scholarly journals Pulmonary Hypertension in Idiopathic Pulmonary Fibrosis

2013 ◽  
Vol 12 (3) ◽  
pp. 127-134 ◽  
Author(s):  
Belinda N. Rivera-Lebron
Keyword(s):  
Pulmonary Hypertension ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Fold Increase ◽  
Right Heart Catheterization ◽  
Noninvasive Testing ◽  
Heart Catheterization ◽  
Risk Of Death ◽  
Hypoxic Vasoconstriction ◽  
Hemodynamic Assessment

Idiopathic pulmonary fibrosis (IPF) is a fatal lung disease with a variable natural history. Pulmonary hypertension (PH) is frequently found in patients with IPF and is associated with an almost 3-fold increase in the risk of death. Pulmonary hypoxic vasoconstriction plays an important role in the pathogenesis of PH in IPF (PH-IPF), although it has become clear that it is not the only mechanism involved. While invasive right heart catheterization is the gold standard modality of hemodynamic assessment, there has been increasing interest in noninvasive testing, such as Doppler echocardiogram, as complementary methods of assessing right ventricular function in these patients. While the expanding array of pharmacologic options for the treatment of pulmonary arterial hypertension has engendered increased interest in the application of these therapies for PH-IPF, supportive evidence for benefit is lacking.

scholarly journals Right Atrial Pressure During Exercise Predicts Survival in Patients With Pulmonary Hypertension

2020 ◽  
Vol 9 (22) ◽  
Author(s):  
Mona Lichtblau ◽  
Patrick R. Bader ◽  
Stéphanie Saxer ◽  
Charlotte Berlier ◽  
Esther I. Schwarz ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Cardiac Output ◽  
Right Heart Catheterization ◽  
Atrial Pressure ◽  
Right Atrial ◽  
Right Atrial Pressure ◽  
Heart Catheterization ◽  
Right Heart ◽  
Risk Of Death ◽  
Exercise Induced

Background We investigated changes in right atrial pressure (RAP) during exercise and their prognostic significance in patients assessed for pulmonary hypertension (PH). Methods and Results Consecutive right heart catheterization data, including RAP recorded during supine, stepwise cycle exercise in 270 patients evaluated for PH, were analyzed retrospectively and compared among groups of patients with PH (mean pulmonary artery pressure [mPAP] ≥25 mm Hg), exercise‐induced PH (exPH; resting mPAP <25 mm Hg, exercise mPAP >30 mm Hg, and mPAP/cardiac output >3 Wood Units (WU)), and without PH (noPH). We investigated RAP changes during exercise and survival over a median (quartiles) observation period of 3.7 (2.8–5.6) years. In 152 patients with PH, 58 with exPH, and 60 with noPH, median (quartiles) resting RAP was 8 (6–11), 6 (4–8), and 6 (4–8) mm Hg ( P <0.005 for noPH and exPH versus PH). Corresponding peak changes (95% CI) in RAP during exercise were 5 (4–6), 3 (2–4), and −1 (−2 to 0) mm Hg (noPH versus PH P <0.001, noPH versus exPH P =0.027). RAP increase during exercise correlated with mPAP/cardiac output increase ( r =0.528, P <0.001). The risk of death or lung transplantation was higher in patients with exercise‐induced RAP increase (hazard ratio, 4.24; 95% CI, 1.69–10.64; P =0.002) compared with patients with unaltered or decreasing RAP during exercise. Conclusions In patients evaluated for PH, RAP during exercise should not be assumed as constant. RAP increase during exercise, as observed in exPH and PH, reflects hemodynamic impairment and poor prognosis. Therefore, our data suggest that changes in RAP during exercise right heart catheterization are clinically important indexes of the cardiovascular function.

scholarly journals DIAGNOSTIC AND PREDICTIVE VALUE OF RIGHT HEART CATHETERIZATION-DERIVED MEASUREMENTS IN PULMONARY HYPERTENSION

2021 ◽  
Vol 74 (3) ◽  
pp. 546-553
Author(s):  
Karolina Barańska-Pawełczak ◽  
Celina Wojciechowska ◽  
Wojciech Jacheć
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
18Th Century ◽  
Right Heart Catheterization ◽  
Right Atrial ◽  
Heart Catheterization ◽  
Right Heart ◽  
Risk Of Death ◽  
Therapeutic Decisions ◽  
Wide Range

