Abstract 14287: Iron Deficiency is Associated With More Severe Pulmonary Vascular Disease in Pulmonary Hypertension Due to Chronic Lung Disease

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Jasmine Tatah ◽  
Marc R Pritzker ◽  
Thenappan Thenappan ◽  
Kurt W Prins
Keyword(s):  
Pulmonary Hypertension ◽  
Iron Deficiency ◽  
Lung Disease ◽  
Vascular Disease ◽  
Chronic Lung Disease ◽  
Pulmonary Vascular Disease ◽  
Iron Deficient ◽  
Pulmonary Arterial ◽  
Group 3 ◽  
Rv Function

Background: Iron deficiency promotes pulmonary vascular remodeling in pre-clinical models, and is associated with worse outcomes in pulmonary arterial hypertension. However, the consequences of iron deficiency in patients with pulmonary hypertension due to chronic lung disease (Group 3 PH) are unexplored. Methods: We studied 122 consecutive Group 3 PH patients from the University of Minnesota Pulmonary Hypertension Repository. Serum soluble transferrin receptor (sTR) levels quantified iron deficiency. We evaluated the relationship between iron deficiency and pulmonary vascular disease, right ventricular (RV) function, exercise capacity, and survival. Results: The iron deficient group (<4.8mg/L sTR) had significantly higher mean pulmonary arterial pressure (40±9 mmHg, n=59 vs. 44±13 mmHg, n=61; p =.02) and lower pulmonary arterial compliance (2.2±1.2 mL/mmHg, n=52 vs. 1.7±0.8 mL/mmHg, n=55; p =.01), but there was no difference in pulmonary vascular resistance. Moreover, there were trends for higher right atrial pressure (7±4 mmHg, n=58 vs. 9±6 mmHg, n=61; p =0.08) in iron deficient patients. However, iron deficiency did not significantly alter RV function by echocardiography, 6-minute walk distance, or survival. Conclusions: Iron deficiency in Group 3 PH is associated with worse pulmonary vascular disease. This suggests iron deficiency could contribute to pulmonary vascular disease in Group 3 PH, and future studies are needed to determine if iron replacement could be a therapy for this deadly type of PH.

scholarly journals Lower DLco% identifies exercise pulmonary hypertension in patients with parenchymal lung disease referred for dyspnea

2020 ◽  
Vol 10 (1) ◽  
pp. 204589401989191 ◽  
Author(s):  
Richard H. Zou ◽  
William D. Wallace ◽  
S. Mehdi Nouraie ◽  
Stephen Y. Chan ◽  
Michael G. Risbano
Keyword(s):  
Pulmonary Hypertension ◽  
Lung Disease ◽  
Vascular Disease ◽  
Pulmonary Function Tests ◽  
Pulmonary Vascular Disease ◽  
Chronic Obstructive ◽  
Exertional Dyspnea ◽  
Pulmonary Arterial ◽  
Parenchymal Lung Disease ◽  
Parenchymal Lung

Exercise pulmonary hypertension is an underappreciated form of physical limitation related to early pulmonary vascular disease. A low diffusing capacity of lungs for carbon monoxide (DLco) can be seen in patients with resting pulmonary hypertension as well as parenchymal lung disease. It remains unclear whether low DLco% identifies early pulmonary vascular disease. We hypothesize that a reduced DLco% differentiates the presence of exercise pulmonary hypertension in patients with parenchymal lung disease. Fifty-six patients referred for unexplained exertional dyspnea with pulmonary function tests within six months of hemodynamic testing underwent exercise right heart catheterization. Exclusion criteria included resting pulmonary arterial or venous hypertension. Receiver operator characteristic curve determined the optimal DLco% cutoffs based on the presence or absence of parenchymal lung disease. Twenty-one (37%) patients had parenchymal lung disease, most common manifesting as chronic obstructive lung disease or interstitial lung disease. In patients with parenchymal lung disease, a DLco of 46% demonstrated 100% sensitivity and 73% specificity for detecting exercise pulmonary hypertension. In patients without parenchymal lung disease, a DLco of 73% demonstrated 58% sensitivity and 94% specificity for detecting exercise pulmonary hypertension. In both cohorts, DLco% below the optimum cutoffs were associated with higher peak mean pulmonary arterial pressure and peak total pulmonary resistance consistent with the hemodynamic definition of exercise pulmonary hypertension. Patients with a DLco < 46% were more often treated with pulmonary vasodilators and had a trend to higher mortality and lung transplant. DLco% is a simple non-invasive screening test for the presence of exercise pulmonary hypertension in our mixed referral population with progressive exertional dyspnea. DLco < 46% with parenchymal lung disease and DLco < 73% without parenchymal lung disease may play a role in differentiating the presence of pulmonary vascular disease prior to invasive hemodynamic testing.

