scholarly journals Prescription Patterns for Pulmonary Vasodilators in the Treatment of Pulmonary Hypertension Associated With Chronic Lung Diseases: Insights From a Clinician Survey

2021 ◽  
Vol 8 ◽  
Author(s):  
Christopher A. Thomas ◽  
Justin Lee ◽  
Roberto J. Bernardo ◽  
Ryan J. Anderson ◽  
Vladimir Glinskii ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Targeted Therapy ◽  
Lung Disease ◽  
Lung Diseases ◽  
Online Survey ◽  
Treatment Approach ◽  
Initial Therapy ◽  
Triple Combination Therapy ◽  
Management Guidelines ◽  
Chronic Lung Diseases

Background: Pulmonary hypertension is a complication of chronic lung diseases (PH-CLD) associated with significant morbidity and mortality. Management guidelines for PH-CLD emphasize the treatment of the underlying lung disease, but the role of PH-targeted therapy remains controversial. We hypothesized that treatment approaches for PH-CLD would be variable across physicians depending on the type of CLD and the severity of PH.Methods and Results: Between May and July 2020, we conducted an online survey of PH experts asking for their preferred treatment approach in seven hypothetical cases of PH-CLD of varying severity. We assessed agreement amongst clinicians for initial therapy choice using Fleiss' kappa calculations. Over 90% of respondents agreed that they would treat cases of severe PH in the context of mild lung disease with some form of PH-targeted therapy. For cases of severe PH in the context of severe lung disease, over 70% of respondents agreed to use PH-targeted therapy. For mild PH and mild lung disease cases, <50% of respondents chose to start PH-specific therapy. There was overall poor agreement between respondents in the choice to use mono-, double or triple combination therapy with PH-specific agents in all cases.Conclusion: Although management guidelines discourage the routine use of PH-targeted therapies to treat PH-CLD patients, most physicians choose to treat patients with some form of PH-targeted therapy. The choice of therapy and treatment approach are variable and appear to be influenced by the severity of the PH and the underlying lung disease.

scholarly journals Pulmonary hypertension and chronic lung disease: where are we headed?

2019 ◽  
Vol 28 (153) ◽  
pp. 190065 ◽  
Author(s):  
Davide Elia ◽  
Antonella Caminati ◽  
Maurizio Zompatori ◽  
Roberto Cassandro ◽  
Chiara Lonati ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Lung Disease ◽  
Chronic Lung Disease ◽  
Lung Diseases ◽  
Pathological Process ◽  
Chronic Lung Diseases ◽  
Clinically Significant ◽  
Parenchymal Lung Disease ◽  
Parenchymal Lung

Pulmonary hypertension related to chronic lung disease, mainly represented by COPD and idiopathic pulmonary fibrosis, is associated with a worse outcome when compared with patients only affected by parenchymal lung disease. At present, no therapies are available to reverse or slow down the pathological process of this condition and most of the clinical trials conducted to date have had no clinically significant impact. Nevertheless, the importance of chronic lung diseases is always more widely recognised and, along with its increasing incidence, associated pulmonary hypertension is also expected to be growing in frequency and as a health burden worldwide. Therefore, it is desirable to develop useful and reliable tools to obtain an early diagnosis and to monitor and follow-up this condition, while new insights in the therapeutic approach are explored.

scholarly journals Dominating Cause of Pulmonary Hypertension May Change Over Time—Diagnostic and Therapeutic Considerations in a Patient with Pulmonary Hypertension Due to Rheumatoid Arthritis with Lung Involvement

Diagnostics ◽  
2021 ◽  
Vol 11 (10) ◽  
pp. 1931
Author(s):  
Monika Szturmowicz ◽  
Monika Franczuk ◽  
Małgorzata Ewa Jędrych ◽  
Dorota Wyrostkiewicz ◽  
Karina Oniszh ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Pulmonary Hypertension ◽  
Lung Disease ◽  
Chronic Lung Disease ◽  
Lung Diseases ◽  
Treatment Modalities ◽  
Term Survival ◽  
Change Over Time ◽  
Chronic Lung Diseases ◽  
Over Time

Chronic lung diseases are one of the most frequent causes of pulmonary hypertension (PH). The diagnostic challenge is to differentiate PH due to chronic lung disease from pulmonary arterial hypertension (PAH) with coexisting chronic lung disease. Moreover, the dominating cause of PH may change over time, requiring the implementation of new diagnostic procedures and new treatment modalities. We present a 68-year-old female, initially diagnosed with PH in the course of interstitial lung disease, with restrictive impairment of lung function. Therapy with immunosuppressive drugs resulted in significant clinical, radiological and functional improvement. However, five years later, arthritis symptoms developed, with PH worsening, despite stable lung disease. The patient was diagnosed with PAH in the course of rheumatoid arthritis. The introduction of sildenafil resulted in marked clinical and hemodynamic responses. Long-term survival (eleven years from PH onset and five years from PAH confirmation) has been achieved, and the patient remains in good functional condition. As the differential diagnosis of PH in patients with lung diseases is complex, the cooperation of pulmonologists and cardiologists is mandatory to obtain therapeutic success.