Right heart catheterization is a unique tool not only in the diagnosis but also in the management of patients with a wide range of cardiovascular diseases. The technique dates back to the 18th century, but the biggest advances were made in the 20th century. This review focuses on pulmonary hypertension for which right heart catheterization remains the diagnostic gold standard. Right heart catheterization-derived parameters help classify pulmonary hypertension into several subgroups, assess risk of adverse events or mortality and make therapeutic decisions. According to the European Society of Cardiology guidelines pulmonary hypertension (PH) is defined as an increase in mean pulmonary artery pressure (PAPm) > 25 mmHg, whereas a distinction between pre- and post-capillary PH is made based on levels of pulmonary artery wedge pressure (PAWP). Moreover, right atrial pressure (RAP), cardiac index (CI) and mixed venous oxygen saturation (SvO2) are the only parameters recommended to assess prognosis and only in patients with pulmonary arterial hypertension (PAH). Patients with RAP > 14 mmHg, CI < 2.0 l/min/m2 and SvO2 < 60% are at high (> 10%) risk of death within the next year. The purpose of this paper is to show that RHC-derived parameters can be used on a considerably larger scale than currently recommended. Several prognostic parameters, with specific thresholds have been identified for each subtype of pulmonary hypertension and can be helpful in everyday practice for treatment of PH.

Prevalence of Pulmonary Hypertension in Systemic Sclerosis in European Caucasians and Metaanalysis of 5 Studies

2010 ◽  
Vol 37 (11) ◽  
pp. 2290-2298 ◽  
Author(s):  
JÉRÔME AVOUAC ◽  
PAOLO AIRÒ ◽  
CHRISTOPHE MEUNE ◽  
LORENZO BERETTA ◽  
PHILIPPE DIEUDE ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Pulmonary Function Tests ◽  
Right Heart Catheterization ◽  
Advanced Age ◽  
Noninvasive Testing ◽  
Heart Catheterization ◽  
Alveolar Volume ◽  
Different Types ◽  
Pulmonary Arterial

Objective.To measure the prevalence of different types of pulmonary hypertension (PH) and to identify patients with systemic sclerosis (SSc) at highest risk in a multicenter European sample, with a metaanalysis of relevant studies.Methods.Consecutive patients with SSc recruited at 11 French and Italian centers underwent detailed evaluations, including Doppler echocardiography, chest computed tomography, pulmonary function tests, and right-heart catheterization (RHC), to detect the presence and causes of PH. A metaanalysis was performed, including data from 4 other studies.Results.Among 206 patients in whom it was suspected, PH was confirmed by RHC in 83 patients (7%). Precapillary PH was found in 64 patients (5%), of whom 42 had pulmonary arterial hypertension (PAH) and 22 had PH secondary to interstitial lung disease (ILD). RHC identified 17 patients (1%) with postcapillary PH secondary to left-heart disease. Patients with DLCO/alveolar volume < 70% were more likely to have precapillary PH (87.5% vs 42%; p < 0.0001). Precapillary and postcapillary PH were associated with advanced age (68 ± 14 vs 59 ± 12 yrs, p < 0.0001, and 74 ± 16 vs 61.5 ± 10 yrs, p < 0.0001, respectively). The metaanalysis of 3818 patients showed a prevalence of precapillary PH of 9% (95% CI 6%–12%) and identified advanced age, longer disease duration, and limited cutaneous disease subset as risk factors for this condition.Conclusion.The prevalence of precapillary PH in our multicenter study of SSc was 5%, and in the metaanalysis 9%. Our observations support use of RHC to confirm the presence of precapillary PH suspected by noninvasive testing. We also identified patients at high risk who should be carefully monitored.

Pulmonary Hypertension in Idiopathic Pulmonary Fibrosis

2015 ◽  
Vol 1 (2) ◽  
pp. 79-86
Author(s):  
Juan Enghelmayer ◽  
Tulio Papucci ◽  
Alejandro Raimondi
Keyword(s):  
Pulmonary Hypertension ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis

scholarly journals The prevalence of pulmonary hypertension assessed using the pulmonary vein‐to‐right pulmonary artery ratio and its association with survival in West Highland white terriers with canine idiopathic pulmonary fibrosis

2021 ◽  
Vol 17 (1) ◽  
Author(s):  
Elodie Roels ◽  
Aline Fastrès ◽  
Anne-Christine Merveille ◽  
Géraldine Bolen ◽  
Erik Teske ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Pulmonary Artery ◽  
Pulmonary Vein ◽  
Survival Data ◽  
Study Objective ◽  
Time Motion ◽  
Co Morbidity ◽  
Right Pulmonary Artery