scholarly journals The Challenge to Decide between Pulmonary Hypertension Due to Chronic Lung Disease and PAH with Chronic Lung Disease

Diagnostics ◽  
2021 ◽  
Vol 11 (2) ◽  
pp. 311
Author(s):  
Horst Olschewski
Keyword(s):  
Pulmonary Hypertension ◽  
Lung Disease ◽  
Chronic Lung Disease ◽  
Diagnostic Procedures ◽  
Disease Group ◽  
Group 4 ◽  
Pulmonary Arterial ◽  
Group 3 ◽  
Group 2 ◽  
Group 1

Chronic lung diseases are strongly associated with pulmonary hypertension (PH), and even mildly elevated pulmonary arterial pressures are associated with increased mortality. Chronic obstructive pulmonary disease (COPD) is the most common chronic lung disease, but few of these patients develop severe PH. Not all these pulmonary pressure elevations are due to COPD, although patients with severe PH due to COPD may represent the largest subgroup within patients with COPD and severe PH. There are also patients with left heart disease (group 2), chronic thromboembolic disease (group 4, CTEPH) and pulmonary arterial hypertension (group 1, PAH) who suffer from COPD or another chronic lung disease as co-morbidity. Because therapeutic consequences very much depend on the cause of pulmonary hypertension, it is important to complete the diagnostic procedures and to decide on the main cause of PH before any decision on PAH drugs is made. The World Symposia on Pulmonary Hypertension (WSPH) have provided guidance for these important decisions. Group 2 PH or complex developmental diseases with elevated postcapillary pressures are relatively easy to identify by means of elevated pulmonary arterial wedge pressures. Group 4 PH can be identified or excluded by perfusion lung scans in combination with chest CT. Group 1 PAH and Group 3 PH, although having quite different disease profiles, may be difficult to discern sometimes. The sixth WSPH suggests that severe pulmonary hypertension in combination with mild impairment in the pulmonary function test (FEV1 > 60 and FVC > 60%), mild parenchymal abnormalities in the high-resolution CT of the chest, and circulatory limitation in the cardiopulmonary exercise test speak in favor of Group 1 PAH. These patients are candidates for PAH therapy. If the patient suffers from group 3 PH, the only possible indication for PAH therapy is severe pulmonary hypertension (mPAP ≥ 35 mmHg or mPAP between 25 and 35 mmHg together with very low cardiac index (CI) < 2.0 L/min/m2), which can only be derived invasively. Right heart catheter investigation has been established nearly 100 years ago, but there are many important details to consider when reading pulmonary pressures in spontaneously breathing patients with severe lung disease. It is important that such diagnostic procedures and the therapeutic decisions are made in expert centers for both pulmonary hypertension and chronic lung disease.

scholarly journals THE DIFFICULTIES IN THE DIAGNOSIS OF PULMONARY HYPERTENSION ASSOCIATED WITH CHRONIC LUNG DISEASE

2020 ◽  
Vol 73 (9) ◽  
pp. 1853-1860
Author(s):  
Sylwia Łukasik ◽  
Dariusz Łukasik ◽  
Michał Tomaszewski ◽  
Weronika Topyła ◽  
Agnieszka Wojtowska ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Lung Disease ◽  
Chronic Lung Disease ◽  
Lung Diseases ◽  
Clinical Symptoms ◽  
Vascular Pathology ◽  
Heart Catheterization ◽  
Chronic Lung Diseases ◽  
Pulmonary Arterial ◽  
Group 3

