scholarly journals Idiopathic and Heritable Pulmonary Hypertension in Children: New Insights into Causes, Evaluation, and Treatment

2011 ◽  
Vol 10 (2) ◽  
pp. 104-108 ◽  
Author(s):  
Mary P. Mullen
Keyword(s):  
Pulmonary Hypertension ◽  
Molecular Mechanisms ◽  
Life Experience ◽  
Pulmonary Vascular Disease ◽  
Diagnostic Strategies ◽  
The Past ◽  
History Of ◽  
Pulmonary Arterial ◽  
Hypertension In Children ◽  
Major Progress

Over the past decade, major progress has occurred in the care of children and adults with pulmonary arterial hypertension (PAH). Recent insights into molecular mechanisms implicated in the development of pulmonary vascular disease have led to revised clinical classification and diagnostic strategies.1 Data from multicenter pulmonary hypertension registries have provided increased understanding of the clinical course and natural history of many subtypes of disease.2–4 Additionally, the development of multiple therapies targeting important pathways of disease and the translation of those treatments to pediatrics have led to improved life experience and survival for many children with PAH.5

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: Pulmonary Hypertension Associated With Neurofibromatosis Type 2

2021 ◽  
pp. 204589402110295
Author(s):  
Hirohisa Taniguchi ◽  
Tomoya Takashima ◽  
Ly Tu ◽  
Raphaël Thuillet ◽  
Asuka Furukawa ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Neurofibromatosis Type ◽  
Pulmonary Vascular Remodeling ◽  
Life Threatening ◽  
History Of ◽  
Pulmonary Arterial ◽  
First Time

Although precapillary pulmonary hypertension (PH) is a rare but severe complication of patients with neurofibromatosis type 1 (NF1), its association with NF2 remains unknown. Herein, we report a case of a 44-year-old woman who was initially diagnosed with idiopathic pulmonary arterial hypertension (IPAH) and treated with PAH-specific combination therapy. However, a careful assessment for a relevant family history of the disease and genetic testing reveal that this patient had a mutation in the NF2 gene. Using immunofluorescence and Western blotting, we demonstrated a decrease in endothelial NF2 protein in lungs from IPAH patients compared to control lungs, suggesting a potential role of NF2 in PAH development. To our knowledge, this is the first time that precapillary PH has been described in a patient with NF2. The altered endothelial NF2 expression pattern in PAH lungs should stimulate work to better understand how NF2 is contributing to the pulmonary vascular remodeling associated to these severe life-threatening conditions.

scholarly journals Pathophysiology, incidence, management, and consequences of cardiac arrhythmia in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension

2019 ◽  
Vol 9 (1) ◽  
pp. 204589401983489 ◽  
Author(s):  
Meghan M. Cirulis ◽  
John J. Ryan ◽  
Stephen L. Archer
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Molecular Mechanisms ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Outcome Data ◽  
Current Evidence ◽  
Clinical Aspects ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial

Arrhythmias are increasingly recognized as serious, end-stage complications of pre-capillary pulmonary hypertension, including pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). Although arrhythmias contribute to symptoms, morbidity, in-hospital mortality, and possibly sudden death in PAH/CTEPH, there remains a paucity of epidemiologic, pathophysiologic, and outcome data to guide management of these patients. This review summarizes the most current evidence on the topic: from the molecular mechanisms driving arrhythmia in the hypertrophied or failing right heart, to the clinical aspects of epidemiology, diagnosis, and management.

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.

Pulmonary Hypertension Associated with Connective Tissue Disease

2019 ◽  
Vol 40 (02) ◽  
pp. 173-183 ◽  
Author(s):  
Hossam Fayed ◽  
J. Gerry Coghlan
Keyword(s):  
Rheumatoid Arthritis ◽  
Pulmonary Hypertension ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Best Practice ◽  
Reference Source ◽  
History Of ◽  
Pulmonary Arterial ◽  
Primary Physicians

Pulmonary hypertension (PH) is common in most forms of connective tissue disease (CTD); the prevalent type of PH depends on the particular CTD. Thus, pulmonary arterial hypertension (PAH) is dominantly associated with scleroderma, while postcapillary PH is most common in rheumatoid arthritis and lung disease-associated PH is typically found in myositis and sarcoidosis.Considerable expertise is required to identify, diagnose, and manage CTD-PH, as the primary physicians providing the majority of care for this population, rheumatologists, need a good working knowledge of CTD-PH, its rather subtle presentation, and how to access the necessary investigations to screen for and identify patients with PH. The role of the rheumatologist does not stop at diagnosis; in some conditions such as lupus, optimizing immunosuppression is key to the management of PH, and unlike simple idiopathic PAH, the natural history of CTD-PH is often punctuated by complications of the CTD rather than just events due to progression of PH or therapy-related adverse events.The aim of this article is to provide an overview of all forms of CTD-PH, and to provide an easy reference source on current best practice.

