When interstitial lung diseases associates pulmonary hypertension, what to do?
David Rodríguez-Plaza , Jesús Ribas , Maria Molina-Molina 1
1 Interstitial Lung Disease Unit, Respiratory Department, University Hospital of Bellvitge, Barcelona; Biomedical Research Institute of Bellvitge (IDIBELL), University of Barcelona, Barcelona; CIBER of Respiratory Diseases (CIBERES), Madrid, Spain
*Correspondence: David Rodríguez-Plaza, Email not available
Abstract
Pulmonary hypertension (PH) is one of the major comorbidities of interstitial lung diseases (ILD) and negatively impacts on patients’ symptoms and prognosis. PH-ILD diagnosis is made following a three-step strategy. In a first step, suspicion of PH-ILD should be raised if a combination of symptoms, signs, functional impairments, radiological features, and biomarkers is present. In a second step, patients in whom PH-ILD is suspected should undergo transthoracic echocardiography (TTE) for PH screening. In a third step, depending on TTE findings and clinical suspicion, right heart catheterization should be performed to diagnose or rule out PH. Once PH-ILD is diagnosed, a holistic approach is needed, considering optimization of the underlying ILD, assessment of comorbidities, pulmonary rehabilitation, oxygen supplementation, symptom control, evaluation for lung transplantation, and PH therapy. The decision about PH-targeted therapies should be made using a multimodal approach in a multidisciplinary PH-ILD committee.