Introduction: Chronic lung disease (WHO group 3) is the second leading cause of pulmonary hypertension (PH). In turn, the development of PH influences the course of lung disease, worsening the clinical symptoms and prognosis. The aim: To analyse the difficulties in the diagnosis of pulmonary hypertension due to chronic lung disease. Review and Discussion: According to recent literature, PH in the course of lung diseases develops as a result of both “parenchymal” and vascular pathology in patients with a genetic predisposition. Prolonged infection (especially viral) may be an additional promoting factor. Elevation of pulmonary arterial pressure (PAP) is usually moderate and correlates with severity of lung disease. In a small minority, PAP may reach that seen in WHO group 1 pulmonary arterial hypertension (PAH). Conclusions: Echocardiography and right heart catheterization are the principal tools for the diagnosis of PH in chronic lung diseases. Unfortunately, current medications for treating PAH have not shown benefit in controlled trials of group 3 PH, hence their routine use is not recommended. Patients with severe group 3 PH should be considered for referral to expert centres or entry into clinical trials.

scholarly journals P1505 Echocardiographic assessment of different pulmonary hypertension groups

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
M Fontes Oliveira ◽  
M Trepa ◽  
R Costa ◽  
A Dias Frias ◽  
I Silveira ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Interventricular Septum ◽  
Pulmonary Vascular Disease ◽  
Eccentricity Index ◽  
Group 4 ◽  
Global Strain ◽  
Group 3 ◽  
Group 2 ◽  
Rv Function ◽  
Group 1

Abstract Introduction Noninvasive echocardiography evaluation of the right ventricle (RV) has been shown to have prognostic value in patients with pulmonary hypertension (PH). Different etiology groups might have different echocardiographic phenotypes. In this study, we aimed to study echocardiographic characterization of the different PH groups and its ability to predict pulmonary vascular disease severity. Methods We collected echocardiographic and right heart catheterization (RHC) data from 97 (75% female, age 65 ± 15 years) consecutive patients referred to an expert tertiary care referral PH centre from 12/2016 to 11/2018. Echocardiographic analysis was performed using Echo-Pac software from GE Healthcare®. Group 3 and 5 were not included in the group comparison analysis due to few patients included. Results Group 2 PH was the most frequent etiology of PH (35), followed by group 1 (26), group 4 (18), group 5 (3) and group 3 (2). The echocardiographic evaluation of this population as a whole showed borderline parameters of RV dysfunction (tricuspid annular plane systolic excursion (TAPSE) 18 ± 4 mm, fractional area change (FAC) 33 ± 10% and S’ tricuspid wave 10 ± 3 cm/sec). Mean RV global strain was -15 ± 5 and RV free wall strain was -17 ± 7. PH group 1 had a significantly lower FAC (26 ± 4%, p = 0.0025), higher eccentricity index (IE) (1.5 ± 0.1, p = 0.01), and more frequently RV outflow tract (RVOT) notching than other groups (62%, p = 0.012). Group 4 presented an intermedium echocardiographic phenotype between group 1 and 2, and showed more abnormal strain values than the other groups. Group 2 had fewer patients in sinus rhythm (atrial fibrillation in 34% of patients, p = 0.02), presented a thicker interventricular septum (11.3 ± 1.8, p = 0.014), a higher FAC (35 ± 3%, p = 0.0025), higher E mitral wave velocity (72 ± 6 cm/s, p &lt; 0.001) and E/E’ ratio (12.7 ± 10.2, p = 0.006), and larger left (45 ± 3 cm3/m3, p &lt; 0.01) and right atria (25 ± 2 cm2, p = 0.03). PH groups 1 and 4 had higher Pulmonary Vascular Resistance (PVR) and Pulmonary Mean Arterial Pressure (PMAP) values than group 2, which significantly correlated with echocardiographic RV function parameters as TAPSE, FAC, RV global strain and IE. In PH group 2, eccentricity index was the only predictor of PVR (β=4.1, p = 0.018). In this population, a left atria volume &lt; 32.7 cm3/m2 (OR 4.25, CI 1.71 - 10.55) and a E/e’ ratio &lt; 12 (OR 4.72, CI 2.05 - 10.87) predicted PECP &lt; 15 mmHg. RV global strain &gt; -17.1 predicted PVR &gt; 3 wood (OR 3.46, CI 1.50 - 8.02) and PMAP &gt; 20 mmHg (OR 4.92, CI 1.67 - 14.51). TAPSE &lt; 18 mm predicted PVR &gt; 3 wood (OR 7.41, CI 2.99 - 18.36, AUC 0.72). Conclusion Different PH groups present mild echocardiographic differences between them. PH group 1 presented with more echocardiographic signs of RV disfunction, and PH group 2 had higher FAC, E/E’ and larger right and left atria. RV function parameters predicted PVR in PH groups 1 and 4, and EI was the only predictor of PVR in PH group 2. Abstract P1505 Figure.