Abstract Background Pulmonary hypertension (PH) is a known co-morbidity in West Highland white terriers (WHWTs) affected with canine idiopathic pulmonary fibrosis (CIPF). The pulmonary vein-to-right pulmonary artery ratio (PV/PA) has recently been described for the detection of pre-capillary PH in dogs. The objective of the present study was to estimate the prevalence of PH at diagnostic, in WHWTs affected with CIPF, by using PV/PA, in comparison with a group of healthy breed-matched controls (CTRLs). Additional study objective was to explore whether the presence of PH at initial diagnosis of CIPF impacted survival time in dogs treated with sildenafil. Results Twenty-five client-owned WHWTs presented with CIPF and 19 CTRLs were included in the study. PV/PA in either two-dimensional mode (2D) or time-motion mode or both were measured from cineloops in each dog. Dogs were classified according to PV/PA value into non/mild PH (PV/PA measured in 2D ≥ 0.7) or moderate/severe PH (PV/PA < 0.7). Survival data of WHWTs affected with CIPF were extracted from medical record to assess association between presence of PH at diagnosis and outcome. 60 % overall prevalence for moderate/severe PH was estimated in this cohort of WHWTs presented with CIPF vs. 5 % in CTRLS (P = 0.0002). The presence of moderate/severe PH at initial presentation was not associated with survival. Conclusions Results of the present study confirm a high prevalence of PH at diagnosis in WHWTs affected with CIPF and highlight the utility of PV/PA as a non-invasive surrogate for assessment of PH in this population.

Pathology and Pathobiology of Pulmonary Hypertension

2017 ◽  
Vol 38 (05) ◽  
pp. 571-584 ◽  
Author(s):  
Peter Dorfmüller ◽  
Christophe Guignabert
Keyword(s):  
Pulmonary Hypertension ◽  
Vascular Inflammation ◽  
Right Heart Catheterization ◽  
Point Of View ◽  
Heart Catheterization ◽  
Pulmonary Endothelium ◽  
Hemodynamic State ◽  
Pulmonary Arterial ◽  
Genetic And Environmental Factors ◽  
And Migration

Pulmonary hypertension (PH) is a hemodynamic state defined by a mean pulmonary artery pressure ≥ 25 mm Hg during resting right heart catheterization. PH can result from precapillary (arterial) or postcapillary (venous) pathophysiological mechanisms. Interestingly, recent PH pathology has shown that pulmonary arterial or pulmonary venous remodelling are rarely independent phenomena, but frequently occur in combined fashion in lungs from patients suffering from different forms of PH, including pulmonary arterial hypertension (PAH). In PAH, it is now becoming clear that aberrant signals present in vessel wall microenvironment, which is largely orchestrated by dysfunctional pulmonary endothelial cells, are key contributors of the pulmonary vascular remodeling process, fostering proliferation, and survival and migration of resident pulmonary vascular cells such as smooth muscle cells, myofibroblasts, and pericytes. In addition, both genetic and environmental factors are also critical in the development of pulmonary vascular inflammation and chronic impairment of the pulmonary endothelium. This article outlines the current understanding of this disease from the point of view of pathology and pathobiology.

scholarly journals Right Heart Size and Right Ventricular Reserve in Pulmonary Hypertension: Impact on Management and Prognosis

Diagnostics ◽  
2020 ◽  
Vol 10 (12) ◽  
pp. 1110
Author(s):  
Ekkehard Grünig ◽  
Christina A. Eichstaedt ◽  
Rebekka Seeger ◽  
Nicola Benjamin
Keyword(s):  
Pulmonary Hypertension ◽  
Right Ventricular ◽  
Ventricular Function ◽  
Right Ventricular Function ◽  
Right Heart Failure ◽  
Right Heart Catheterization ◽  
Contractile Reserve ◽  
Heart Catheterization ◽  
Right Heart ◽  
Heart Size

Various parameters reflecting right heart size, right ventricular function and capacitance have been shown to be prognostically important in patients with pulmonary hypertension (PH). In the advanced disease, patients suffer from right heart failure, which is a main reason for an impaired prognosis. Right heart size has shown to be associated with right ventricular function and reserve and is correlated with prognosis in patients with PH. Right ventricular reserve, defined as the ability of the ventricle to adjust to exercise or pharmacologic stress, is expressed by various parameters, which may be determined invasively by right heart catheterization or by stress-Doppler-echocardiography as a noninvasive approach. As the term “right ventricular contractile reserve” may be misleading, “right ventricular output reserve” seems desirable as a preferred term of increase in cardiac output during exercise. Both right heart size and right ventricular reserve have been shown to be of prognostic importance and may therefore be useful for risk assessment in patients with pulmonary hypertension. In this article we aim to display different aspects of right heart size and right ventricular reserve and their prognostic role in PH.

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