oxygen administration immediately before and after the first BPA; and immediately before the second BPA, as the "post-delayed changes" after BPA. BPA significantly improved the mean pulmonary artery pressure (mPAP, mmHg) and pulmonary vascular resistance (PVR, dyn-s/cm5) from 36 (32-43) mmHg and 449 (312-627) dyn-s/cm5 before the first BPA to 28 (23-32) mmHg and 275 (217-366) dyn-s/cm5 immediately after BPA, and further significantly improved the values to 23 (20-28) mmHg and 225 (175-301) dyn-s/cm5 post-delayed changes after BPA, respectively. Improvement observed on account of delayed changes was observed both with and without pulmonary hypertension drugs. The delayed changes were observed during a period of 5-180days, which did not correlate with the changes in mPAP and PVR. Hemodynamic improvement owing to BPA was observed immediately after BPA; however, further improvement was observed as a result of delayed changes. mPAP improved by 3.4 ± 5.2mmHg and PVR by 53 (10-99) dyn-s/cm5 as delayed improvement. mPAP and PVR showed delayed improved by approximately 10% of their values before BPA.

Elevated pulmonary vascular resistance is associated with increased lung transplant waitlist mortality among patients with chronic obstructive pulmonary disease and pulmonary hypertension: a retrospective cohort analysis

BackgroundThe latest European Society of Cardiology and European Respiratory Society guidelines have changed the definition of both pre-capillary pulmonary hypertension (PH) and severe PH in chronic lung disease. The clinical significance of these new criteria are unclear among patients with chronic obstructive pulmonary disease (COPD)-PH. We aim to examine the clinical significance of the new PH definitions with regards to lung transplant waitlist mortality amongst patients with COPD-PH.MethodsThis was a retrospective cohort study of adult patients with COPD-PH listed for lung transplantation. Kaplan–Meier survival analyses were performed comparing patients with newly defined pre-capillary PH to those without pre-capillary PH and comparing patients with severe PH, defined as pulmonary vascular resistance (PVR) > 5 WU, to those without severe PH. Both mean pulmonary artery pressure (mPAP) and PVR were analyzed for potential cut-off points associated with increased waitlist mortality. Predictors of waitlist mortality were identified via Cox regression.ResultsAmong 6495 patients with COPD-PH listed for lung transplantation, pre-capillary PH was not associated with increased waitlist mortality (logrank p = 0.43), while severe PH was (logrank p < 0.001). Both severe PH (HR 1.79, 95% CI 1.22–2.60, p = 0.003) and PVR > 3.9 WU (HR 1.49, 95% CI 1.14–1.95, p = 0.004) were independently and significantly associated with increased waitlist mortality.ConclusionsPVR may serve as a strong predictor of lung transplant waitlist mortality among patients with COPD-PH as compared to other pulmonary hemodynamic parameters when predicting transplant waitlist mortality.

Severe pulmonary hypertension-interstitial lung disease presenting as right ventricular failure: stabilisation with intravenous prostacyclin and maintenance with inhaled prostacyclin.

Pulmonary hypertension (PH) leads to increased morbidity and mortality in interstitial lung disease (ILD). While the INCREASE trial highlighted the use of inhaled prostacyclin in PH-ILD patients, such therapy may be inadequate when right ventricular failure (RVF) is also present. In this study, we report the use of intravenous prostacyclin in three PH-ILD patients to stabilise right ventricular (RV) function, with a subsequent transition to maintenance therapy with inhaled prostacyclin. We evaluated three consecutive PH-ILD patients with RVF. RV afterload and pulmonary vascular resistance (PVR) were treated with intravenous prostacyclin during the induction phase of the therapy. Patients transitioned from intravenous prostacyclin to the maintenance phase of the treatment with inhaled prostacyclin once three transition criteria were met: cardiac index (CI) >2 L·min-1·m-2, PVR <7 Wood units (WU) and tricuspid annular plane systolic excursion (TAPSE) change >1 mm or TAPSE >1.6 cm. Pre-treatment parameters for the three patients were a mean PVR of 14.3 WU, a mean Fick CI of 1.8 L·min-1·m-2 and a mean TAPSE of 1.4 cm. The average intravenous prostacyclin dose at the time of transition to maintenance therapy was 20.7 ng·kg-1·m-2 of treprostinil. At 3-months follow-up, the mean PVR was 6.3 WU, Fick CI 2.2 L·min-1·m-2 and TAPSE 1.7 cm. This case series of three PH-ILD patients with RVF introduces the concept of an initial intravenous prostacyclin induction phase, followed by a transition to maintenance therapy with inhaled prostacyclin. Further development of this treatment algorithm with a refinement of the transition criteria, potential testing in a clinical trial and a longer-term follow-up period is warranted to improve the outcomes of advanced PH-ILD patients with concomitant RVF.

