scholarly journals Current and Emerging Biomarkers for Pulmonary Hypertension

2018 ◽  
Vol 16 (3) ◽  
pp. 136-140 ◽  
Author(s):  
Anna R. Hemnes
Keyword(s):  
Pulmonary Hypertension ◽  
Cancer Biology ◽  
The Body ◽  
National Institutes Of Health ◽  
Pulmonary Vascular Disease ◽  
Future Directions ◽  
Predictors Of Mortality ◽  
Pulmonary Hemodynamic ◽  
Hemodynamic Assessment ◽  
Pulmonary Arterial

“A biological molecule found in blood, other body fluids, or tissues that is a sign of a normal or abnormal process, or of a condition or disease. A biomarker may be used to see how well the body responds to a treatment for a disease or condition. Also called molecular marker or signature molecule” – Biomarker definition, National Institutes of Health, National Cancer Institute, Dictionary of Cancer Terms Although this definition pertains to the field of cancer and cancer biology, it applies to many disciplines, including the field of pulmonary vascular disease. In the world of pulmonary hypertension (PH), biomarkers hold a special place. In the diagnosis of PH subtype, we are limited by the risk of lung biopsy1 and, further, in patients with pulmonary arterial hypertension (PAH) we are limited by the inability to frequently obtain our strongest predictors of mortality: invasive pulmonary hemodynamic assessment. Thus, biomarkers hold much promise for the field of PH. We are searching for markers of PH subtypes and endophenotypes. We are looking for predictors of mortality in all forms of PH and, critically, we are hoping to find peripheral blood markers that will help us discover which drugs are likely to benefit a particular patient. Although we have made inroads in all 3 areas, there are substantial opportunities for refinement of our current biomarkers and discovery of novel markers to improve the care of PH patients. This review will cover the state of current biomarkers in PH and discuss challenges and future directions.

Non-invasive algorithms for the diagnosis of pulmonary hypertension

2012 ◽  
Vol 108 (12) ◽  
pp. 1037-1041 ◽  
Author(s):  
Paul Wexberg ◽  
Harald Heinzl ◽  
Irene Lang ◽  
Diana Bonderman
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Vascular Disease ◽  
Multiple Parameters ◽  
Non Invasive ◽  
Mean Pulmonary Arterial Pressure ◽  
Pulmonary Capillary ◽  
Vulnerable Patients ◽  
Hemodynamic Assessment ◽  
Unnecessary Procedures ◽  
Pulmonary Arterial

SummaryPrecapillary pulmonary hypertension (PH) is diagnosed when mean pulmonary arterial pressure (mPAP) equals or exceeds 25 mmHg and the pulmonary capillary wedge pressure (PCWP) is equal or lower than 15 mmHg. Because both parameters can only be derived from invasive hemodynamic assessment, right heart catheter (RHC) is still a gold standard for the diagnosis of PH. Severe precapillary PH corresponds to pulmonary vascular disease and carries a poor prognosis. Unfortunately, due to a generally low specificity of non-invasive estimates of systolic pulmonary pressure, at least 50% of patients with suspicion of PH need to undergo invasive RHC for exclusion of precapillary PH. Therefore, and also in order to manage the growing number of postcapillary PH due to heart and lung disease in the general population, pulmonary and cardiologic diagnostic algorithms combining multiple parameters have been developed. Recent disease scores are reviewed, and an outlook is given on emerging evidence from the DETECT clinical study holding the promise to non-invasively predict precapillary PH in vulnerable patients. These diagnostic trees help limit unnecessary procedures and help differentiate the current categories of PH. However, one has to keep in mind that the diagnosis of PH is still made by hemodynamic assessment.

scholarly journals Heart and lung transplantation in pulmonary arterial hypertension related to congenital heart disease: an unusual indication

2020 ◽  
Vol 4 (S1) ◽  
Author(s):  
Rosaria Barracano ◽  
Heba Nashat ◽  
Andrew Constantine ◽  
Konstantinos Dimopoulos
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Lung Transplantation ◽  
Atrial Septal Defect ◽  
Septal Defect ◽  
Pulmonary Vascular Disease ◽  
Eisenmenger Syndrome ◽  
Pulmonary Arterial ◽  
Recurrent Haemoptysis

