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Cardiac output (CO) is one of the primary prognostic factors evaluated during the follow-up of patients treated for pulmonary hypertension (PH). It is recommended that it be measured using the thermodilution technique during right heart catheterization. The difficulty to perform iterative invasive measurements on the same individual led us to consider a non-invasive option. The aims of the present study were to assess the agreement between CO values obtained using bioreactance (Starling™ SV) and thermodilution, and to evaluate the ability of the bioreactance monitor to detect patients whose CO decreased by more than 15% during follow-up and, accordingly, its usefulness for patient monitoring. A prospective cohort study evaluating the performance of the Starling™ SV monitor was conducted in patients with clinically stable PH. Sixty patients referred for hemodynamic assessment were included. CO was measured using both the thermodilution technique and bioreactance during two follow-up visits. A total of 60 PH patients were included. All datasets were available at the baseline visit (V0) and 50 of them were usable during the follow-up visit (V1). Median [IQR] CO was 4.20 l/min [3.60-4.70] when assessed by bioreactance, and 5.30 l/min [4.57-6.20] by thermodilution (p<0.001). The Spearman correlation coefficient was 0.51 [0.36-0.64], and the average deviation on Bland-Altman plot was -1.25 l/min (95% CI [-1.48-1.01], p<0.001). The ability of the monitor to detect a variation in CO of more than 15% between two follow-up measurements, when such variation existed using thermodilution, was insufficient for clinical practice (AUC = 0.54, 95% CI [0.33-0.75])., Competing Interests: The authors have declared that no competing interests exist., (Copyright: © 2024 Turquier et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.)

Over the last few years, the advent of balloon pulmonary angioplasty (BPA) had led to changes in the management of chronic thromboembolic pulmonary hypertension (CTEPH). We reviewed data from 98 CTEPH patients diagnosed during the last decade in a pulmonary hypertension (PH) expert centre. The management modalities of 2 periods (Period A: 2011-15 and Period B: 2016-20) were compared. Age (period A: 72 [58-80] years; period B: 69 [62-79] years), clinical (New York Heart Association (NYHA) functional class III-IV: 25/41, 61% vs 39/57, 68%), and hemodynamic assessments (pulmonary vascular resistance: 7.5 [6.2-8.7] WU vs 8.0 [6.0-10.2] WU) at baseline were not significantly different. Pulmonary endarterectomy was performed in less than one third of patients (12, 29.3% vs 15, 26.3%). For patients not eligible for surgery, medical therapy was mostly prescribed alone during period A (medical therapy alone, patients diagnosed in period A: 61% vs in period B: 17.5%) while it was associated with BPA during period B (medical therapy + BPA, 12% vs 61.4%). The 5-year survival rate was excellent for patients who underwent surgery (96.3%) or BPA (95.2%), but was only 42.1% for patients under oral medication only (p < 0.0001). Patients diagnosed with CTEPH who cannot be operated should undergo BPA. The survival rate after BPA is as good as after surgery and significantly better than that of oral medication only., Competing Interests: Declaration of Competing Interest None., (Copyright © 2023 SPLF and Elsevier Masson SAS. All rights reserved.)

Background: Patients treated for pulmonary arterial hypertension (PAH) frequently receive vitamin K antagonists (VKAs) for PAH or validated indications (such as atrial fibrillation or venous thromboembolism). In these latter indications, VKAs are challenged by direct oral anticoagulants (DOAs). Decreased dosage of DOAs has been proposed in patients at risk of bioaccumulation., Objectives: We aimed to evaluate the frequency of bioaccumulation risks in patients treated with PAH-targeted therapy, particularly regarding the presence of validated indications., Methods: We conducted a retrospective study in three different PAH referral centers. All patients receiving PAH-targeted therapy were classified according to demographics, prescription and indications of VKAs, and the presence of major bioaccumulation risk factors (renal failure, low body weight, strong P-glycoprotein or cytochrome P3A4 inhibitors)., Results: Two hundred and thirty-nine of the 366 patients included received VKAs, 94 for validated indications. At least one major risk factor was found in 231 (63.1%) of the whole study population, and in 54 (57.4%) of the patients anticoagulated for a validated indication. No specific patient phenotype could be individualized., Conclusions: About 1 in 2 patients treated with PAH therapy has at least one of the three major risk factors for DOA bioaccumulation. DOAs in the PH setting could be associated with bioaccumulation and should be individualized, mainly in patients with confirmed indication., (© 2016 S. Karger AG, Basel.)