Methamphetamine-associated pulmonary arterial hypertension: data from the national biological sample and data repository for pulmonary arterial hypertension (PAH Biobank)

ObjectiveThis study compares the clinical and haemodynamic severity of methamphetamine-associated pulmonary arterial hypertension (MA-PAH) with idiopathic pulmonary arterial hypertension (IPAH) and connective tissue-associated pulmonary arterial hypertension (CTD-PAH). It also examines...

Adult-onset idiopathic peripheral pulmonary artery stenosis.

Peripheral pulmonary artery stenosis (PPS) refers to stenosis of the pulmonary artery from the trunk to the peripheral arteries. Although paediatric PPS is well described, the clinical characteristics of adult-onset idiopathic PPS have not been established. Our objectives in this study were to characterise the disease profile of adult-onset PPS. We collected data in Japanese centres. This cohort included patients who underwent pulmonary angiography (PAG) and excluded patients with chronic thromboembolic pulmonary hypertension or Takayasu arteritis. Patient backgrounds, right heart catheterisation (RHC) findings, imaging findings and treatment profiles were collected. 44 patients (median (interquartile range) age 39 (29-57) years; 29 females (65.9%)) with PPS were enrolled from 20 centres. In PAG, stenosis of segmental and peripheral pulmonary arteries was observed in 41 (93.2%) and 36 patients (81.8%), respectively. 35 patients (79.5%) received medications approved for pulmonary arterial hypertension (PAH) and 22 patients (50.0%) received combination therapy. 25 patients (56.8%) underwent transcatheter pulmonary angioplasty. RHC data showed improvements in both mean pulmonary arterial pressure (44 versus 40 mmHg; p<0.001) and pulmonary vascular resistance (760 versus 514 dyn·s·cm-5; p<0.001) from baseline to final follow-up. The 3-, 5- and 10-year survival rates of patients with PPS were 97.5% (95% CI 83.5-99.6%), 89.0% (95% CI 68.9-96.4%) and 67.0% (95% CI 41.4-83.3%), respectively. In this study, patients with adult-onset idiopathic PPS presented with segmental and peripheral pulmonary artery stenosis. Although patients had severe pulmonary hypertension at baseline, they showed a favourable treatment response to PAH drugs combined with transcatheter pulmonary angioplasty.

Characteristics in patients with adult-onset idiopathic peripheral pulmonary artery stenosis