Abstract Background Eisenmenger syndrome is a multisystem disorder, characterised by a significant cardiac defect, severe pulmonary hypertension and long-standing cyanosis. Despite the availability of pulmonary hypertension therapies and improved supportive care in specialist centres, Eisenmenger patients are still faced with significant morbidity and mortality. Case presentation We describe the case of a 44-year-old woman with Eisenmenger syndrome secondary to a large secundum atrial septal defect. Her pulmonary vascular disease was treated with pulmonary vasodilators, but she experienced a progressive decline in exercise tolerance, increasing atrial arrhythmias, resulting in referral for transplantation. Her condition was complicated by significant recurrent haemoptysis in the context of extremely dilated pulmonary arteries and in-situ thrombosis, which prompted successful heart and lung transplantation. She made a slow recovery but remains well 3 years post-transplant. Conclusions Patients with Eisenmenger syndrome secondary to a pre-tricuspid lesion, such as an atrial septal defect have a natural history that differs to patients with post-tricuspid shunts; the disease tends to present later in life but is more aggressive, prompting early and aggressive medical intervention with pulmonary arterial hypertension therapies. This case illustrates that severe recurrent haemoptysis can be an indication for expediting transplantation in Eisenmenger syndrome patients.

scholarly journals EXPRESS: What’s New in Pulmonary Hypertension Clinical Research: Lessons from the Best Abstracts at the 2020 American Thoracic Society International Conference

2021 ◽  
pp. 204589402110407
Author(s):  
Andrew J Sweatt ◽  
Raju Reddy ◽  
Farbod Rahaghi ◽  
Nadine Al-Naamani
Keyword(s):  
Pulmonary Hypertension ◽  
Clinical Research ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
American Thoracic Society ◽  
Future Directions ◽  
International Conference ◽  
Metabolic Dysregulation ◽  
Current State ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial

In this conference paper, we review the 2020 American Thoracic Society (ATS) International Conference session titled, “What’s New in Pulmonary Hypertension Clinical Research: Lessons from the Best Abstracts”. This virtual mini-symposium took place on October 21, 2020, in lieu of the annual in-person ATS International Conference which was cancelled due to the COVID-19 pandemic. Seven clinical research abstracts were selected for presentation in the session, which encompassed five major themes: (1) standardizing diagnosis and management of pulmonary hypertension, (2) improving risk assessment in pulmonary arterial hypertension, (3) evaluating biomarkers of disease activity, (4) understanding metabolic dysregulation across the spectrum of pulmonary hypertension, and (5) advancing knowledge in chronic thromboembolic pulmonary hypertension. Focusing on these five thematic contexts we review the current state of knowledge, summarize presented research abstracts, appraise their significance and limitations, and then discuss relevant future directions in pulmonary hypertension clinical research.

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.

Shear stress paradigm for perinatal fractal arterial network remodeling in lambs with pulmonary hypertension and increased pulmonary blood flow

2007 ◽  
Vol 292 (6) ◽  
pp. H3006-H3018 ◽  
Author(s):  
Zahra Ghorishi ◽  
Jay M. Milstein ◽  
Francis R. Poulain ◽  
Anita Moon-Grady ◽  
Theresa Tacy ◽  
...  
Keyword(s):  
Blood Flow ◽  
Pulmonary Hypertension ◽  
Shear Stress ◽  
High Resistance ◽  
Vascular Reactivity ◽  
Fractal Model ◽  
Pulmonary Hemodynamic ◽  
Pulmonary Arterial ◽  
Hemodynamic Reactivity ◽  
And Control