scholarly journals ERS International Congress, Madrid, 2019: highlights from the Pulmonary Vascular Diseases Assembly

2020 ◽  
Vol 6 (4) ◽  
pp. 00304-2020
Author(s):  
Sheila Ramjug ◽  
Jason Weatherald ◽  
Sandeep Sahay ◽  
Johad Khoury ◽  
Vasile Foris ◽  
...  
Keyword(s):  
Pulmonary Embolism ◽  
Pulmonary Hypertension ◽  
Risk Stratification ◽  
Lung Disease ◽  
Vascular Disease ◽  
Acute Pulmonary Embolism ◽  
Current Knowledge ◽  
International Congress ◽  
Pulmonary Vascular Disease ◽  
Esc Guidelines

The 2019 European Respiratory Society (ERS) International Congress, held in Madrid, Spain, had exciting sessions regarding the field of pulmonary vascular disease. The symposia related to the new ERS/European Society of Cardiology (ESC) Guidelines for the diagnosis and management of acute pulmonary embolism were well received, as were sessions on pulmonary hypertension related to lung disease, demonstrating the concept of pulmonary hypertension not being the rarity that it was previously thought to be. The use of risk stratification in relation to pulmonary arterial hypertension (PAH) was heavily featured and the scientific sessions informing the respiratory community of potential biomarkers and targets for future therapies were thought-provoking.This article discusses highlights of the 2019 pulmonary vascular disease sessions as a summary of current knowledge and practice. We have summarised the key points from the sessions pertaining to the new ERS/ESC Guidelines for the management of acute pulmonary embolism. We have also focused on prognostic factors and potential therapies in pulmonary hypertension related to interstitial lung disease. Relating to PAH, we have reviewed the symposia on risk stratification, along with the use of noninvasive measures and the sessions relating to biomarkers in PAH.

scholarly journals Clinical case of pulmonary hypertension, associated with rare lung disease

2020 ◽  
Vol 27 (1) ◽  
pp. 45-57
Author(s):  
G. D. Radchenko ◽  
C. M. Kushnir ◽  
Yu. M. Sirenko
Keyword(s):  
Pulmonary Hypertension ◽  
Lung Disease ◽  
Clinical Symptoms ◽  
Specific Therapy ◽  
Younger Women ◽  
Clinical Presentations ◽  
Hypertension Diagnosis ◽  
Pulmonary Arterial ◽  
Group 3 ◽  
Hypertension Development

This article is clinical presentation of pulmonary hypertension case, associated with rare lung disease – lymphangioleiomyomatosis. There are elucidated: diagnostic criteria of this disease, its prevalence, pathogenesis, including mechanisms of pulmonary hypertension development, clinical presentations and methods of treatment. Authors concluded some special features of this case: rare disease that could be diagnosed by only experienced staff; late patient’s age is atypical for this disease (usual this disease is diagnosed in younger women (childbirth potential age); moderate lung function disorders were accompanied by severe pulmonary hypertension, that needed additional examination in expert center; in spite of general recommendations not to use specific therapy in patients with lung disease the prostaglandins were used in this patient with improving of clinical symptoms. Based on summary of 6th World Symposium on Pulmonary Hypertension (Nice, 2018) there were discussed the particularities of pulmonary hypertension diagnosis and treatment in patients with lung disease or chronic hypoxia (group 3) and their differences with pulmonary arterial hypertension (group 1). It was stressed the necessity of individual approaches in specific therapy using in patients with lung diseases and high pulmonary artery pressure and providing the clinical trials for evaluation of this therapy influence on prognosis.

scholarly journals Optimization of combined measures of airway physiology and cardiovascular hemodynamics in mice