Abstract Background Peripheral pulmonary artery stenosis (PPS) refers to the obstruction of the pulmonary artery from the pulmonary artery trunk to the peripheral arteries. Although pediatric PPS is well described, the clinical characteristics of adult-onset idiopathic PPS have not been established. Purpose To establish the disease profile of adult-onset PPS, we characterized the clinical background, laboratory and imaging findings, treatment, and prognosis. Methods The nationwide survey in Japan, supported by the government, retrospectively collected clinical data on patients with idiopathic PPS from multiple centers. This cohort comprised individuals aged 15 years and above who had pulmonary arterial stenosis confirmed through pulmonary angiography, indeed patients with chronic thromboembolic pulmonary hypertension or Takayasu arteritis were excluded. Patient backgrounds, imaging findings, treatment profiles, right heart catheterization findings, and long-term prognosis were collected. A comparison was made between the data obtained from prior-treatment and post-treatment right heart catheterization. Results Forty-five patients (median age at diagnosis: 38 years [Q1–Q3:28–56]; 29 males [64.4%]) with idiopathic PPS were enrolled from 21 centers. The median period between diagnosis of PPS and the final follow-up was 60 months [Q1–Q3: 24–114 months]. Eight patients (17.8%) were associated with moyamoya disease. Pulmonary angiography was performed in all patients for a definite diagnosis, and stenosis of segmental and peripheral pulmonary arteries was observed in 41 (91.1%) and 36 patients (80.0%), respectively. Thirty-six patients (80.0%) received treatment with pulmonary arterial hypertension (PAH)-specific drugs, 23 patients (51.1%) received combination therapy, and 25 patients (55.6%) received transcatheter pulmonary angioplasty. Right heart catheterization data showed improvements in both the mean pulmonary artery pressure (46 vs. 41 mmHg; p&amp;lt;0.001) and pulmonary vascular resistance (738 vs. 506 dyn.s.cm-5; p&amp;lt;0.001) from baseline to the final follow-up. The 3-, 5-, and 10-year survival rates of patients with idiopathic PPS were 97.6% (95% confidence interval [CI]:83.9–99.7), 89.7% [95% CI:69.9–96.6), and 68.6% (95% CI:43.9–84.2), respectively. Conclusion In this study, the patients with adult-onset idiopathic PPS presented with segmental and peripheral pulmonary artery stenosis. Although patients had severe pulmonary hypertension at baseline, they showed a favorable treatment response to the PAH-specific drugs combined with transcatheter pulmonary angioplasty.

Reversibility and outcome of fenestrated surgical closure in adult patients with congenital left-to-right shunts and pulmonary hypertension: a result of cohard-ph registry

Abstract Background Uncorrected congenital heart disease in the adults often come with a complication of pulmonary hypertension (PH) , resulting in high mortality and low quality of life due to right heart failure and arrythmias. Shunt closure in those patients has been shown to reduce such complications. However, in patients who develop PH, total shunt closure posseses risk that increase mortality. ESC guidelines recommend avoidance of ASD closure and careful evaluation of VSD and PDA closure in those with PVRI&amp;gt;5 WU. Nevertheless, some Asian countries reported good outcome in LtoR shunt closure in patients with PVRI&amp;gt;5. Congenital Heart Disease in Adults and Pulmonary Hypertension (COHARD-PH) Registry is a single tertiary center registry in Indonesia. The data showed that among congenital LtoR shunts, PAH occurred in 66.9% patients and Eisenmenger Syndrome in 18.7%. In this study we aim to evaluate the outcome and the PH reversibility of adult patients underwent fenestrated closure in this registry. Methods Patients with intracardiac shunts who underwent surgical closure with atrial septal fenestration on January 2019-July 2022 were recruited and the follow-up data was obtained within 6 months after surgery. Results Thirty-eight patients underwent surgical fenestrated closure were identified. Age was ranging between 19-70 (mean 39) years-old and 81.6% were female. Thirty patients (30%) were ASD, 7 (18.4%) were VSD, and 1 patient (2.6%) was incomplete AVSD. All patients underwent 4-5 mm atrial septal fenestration despite the repair of their anatomical lesion. Hemodynamic measurement before surgery showed that the average Qp/Qs was 3.2; mPAP 51±17 mmHg and the mean pulmonary vascular resistance index (PVRI) was 7.2±4.4 WU before surgery. We performed acute vasoreactivity test (AVT) to 21 patients and showed positive result, while the rest of the patients did not have AVT due to PVRI of 4 WU or less. Short and mid-term follow up reveal a mean SICU length of stay was 2.5 days and mean total hospital stay was 9 days. Three patients died during their hospital stay after discharged from the SICU due to post operative sepsis. On 6 month follow up, the remaining 35 subjects have no remaining symptoms. The reversibility of PH was assesed by echocardiography and showed that mean TVG was reduced from 78 to 36 (by 54%). None of these patients showed signs of heart failure or pulmonary hypertension on follow up. Conclusion Atrial septal fenestration in patients with adult congenital left to right shunt undergoing surgical closure might be considered in selected patients with pulmonary hypertension including those with positive acute vasoreactivity test. Further study with larger subjects is warranted to evaluate the current guidelines.

