Management of Pulmonary Hypertension Associated with Chronic Lung Disease

Isabel Blanco; Fernanda Hernández-González; Agustín García; Rodrigo Torres-Castro; Joan A. Barberà

doi:10.1055/s-0043-1770121

Seminars in Respiratory and Critical Care Medicine, Inhaltsverzeichnis

Semin Respir Crit Care Med 2023; 44(06): 826-839
DOI: 10.1055/s-0043-1770121

Review Article

Management of Pulmonary Hypertension Associated with Chronic Lung Disease

Isabel Blanco

¹Department of Pulmonary Medicine, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Hospital Clínic-University of Barcelona, Barcelona, Spain

²Biomedical Research Networking Center on Respiratory Diseases (CIBERES), Madrid, Spain

³European Reference Network on Rare Pulmonary Diseases (ERN-LUNG), Spain

,

Fernanda Hernández-González

¹Department of Pulmonary Medicine, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Hospital Clínic-University of Barcelona, Barcelona, Spain

²Biomedical Research Networking Center on Respiratory Diseases (CIBERES), Madrid, Spain

³European Reference Network on Rare Pulmonary Diseases (ERN-LUNG), Spain

,

Agustín García

¹Department of Pulmonary Medicine, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Hospital Clínic-University of Barcelona, Barcelona, Spain

²Biomedical Research Networking Center on Respiratory Diseases (CIBERES), Madrid, Spain

³European Reference Network on Rare Pulmonary Diseases (ERN-LUNG), Spain

,

Rodrigo Torres-Castro

¹Department of Pulmonary Medicine, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Hospital Clínic-University of Barcelona, Barcelona, Spain

²Biomedical Research Networking Center on Respiratory Diseases (CIBERES), Madrid, Spain

³European Reference Network on Rare Pulmonary Diseases (ERN-LUNG), Spain

,

Joan A. Barberà

¹Department of Pulmonary Medicine, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Hospital Clínic-University of Barcelona, Barcelona, Spain

²Biomedical Research Networking Center on Respiratory Diseases (CIBERES), Madrid, Spain

³European Reference Network on Rare Pulmonary Diseases (ERN-LUNG), Spain

› Institutsangaben

Abstract

Pulmonary hypertension (PH) is a common complication of chronic lung diseases, particularly in chronic obstructive pulmonary disease (COPD) and interstitial lung diseases (ILD) and especially in advanced disease. It is associated with greater mortality and worse clinical course. Given the high prevalence of some respiratory disorders and because lung parenchymal abnormalities might be present in other PH groups, the appropriate diagnosis of PH associated with respiratory disease represents a clinical challenge. Patients with chronic lung disease presenting symptoms that exceed those expected by the pulmonary disease should be further evaluated by echocardiography. Confirmatory right heart catheterization is indicated in candidates to surgical treatments, suspected severe PH potentially amenable with targeted therapy, and, in general, in those conditions where the result of the hemodynamic assessment will determine treatment options. The treatment of choice for these patients who are hypoxemic is long-term oxygen therapy and pulmonary rehabilitation to improve symptoms. Lung transplant is the only curative therapy and can be considered in appropriate cases. Conventional vasodilators or drugs approved for pulmonary arterial hypertension (PAH) are not recommended in patients with mild-to-moderate PH because they may impair gas exchange and their lack of efficacy shown in randomized controlled trials. Patients with severe PH (as defined by pulmonary vascular resistance >5 Wood units) should be referred to a center with expertise in PH and lung diseases and ideally included in randomized controlled trials. Targeted PAH therapy might be considered in this subset of patients, with careful monitoring of gas exchange. In patients with ILD, inhaled treprostinil has been shown to improve functional ability and to delay clinical worsening.

Keywords

pulmonary circulation - chronic respiratory diseases - chronic obstructive pulmonary disease - interstitial lung disease - idiopathic pulmonary fibrosis - PAH-targeted therapy

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