Inhaled Treprostinil in Pulmonary Hypertension Associated with Lung Disease

Group 3 PH is the term used for pulmonary hypertension (PH) in the setting of parenchymal lung disease. Even though Group 3 PH leads to poor survival, compromised functional capacity, and reduced quality of life there are no approved PH treatments for this patient population as PH-specific drugs may result in ventilation/perfusion imbalance.

The author Mariana Faria‑Urbina and colleague conducted a study under the title “Inhaled Treprostinil in Pulmonary Hypertension Associated with Lung Disease” published in the National Library of Medicine. The summary of this study is below:

Objective:

To evaluate the effect of Inhaled Treprostinil in pulmonary hypertension associated with lung disease.

Method:

This retrospective study includes data from 22 patients with PH associated with lung disease. The participants were treated with inhaled treprostinil (iTre) and were followed up clinically for at least 3 months.

Findings:

The slow and sustained pulmonary vasodilatory effect was observed with iTre. The study reports that iTre therapy significantly improved, systolic pulmonary arterial pressure (PAP), WHO functional class (WHO-FC), and 6-min walking test (6MWT) distance. There were no significant changes in resting SpO₂ and supplemental oxygen therapy requirements during follow-up. iTre was tolerated well in patients, with cough being the most commonly reported adverse event. Although most of the patients were using supplementary oxygen at baseline, only one subject experienced increased oxygen requirement after therapy with iTre.

Hence, iTre can be a therapeutic option in patients with Group 3 PH, since iTre might ameliorate perfusion to areas where the ventilation is preserved, improving, therefore, ventilation/perfusion (V/Q) matching.

Limitation:

Authors acknowledge that the retrospective, single-center, observational, uncontrolled design of the study is the main limitation. It is suggested that the results of this study should be interpreted carefully, due to the small sample size and heterogeneity of the population. Another limitation might be that results favoring the potential use of iTRe in Group 3-PH might have been influenced by the presence of pulmonary vascular disease. The study is also limited by the intrinsic subjective nature of FC assessment and the lack of a control group. Investigators unable to comment on the acute effect of iTre on SpO₂ as information about the timing of SpO₂ assessment in relation to the administration of iTre was not available.

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