Pulmonary Congestion by Conventional Chest Radiography: Relationship With Hemodynamics and Mortality in Patients With Severe Aortic Stenosis

BackgroundThe relationship between chest radiograph (CXR) findings of pulmonary congestion and invasive hemodynamics and clinical outcomes in patients with cardiac diseases is unclear. We assessed the correlation between a CXR-based congestion score (RxCS) and the mean pulmonary artery wedge pressure (mPAWP) and the prognostic impact of RxCS and mPAWP in severe aortic stenosis (AS). MethodsIn 471 patients with severe AS undergoing right heart catheterization and upright CXR, the RxCS was calculated (6 items, maximum score: 10 points) independently by 2 radiologists (average value taken) blinded to clinical data. Congestion was defined as an RxCS > 1. Four patterns were defined based on the presence or absence of congestion (C+ or C–) and elevated (> 15 mm Hg) or normal mPAWP (P+ or P–). ResultsThe median (interquartile range) RxCS was 1 (0-2). Patients with an RxCS > 1 (n = 207) had a higher mean right atrial pressure, mean pulmonary artery pressure, mPAWP, and pulmonary vascular resistance than patients with an RxCS ≤ 1 (n = 264). However, the correlation between the RxCS and the mPAWP was moderate only (r = 0.45). Patients with a C+/P+ pattern had the worst hemodynamics, whereas C–/P– patients had the most favourable constellation. After a median post–valve replacement follow-up of 1361 days, mortality was higher in patients with RxCs > 1 vs ≤ 1 as well as mPAWP > 15 mm Hg vs ≤15 mm Hg. Mortality was highest in C+/P+ patients and lowest in C–/P– patients, whereas it was intermediate in C–/P+ and C+/P– patients. ConclusionsIn AS patients, RxCS and mPAWP have a significant but moderate correlation. Both RxCS and mPAWP provide prognostic information.

Evaluation of right ventricular longitudinal strain in pediatric patients with pulmonary hypertension by two-dimensional speckle-tracking echocardiography.

We aimed to investigate the association between right ventricular longitudinal strain measured by two-dimensional speckle-tracking echocardiography (2D-STE) and right heart catheterization data in pediatric patients with pulmonary hypertension (PH). Two groups were evaluated, each consisting of 58 patients. Group 1, patients with PH; Group 2, normal matched controls. Data were collected from 58 patients with PH who underwent invasive hemodynamic evaluation. Standard transthoracic echocardiographic assessment was performed in all patients under the same circumstances. All patients underwent 2D-STE, and off-line analysis generated right ventricle longitudinal strain (RVLS) and right ventricular free wall strain (RVFW) and collected echocardiographic conventional parameters of right ventricular function, including the control group. The relationship between invasive characteristics and right ventricular function parameters was analyzed. In all, 58 PH patients were included in our study. The mean pulmonary artery pressure (mPAP) and pulmonary vascular resistance (PVR) were strongly correlated with right ventricular free wall strain (RVFW) and right ventricular longitudinal strain (RVLS), moderately correlated with the right ventricle myocardial performance index (Tei index), weakly correlated with the transverse diameter of the right ventricle (RV) and the transverse diameter of the right atrium (RA), and moderately negatively correlated with right ventricular fractional area change (RVFAC). In terms of segments of the right ventricular free wall, the basal segment had the highest correlation coefficient with mPAP and PVR (r = 0.413, 0.523, 0.578, r = 0.421, 0.533, 0.575, p < 0.05, respectively). Tricuspid annular plane systolic excursion (TAPSE), main pulmonary artery diameter (MPA), peak systolic velocity of the right ventricle (RV-S'), and RA area parameters were not associated with mPAP and PVR (p > 0.05). Right ventricular longitudinal strain is a reliable indicator to evaluate right ventricular function in pediatric patients with PH. It can provide valuable reference information for the clinical judgment of the status and severity of the disease in children.