The Effect of Borderline Pulmonary Hypertension on Survival in Chronic Lung Disease

Piccari, Lucilla; Wort, Stephen J.; Meloni, Federica; Rizzo, M.; Price, Laura; Martino, Lavinia; Salvaterra, Elena; Meseguer, Manuel López; Blanco, Isabel; Callari, A.; González, V. Pérez; Tuzzolino, Fabio; McCabe, Colm; Chiaradia, Diego Agustín Rodríguez; Vitulo, Patrizio

doi:10.1159/000524263

Cited by 22 publications

(45 citation statements)

References 31 publications

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“…Interestingly, two recent studies [59,89] found that PVR more than 5 wood units (WU) was correlated with mortality in both conditions, PVR more than 8 WU conferring even better discrimination in ILD. Although some findings concerning COPD patients were replicated [87], differing results in ILD may be due to the different populations: the inclusion [87,88] or exclusion [59] of a cohort without PH, the proportion of patients treated with pulmonary vasodilators (100% [59] versus 17% [87]), the inclusion [59] or exclusion [87] of combined pulmonary fibrosis and emphysema patients (who have a distinct phenotype [7] and are gravely affected by PH [90]) and the distribution of PH severity in the overall ILD cohort (PVR, median and quartiles 1–3: 7.6, 6.0–10.6 WU [59] and 3.6, 2.4–5.0 WU [87]). At any rate, there is likely not one single parameter accurately describing PH severity in all Group 3 PH patients, as the differences in diverse populations highlight.…”

Section: Natural Historymentioning

confidence: 99%

Pulmonary hypertension in interstitial lung disease and in chronic obstructive pulmonary disease: different entities?

Piccari

Aguilar-Colindres

Rodríguez-Chiaradia

2023

Current Opinion in Pulmonary Medicine

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Purpose of review Pulmonary hypertension (PH) is a common complication of both chronic obstructive pulmonary disease (COPD) and interstitial lung disease (ILD), classified as Group 3 PH. To which extent PH presents and behaves similarly in COPD and ILD is unclear. This review examines the similarities and differences in pathogenesis, clinical presentation, natural history and treatment response of PH in COPD and ILD. Recent findings The latest studies on PH in chronic lung disease have re-evaluated the role of traditionally held etiopathogenetic factors such as tobacco exposure and hypoxia, although new ones such as airborne pollutant and genetic mutations are increasingly recognized. We examine common and diverging factors involved in PH development in COPD and ILD, as well as common and diverging clinical features of presentation, natural history and response to treatment and highlight areas for future research. Summary The development of PH in lung disease significantly worsens the morbidity and mortality of patients with COPD and ILD. However, recent findings show importance of recognizing distinct patterns and behaviors of pulmonary vascular disease, taking into account the specific underlying lung disease and severity of the hemodynamic involvement. Further studies are needed to build evidence on these aspects, especially in early disease.

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Section: Natural Historymentioning

confidence: 99%

Pulmonary hypertension in interstitial lung disease and in chronic obstructive pulmonary disease: different entities?

Piccari

Aguilar-Colindres

Rodríguez-Chiaradia

2023

Current Opinion in Pulmonary Medicine

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“… 87 Any level of PH is associated with reduced survival. 1 , 116 , 117 , 118 In fact, levels of mPAP below the current cut off definition of PH are also associated with reduced exercise capacity and poorer outcomes, 3 , 119 indicating that there is a continuous relationship between an increasing mPAP and mortality. 1 Recently, it has been suggested that the threshold for the diagnosis for PH be lowered to mPAP >20 mmHg with a PVR ≥2 WU, 98 which likely will lead to capturing patients at an earlier stage of pulmonary vascular disease 120 ; this new threshold also has strong physiological basis.…”

Section: Phenotypes Of Ph In Ildmentioning

confidence: 99%

Pathogenesis, clinical features, and phenotypes of pulmonary hypertension associated with interstitial lung disease: A consensus statement from the Pulmonary Vascular Research Institute's Innovative Drug Development Initiative ‐ Group 3 Pulmonary Hypertension

et al. 2023

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Pulmonary hypertension (PH) is a frequent complication of interstitial lung disease (ILD). Although PH has mostly been described in idiopathic pulmonary fibrosis, it can manifest in association with many other forms of ILD. Associated pathogenetic mechanisms are complex and incompletely understood but there is evidence of disruption of molecular and genetic pathways, with panvascular histopathologic changes, multiple pathophysiologic sequelae, and profound clinical ramifications. While there are some recognized clinical phenotypes such as combined pulmonary fibrosis and emphysema and some possible phenotypes such as connective tissue disease associated with ILD and PH, the identification of further phenotypes of PH in ILD has thus far proven elusive. This statement reviews the current evidence on the pathogenesis, recognized patterns, and useful diagnostic tools to detect phenotypes of PH in ILD. Distinct phenotypes warrant recognition if they are characterized through either a distinct presentation, clinical course, or treatment response. Furthermore, we propose a set of recommendations for future studies that might enable the recognition of new phenotypes.

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“…6,10 However, according to this registry, there is no difference in survival between the different ILDs associated with PH. Recently, Piccari et al 76 showed that even mild increases in PAP had a negative prognosis impact in ILD.…”

Section: Epidemiologymentioning

confidence: 99%

Management of Pulmonary Hypertension Associated with Chronic Lung Disease

Blanco

Hernández-González

García

et al. 2023

Semin Respir Crit Care Med

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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.

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The Effect of Borderline Pulmonary Hypertension on Survival in Chronic Lung Disease

Cited by 22 publications

References 31 publications

Pulmonary hypertension in interstitial lung disease and in chronic obstructive pulmonary disease: different entities?

Pulmonary hypertension in interstitial lung disease and in chronic obstructive pulmonary disease: different entities?

Pathogenesis, clinical features, and phenotypes of pulmonary hypertension associated with interstitial lung disease: A consensus statement from the Pulmonary Vascular Research Institute's Innovative Drug Development Initiative ‐ Group 3 Pulmonary Hypertension

Management of Pulmonary Hypertension Associated with Chronic Lung Disease

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