2020 ◽  
Vol 10 (1) ◽  
pp. 204589402091293 ◽  
Author(s):  
Katrina W. Kopf ◽  
Julie W. Harral ◽  
Emily A. Staker ◽  
Megan E. Summers ◽  
Irina Petrache ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Lung Disease ◽  
Chronic Lung Disease ◽  
Chronic Obstructive ◽  
Murine Models ◽  
Comprehensive Overview ◽  
Cardiovascular Hemodynamics ◽  
Chronic Lung Diseases ◽  
Group 3 ◽  
Airway Physiology

Pulmonary hypertension may arise as a complication of chronic lung disease typically associated with tissue hypoxia, as well as infectious agents or injury eliciting a type 2 immune response. The onset of pulmonary hypertension in this setting (classified as Group 3) often complicates treatment and worsens prognosis of chronic lung disease. Chronic lung diseases such as chronic obstructive lung disease (COPD), emphysema, and interstitial lung fibrosis impair airflow and alter lung elastance in addition to affecting pulmonary vascular hemodynamics that may culminate in right ventricle dysfunction. To date, functional endpoints in murine models of chronic lung disease have typically been limited to separately measuring airway and lung parenchyma physiology. These approaches may be lengthy and require a large number of animals per experiment. Here, we provide a detailed protocol for combined assessment of airway physiology with cardiovascular hemodynamics in mice. Ultimately, a comprehensive overview of pulmonary function in murine models of injury and disease will facilitate the integration of studies of the airway and vascular biology necessary to understand underlying pathophysiology of Group 3 pulmonary hypertension.

scholarly journals Pulmonary hypertension in systemic sclerosis: different phenotypes

2017 ◽  
Vol 26 (145) ◽  
pp. 170056 ◽  
Author(s):  
David Launay ◽  
Vincent Sobanski ◽  
Eric Hachulla ◽  
Marc Humbert
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Lung Disease ◽  
Pulmonary Arteries ◽  
Left Ventricular ◽  
High Prevalence ◽  
Pulmonary Arterial ◽  
Group 3 ◽  
Group 2 ◽  
Group 1

Pulmonary hypertension (PH) is a frequent and severe complication of systemic sclerosis (SSc). PH in SSc is highly heterogeneous because of the various clinical phenotypes of SSc itself and because the mechanisms of PH can vary from one patient to another. PH in SSc may be due to vasculopathy of the small pulmonary arteries (group 1; pulmonary arterial hypertension), interstitial lung disease (group 3; PH due to lung disease or chronic hypoxia) or myocardial fibrosis leading to left ventricular systolic or diastolic dysfunction (group 2; PH due to chronic left-heart disease). Pulmonary veno-occlusive disease is not uncommon in SSc and may also cause PH in some patients (group 1′). There is a high prevalence of each of these conditions in SSc and, as such, it may be difficult to determine the dominant cause of PH in a particular patient. However, careful phenotyping of PH in SSc is important as the therapy required for each of these underlying conditions is very different. In this review, we will decipher the different phenotypes of SSc-PH.

scholarly journals Adiponectin deficiency: a model of pulmonary hypertension associated with pulmonary vascular disease

2009 ◽  
Vol 297 (3) ◽  
pp. L432-L438 ◽  
Author(s):  
Ross Summer ◽  
Christopher A. Fiack ◽  
Yasumasa Ikeda ◽  
Kaori Sato ◽  
Daniel Dwyer ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Vascular Disease ◽  
Pulmonary Vascular Disease ◽  
Targeted Deletion ◽  
Vascular Homeostasis ◽  
Vascular Phenotype ◽  
Luminal Side ◽  
Pulmonary Arterial ◽  
High Concentrations

Adiponectin (APN) is an adipocyte-derived factor that exists at high concentrations in serum and has anti-inflammatory and systemic vascular-protective properties. In this study, we investigated the role of APN in pulmonary vascular homeostasis. We found that APN localizes to the luminal side of blood vessels in lung and acts in vitro to block TNF-α-induced E-selectin upregulation in pulmonary artery endothelial cells. Targeted deletion of the APN gene in mice leads to a vascular phenotype in lung characterized by E-selectin upregulation and age-dependent increases in perivascular inflammatory cell infiltration and pulmonary arterial pressures. Taken together, these findings demonstrate an important role for APN in lung vascular homeostasis and suggest that APN-deficient states may contribute to the pathogenesis of inflammatory pulmonary vascular disease and to the development of pulmonary hypertension.

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