Right ventricle ejection fraction (RVEF) evaluated with magnetic resonance imaging is a strong determinant of patient outcomes in pulmonary arterial hypertension. We evaluated the prognostic value of RVEF assessed with conventional planar equilibrium radionuclide angiography at baseline and change 3-6 months after initiating pulmonary arterial hypertension-specific therapy. In a prospective cohort of newly diagnosed patients with idiopathic, heritable or anorexigen-associated pulmonary arterial hypertension, RVEF was measured at baseline (n=100) and 3-6 months after initiation of therapy (n=78). After a median follow-up of 4.1 years, 41 deaths occurred, including 35 from cardiovascular causes. Patients with a (median) baseline RVEF >25% had better survival than those with a RVEF <25% using Kaplan-Meier analysis (p=0.010). RVEF at baseline was an independent predictor of all-cause and cardiovascular mortality in adjusted Cox regression model (p=0.002 and p=0.007, respectively; HR 0.93 for both). Patients with stable or increased RVEF at 3-6 months had a trend for improved all-cause survival (HR 2.43, p=0.086) and had less cardiovascular mortality (HR 3.25, p=0.034) than those in whom RVEF decreased despite therapy. RVEF assessed with conventional planar equilibrium radionuclide angiography at baseline and change in RVEF 3-6 months after therapy initiation independently predict outcomes in patients with pulmonary arterial hypertension., (Copyright ©ERS 2015.)

Background: Right ventricular function is a strong determinant of prognosis in severe pulmonary hypertension., Methods: The aim of this study was to evaluate the prognostic value of estimates of right ventricular function obtained by echocardiography and Doppler tissue imaging and of functional class and 6-min walk distance (6MWD) in 142 patients with either pulmonary arterial hypertension (n = 104) or chronic thromboembolic pulmonary hypertension (n = 38). Echocardiography was prospectively performed, and demographics, medications, associated medical conditions, New York Heart Association class, and 6MWD at inclusion in addition to vital status, transplantation, and hospital admission related to pulmonary hypertension at follow-up were then collected by review of the medical records., Results: Variables associated with overall survival by univariate analysis were 6MWD (P = .009), functional class (P = .024), tricuspid annular plane systolic excursion (P = .03) and isovolumic peak velocity at the tricuspid annulus (IVCv) (P = .003). On multivariate analysis, IVCv (P = .015) and 6MWD (P = .016) were the only independent predictors of survival. Kaplan-Meier estimates of survival at 1 year were 95% in patients with IVCv > 9 cm/sec and 80% in those with IVCv ≤ 9 cm/sec (P = .002). Intraobserver and interobserver variability of IVCv measurement were 5% and 9%, respectively., Conclusions: Measurement of right ventricular function by Doppler tissue imaging, an easy, noninvasive, and reproducible method, is an independent predictor of clinical outcomes in patients with severe pulmonary hypertension., (Copyright © 2013 American Society of Echocardiography. Published by Mosby, Inc. All rights reserved.)

