Topic-Based, Recent Literature Review on Pulmonary Hypertension

Abstract

Pulmonary hypertension is a complex condition but a relatively common manifestation of severe cardiopulmonary disease. By contrast, pulmonary arterial hypertension is uncommon and is more prevalent in young women. To better categorize patients and to guide clinical decision-making, 5 diagnostic groups and associated subgroups characterize the spectrum of disease. A multidisciplinary approach to evaluation and treatment is recommended by published guidelines and often entails referral to a designated pulmonary hypertension center. Several key publications during the last couple of years merit review. The PubMed database was searched for English-language studies and guidelines relating to pulmonary hypertension. The following terms were searched, alone and in combination: pulmonary hypertension, pulmonary arterial hypertension, portopulmonary hypertension, and chronic thromboembolic pulmonary hypertension. The focus was on those publications with new information on evaluation and management of pulmonary hypertension between January 1, 2019, and January 31, 2021. Of the subgroups, 2 were of particular interest for this review: portopulmonary hypertension and chronic thromboembolic pulmonary hypertension. Last, available data on the impact of the coronavirus disease 2019 pandemic and newer treatment agents in early trials were selectively reviewed. The review is therefore intended to serve as a practical, focused review of important topics germane to those clinicians caring for patients with pulmonary hypertension.pulmonary hypertension, pulmonary arterial hypertension, right ventricular failure

Dynamic association of ambient air pollution with incidence and mortality of pulmonary hypertension: A multistate trajectory analysis

“…Clinical variables including associated conditions and medication use. Associated conditions include left-heart disease, obstructive pulmonary disease, restrictive lung disease, complex congenital heart disease and connective tissue disease ( Burger et al, 2021 ). In addition, self-reported information on medication use, such as lipid- and blood pressure-lowering medications, as well as insulin, was collected.…”

Current clinical understanding and effectiveness of portopulmonary hypertension treatment

“…There are several possible reasons for this. First, PoPH has conventionally been excluded from drug trials because of concerns about hepatotoxicity ( 50 ). For example, in a previous study, bosentan (an endothelin receptor antagonist) led to an elevation in transaminases in approximately 10% of patients with Group 1 PAH without previous liver disease ( 51 ).…”

Immunosuppressive Treatment for an anti-U₁ Ribonucleoprotein Antibody-positive Patient with Pulmonary Arterial Hypertension

“…Both idiopathic PAH (IPAH) and PAH with connective tissue disease (CTD-PAH) are considered group 1, although they differ in not only pathophysiology but also treatment strategy. In IPAH, combination oral therapy with an endothelin receptor antagonist plus a phosphodiesterase type 5 inhibitor or prostanoid therapy infused with either treprostinil or epoprostenol combined with oral therapy is recommended to improve the outcome ( 2 ). For patients with PAH associated with systemic lupus erythematosus or mixed CTD (MCTD), immunosuppressive therapy combined with pulmonary vasodilators is suggested in the guidelines ( 3 ).…”