Congenital heart disease with increased blood flow commonly leads to the development of increased pulmonary vascular reactivity and pulmonary arterial hypertension by mechanisms that remain unclear. We hypothesized a shear stress paradigm of hemodynamic reactivity and network remodeling via the persistence and/or exacerbation of a fetal diameter bifurcation phenotype [parent diameter d0and daughters d1≥ d2with α < 2 in ( d1/ d0)α+ ( d2/ d0)αand area ratio β < 1 in β = ( d12+ d22)/ d02] that mechanically acts as a high resistance magnifier/shear stress amplifier to blood flow. Evidence of a hemodynamic influence on network remodeling was assessed with a lamb model of high-flow-induced secondary pulmonary hypertension in which an aortopulmonary graft was surgically placed in one twin in utero (Shunt twin) but not in the other (Control twin). Eight weeks after birth arterial casts were made of the left pulmonary arterial circulation. Bifurcation diameter measurements down to 0.010 mm in the Shunt and Control twins were then compared with those of an unoperated fetal cast. Network organization, cumulative resistance, and pressure/shear stress distributions were evaluated via a fractal model whose dimension D0≈ α delineates hemodynamic reactivity. Fetus and Control twin D0differed: fetus D0= 1.72, a high-resistance/shear stress amplifying condition; control twin D0= 2.02, an area-preserving transport configuration. The Shunt twin ( D0= 1.72) maintained a fetal design but paradoxically remodeled diameter geometry to decrease cumulative resistance relative to the Control twin. Our results indicate that fetal/neonatal pulmonary hemodynamic reactivity remodels in response to shear stress, but the response to elevated blood flow and pulmonary hypertension involves the persistence and exacerbation of a fetal diameter bifurcation phenotype that facilitates endothelial dysfunction/injury.

scholarly journals Update From the NHLBI on Lung Vascular and Pulmonary Hypertension Research

2010 ◽  
Vol 9 (3) ◽  
pp. 152-155
Author(s):  
Timothy M. Moore ◽  
Dorothy B. Gail ◽  
James P. Kiley
Keyword(s):  
Pulmonary Hypertension ◽  
Molecular Mechanisms ◽  
Therapeutic Potential ◽  
National Institutes Of Health ◽  
Advocacy Organizations ◽  
Public And Private ◽  
Clinical Prognosis ◽  
Blood Institute ◽  
National Heart Lung ◽  
Pulmonary Arterial

Over the past decade, significant advances in treating pulmonary arterial hypertension (PAH) patients have occurred. These advances have come as a result of collective efforts by physicians, scientists, patient advocacy organizations, pharmaceutical companies, and public and private grants-awarding agencies. The National Institutes of Health (NIH) registry was instrumental in characterizing the devastating nature of this disease1 and National Heart, Lung, and Blood Institute (NHLBI)-supported research on the cellular and molecular mechanisms underlying basic pulmonary vascular tone regulation and abnormal vasoconstriction have been pivotal in identifying the therapeutic potential of the PAH drugs now in clinical practice. With the new therapies, the prognosis of PAH is improving and predictors of poor clinical prognosis have become better appreciated. However, better disease phenotyping, elucidating pathogenesis, and decreasing morbidity and mortality remain as research goals. The limitations of current therapeutic options necessitate that lung vascular and pulmonary hypertension research remains a high priority for the NHLBI.

Assisting Pulmonary Hypertension Patients and Families With Treatment Decisions

2010 ◽  
Vol 8 (4) ◽  
pp. 239-243
Author(s):  
Karen Frutiger ◽  
Martha Kingman ◽  
Abby Poms ◽  
Glenna Traiger
Keyword(s):  
North Carolina ◽  
Pulmonary Hypertension ◽  
Los Angeles ◽  
Medical Center ◽  
Pulmonary Vascular Disease ◽  
University Of Texas ◽  
Program Manager ◽  
Nurse Coordinator ◽  
Duke University ◽  
Pulmonary Arterial

To complement this issue's theme, “Living With Pulmonary Hypertension,” a discussion on assisting patients with therapy decisions was led by guest editor Glenna Traiger, RN, MSN, Pulmonary Hypertension CNS, University of California, Los Angeles. The panelists included Karen Frutiger, RN, Clinical Nurse Coordinator, University of Rochester Pulmonary Arterial Hypertension Program, Rochester, NY; Martha Kingman, Nurse Practitioner, University of Texas Southwestern Medical Center, Dallas; and Abby Poms, RRT, Duke University Pulmonary Vascular Disease Program Manager, Duke University Medical Center, Durham, North Carolina.

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