Objective: To identify conditions associated with severe carbon monoxide transfer coefficient reduction (Kco < 40% of predicted values)., Patients and Methods: We retrospectively reviewed pulmonary function tests, such as Kco measurement, in consecutive patients evaluated in the physiology unit of a University hospital over a 6-year period. Patients were included if they had at least 1 measure of severe Kco reduction, with further detailed pulmonary function tests, clinical data, and diagnostic procedures conducted within 6 months., Results: 5576 patients underwent 9061 Kco measurements. 195 patients (3.5%) with Kco < 40% were investigated (156 males; mean age 62 ± 12 years). Mean Kco was 29 ± 9% of predicted values. The main conditions associated with severe Kco reduction were: emphysema (46%); interstitial lung disease (28%); combined pulmonary fibrosis and emphysema (16%); and pre-capillary pulmonary hypertension (8%). Systolic pulmonary artery pressure was ≥ 35 mmHg at echocardiography in 88% of patients. Right heart catheterization performed in 97 patients showed pre-capillary pulmonary hypertension in 86 of 195 patients (44%). Pulmonary hypertension was the most frequent condition associated with Kco severe reduction. Pulmonary hypertension was present in 29%, 53%, and 48% of patients with chronic obstructive pulmonary disease, interstitial lung disease, and combined pulmonary fibrosis and emphysema, respectively, and was disproportionate to the parenchymal lung disease (mean pulmonary artery pressure > 35 mmHg) in 63% of cases., Conclusion: Severe Kco reduction is frequently related to pulmonary hypertension, especially when associated with emphysema and/or interstitial lung disease. Systematic echocardiography is thus warranted in any patient with severe Kco diminution., (Copyright © 2011 Elsevier Ltd. All rights reserved.)

Background: Hypoxemia is common in pulmonary hypertension (PH) and may be partly related to ventilation/perfusion mismatch, low diffusion capacity, low cardiac output, and/or right-to-left (RL) shunting., Methods: To determine whether true RL shunting causing hypoxemia is caused by intracardiac shunting, as classically considered, a retrospective single center study was conducted in consecutive patients with precapillary PH, with hypoxemia at rest (PaO2 < 10 kPa), shunt fraction (Qs/Qt) greater than 5%, elevated alveolar-arterial difference of PO2 (AaPO2), and with transthoracic contrast echocardiography performed within 3 months., Results: Among 263 patients with precapillary PH, 34 patients were included: pulmonary arterial hypertension, 21%; PH associated with lung disease, 47% (chronic obstructive pulmonary disease, 23%; interstitial lung disease, 9%; other, 15%); chronic thromboembolic PH, 26%; miscellaneous causes, 6%. Mean pulmonary artery pressure, cardiac index, and pulmonary vascular resistance were 45.8 +/- 10.8 mmHg, 2.2 +/- 0.6 L/min/m2, and 469 +/- 275 dyn.s.cm-5, respectively. PaO2 in room air was 6.8 +/- 1.3 kPa. Qs/Qt was 10.2 +/- 4.2%. AaPO2 under 100% oxygen was 32.5 +/- 12.4 kPa. Positive contrast was present at transthoracic contrast echocardiography in 6/34 (18%) of patients, including only 4/34 (12%) with intracardiac RL shunting. Qs/Qt did not correlate with hemodynamic parameters. Patients' characteristics did not differ according to the result of contrast echocardiography., Conclusion: When present in patients with precapillary PH, RL shunting is usually not related to reopening of patent foramen ovale, whatever the etiology of PH.

Background: Lung transplantation (LT) may represent a therapeutic option in case of advanced pulmonary Langerhans' cell histiocytosis (PLCH). Little is known however about the characteristics of the patients considered for LT or its results., Methods: We conducted a retrospective multicenter study by questionnaire on 39 patients who underwent LT for end-stage PLCH at seven centers in France., Results: Of the 39 patients, 15 received single lung transplantation, 15 double lung transplantation and 9 heart-lung transplantation. At evaluation, extrapulmonary involvement was present in 31% of the patients, pulmonary hypertension (PAPm>25 mm Hg) was observed in 92% of cases and was moderate-to-severe (PAPm> or =35 mm Hg) in 72.5%. The survival was 76.9% at 1 year, 63.6% at 2 years, 57.2% at 5 years, and 53.7% at 10 years. Recurrence of the disease occurred in eight cases (20.5%) with no impact on the survival rate. The sole risk factor for recurrence of the disease was the presence of preoperative extrapulmonary involvement., Conclusion: Severe pulmonary hypertension is a common feature in patients with end-stage PLCH. Given the good postransplant survival rate and despite a recurrence rate of the disease of approximately 20% after LT, we conclude that LT is a therapeutic option in this setting.