Hypoxia- or PDGF-BB-dependent paxillin tyrosine phosphorylation in pulmonary hypertension is reversed by HIF-1α depletion or imatinib treatment

2014 ◽  
Vol 112 (12) ◽  
pp. 1288-1303 ◽  
Author(s):  
Christine Veith ◽  
Dariusz Zakrzewicz ◽  
Bhola Kumar Dahal ◽  
Zoltán Bálint ◽  
Kirsten Murmann ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Tyrosine Phosphorylation ◽  
Molecular Mechanisms ◽  
Hypoxia Inducible Factor ◽  
Pulmonary Vasculature ◽  
Hypoxic Exposure ◽  
Imatinib Treatment ◽  
Pulmonary Arterial ◽  
Arterial Smooth Muscle Cells ◽  
Pulmonary Arterial Smooth Muscle

SummaryChronic exposure to hypoxia induces a pronounced remodelling of the pulmonary vasculature leading to pulmonary hypertension (PH). The remodelling process also entails increased proliferation and decreased apoptosis of pulmonary arterial smooth muscle cells (PASMC), processes regulated by the cytoskeletal protein paxillin. In this study, we aimed to examine the molecular mechanisms leading to deregulation of paxillin in PH. We detected a time-dependent increase in paxillin tyrosine 31 (Y31) and 118 (Y118) phosphorylation following hypoxic exposure (1 % O2) or platelet-derived growth factor (PDGF)-BB stimulation of primary human PASMC. In addition, both, hypoxia- and PDGF-BB increased the nuclear localisation of phospho-paxillin Y31 as indicated by immunofluorescence staining in human PASMC. Elevated paxillin tyrosine phosphorylation in human PASMC was attenuated by hypoxia-inducible factor (HIF)-1α depletion or by treatment with the PDGF-BB receptor antagonist, imatinib. Moreover, we observed elevated paxillin Y31 and Y118 phosphorylation in the pulmonary vasculature of chronic hypoxic mice (21 days, 10 % O2) which was reversible by imatinib-treatment. PDGF-BB-dependent PASMC proliferation was regulated via the paxillin-Erk1/2-cyclin D1 pathway. In conclusion, we suggest paxillin up-regulation and phosphorylation as an important mechanism of vascular remodelling underlying pulmonary hypertension.Note: Parts of the doctoral thesis of Christine Veith are integrated into this report.

Pediatric Pulmonary Hypertension: A Pharmacotherapeutic Review

2009 ◽  
Vol 22 (2) ◽  
pp. 166-178
Author(s):  
Kimberly A. Pesaturo ◽  
Peter N. Johnson ◽  
E. Zachary Ramsey
Keyword(s):  
Nitric Oxide ◽  
Pulmonary Hypertension ◽  
Cardiac Dysfunction ◽  
Dosage Forms ◽  
New Agents ◽  
The Past ◽  
Endothelin Antagonists ◽  
Pharmacological Data ◽  
Hypertension In Children ◽  
Prostacyclin Analogues

Pulmonary hypertension in children is a disorder associated with increased pulmonary vascular resistance and arterial pressure, decreased cardiac output, and right-sided cardiac dysfunction that is caused by numerous etiologies. Although treatment will vary with underlying cause, pharmacological treatment has historically included inhaled nitric oxide and prostacyclin analogues. Over the past several years new agents have been added to the treatment armamentarium, including phosphodiesterase V inhibitors (eg sildenafil) and endothelin antagonists (eg bosentan). Further, more agents are currently under investigation for pulmonary hypertension in children including immunosuppressives and other endothelin antagonist entities. Limitations to treatment include the availability of appropriate, robust pediatric pharmacological data and constraints with dosage forms.

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.

Sign in / Sign up

Export Citation Format

Share Document