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 Pulmonary Hypertension in Chronic Lung Diseases and/or Hypoxia

10.5772/55681 ◽  
2013 ◽  
Author(s):  
Dimitar Sajkov ◽  
Bliegh Mupunga ◽  
Jeffrey J. ◽  
Nikolai Petrovsky
Keyword(s):  
Pulmonary Hypertension ◽  
Lung Diseases ◽  
Chronic Lung Diseases

824 Case Series on the Use of Volume Assured Pressure Support in Patients with Chronic Pulmonary Disease and Progressive Hypercapnia

SLEEP ◽  
2021 ◽  
Vol 44 (Supplement_2) ◽  
pp. A321-A322
Author(s):  
William LeMaster ◽  
Dale Jun ◽  
Sharon De Cruz ◽  
Michelle Zeidler ◽  
Rajan Saggar
Keyword(s):  
Respiratory Failure ◽  
Lung Disease ◽  
Pulmonary Disease ◽  
Lung Diseases ◽  
Lung Transplant ◽  
Pressure Support ◽  
Case Series ◽  
Chronic Obstructive ◽  
Hypoxemic Respiratory Failure ◽  
Chronic Lung Diseases

Abstract Introduction Chronic hypercapnia results from destruction of lung parenchyma which occurs in chronic lung diseases including interstitial lung disease (ILD), bronchiectasis, and chronic lung transplant rejection. Many patients with these diseases will experience progressive respiratory failure eventually requiring consideration of transplantation or re-transplantation. Due to physiologic changes in sleep including reduction in tidal volume, worsening air tapping, and REM atonia, hypoventilation can be exacerbated during the sleeping hours. We present four patients who were prescribed nocturnal Volume Assured Pressure Support VAPS for their progressive hypercapnia. Report of case(s) Subject 1 is a 72 year old female with severe bronchiectasis and restrictive lung disease due to TB pneumonia at a young age. Subject 2 is a 45 year old male with history of pulmonary cavitation due to extensive TB disease when he was younger. Subject 3 is a 45-year-old woman with rheumatoid arthritis related ILD with associated pulmonary arterial hypertension. Subject 4 is a 74 year old patient with a bilateral lung transplant for IPF complicated by bronchiolitis obliterans syndrome who presented with progressive dyspnea and hypercapnia. Despite optimal therapy, all of these patients were admitted for hypercapnic and hypoxemic respiratory failure requiring treatment with BPAP then transitioned to nocturnal VAPS on discharge. For all patients, dyspnea and pCO2 improved as outpatients although all patients did eventually experience an exacerbation of their lung disease requiring repeat admission. Conclusion Due to the physiologic changes that occur with sleep, patients with severe lung disease may experience worsening CO2 retention while sleeping. There is little data assessing the use of chronic nocturnal non-invasive ventilation (NIV) to treat the hypercapnia of chronic lung diseases other than chronic obstructive pulmonary disease, extra-thoracic restriction, and neuromuscular disease. In this case series, nocturnal VAPS stabilized and/or reduced pCO2 in patients with pulmonary parenchymal disease of various etiologies. Additional studies are needed to assess long term effects of VAPS in these patients, including exacerbations, symptoms, and overall mortality. Support (if any):

scholarly journals Macrophage bone morphogenic protein receptor 2 depletion in idiopathic pulmonary fibrosis and Group III pulmonary hypertension

2016 ◽  
Vol 311 (2) ◽  
pp. L238-L254 ◽  
Author(s):  
Ning-Yuan Chen ◽  
Scott D. Collum ◽  
Fayong Luo ◽  
Tingting Weng ◽  
Thuy-Trahn Le ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Diseases ◽  
Vascular Lesions ◽  
Unknown Etiology ◽  
Group Iii ◽  
Chronic Lung Diseases ◽  
Protein Receptor ◽  
Stat3 Phosphorylation

Idiopathic pulmonary fibrosis (IPF) is a lethal lung disease of unknown etiology. The development of pulmonary hypertension (PH) is considered the single most significant predictor of mortality in patients with chronic lung diseases. The processes that govern the progression and development of fibroproliferative and vascular lesions in IPF are not fully understood. Using human lung explant samples from patients with IPF with or without a diagnosis of PH as well as normal control tissue, we report reduced BMPR2 expression in patients with IPF or IPF+PH. These changes were consistent with dampened P-SMAD 1/5/8 and elevated P-SMAD 2/3, demonstrating reduced BMPR2 signaling and elevated TGF-β activity in IPF. In the bleomycin (BLM) model of lung fibrosis and PH, we also report decreased BMPR2 expression compared with control animals that correlated with vascular remodeling and PH. We show that genetic abrogation or pharmacological inhibition of interleukin-6 leads to diminished markers of fibrosis and PH consistent with elevated levels of BMPR2 and reduced levels of a collection of microRNAs (miRs) that are able to degrade BMPR2. We also demonstrate that isolated bone marrow-derived macrophages from BLM-exposed mice show reduced BMPR2 levels upon exposure with IL6 or the IL6+IL6R complex that are consistent with immunohistochemistry showing reduced BMPR2 in CD206 expressing macrophages from lung sections from IPF and IPF+PH patients. In conclusion, our data suggest that depletion of BMPR2 mediated by a collection of miRs induced by IL6 and subsequent STAT3 phosphorylation as a novel mechanism participating to fibroproliferative and vascular injuries in IPF.

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