Advances in the Investigation, Assessment and Treatment of Pulmonary Hypertension

Dear Colleagues,

Pulmonary hypertension (PH) is a vascular disease, characterized by a progressive increase in pulmonary pressures, ultimately leading to death from right heart failure.

PH may occur as a consequence of pulmonary vascular disease, chronic left heart or lung disease, pulmonary embolism, or other etiologies. The worldwide symposium on PH has led to an internationally accepted classification of PH into 5 groups: Group 1 comprises pulmonary arterial hypertension (PAH). Group 2 comprises PH associated with left heart disease. Group 3 consists of PH associated with lung disease and/or hypoxia. Group 4 concerns chronic thromboembolic PH. Finally, Group 5 constitutes PH due to various causes, including hemolytic anemia, sarcoidosis, histiocytosis X, glycogen storage disease, and renal diseases.

Pulmonary arterial hypertension (PAH) is a rare and complex pulmonary vasculopathy, characterized by pulmonary artery vasoconstriction and vascular remodeling, leading to vascular rarefaction with elevation of pulmonary arterial pressures and pulmonary vascular resistance. Current PAH-targeted therapies improve functional capacity and pulmonary hemodynamics and reduce hospitalization. Nevertheless, to date, PAH remains incurable and is often refractory to medical therapy, underscoring the need for further research. In recent decades, PAH has evolved from a disease of unknown pathogenesis devoid of effective therapy to a condition whose cellular, genetic, and molecular underpinnings are unfolding.

This review provides an update on current knowledge and summarizes the progression in recent advances in pharmacological therapy in PAH.

Additionally, at present, no Group 2 or 3 PH targeted therapies have been shown to improve patient outcomes. New discoveries in the treatment of Groups 2 and 3 have provided new hope for these devastating syndromes. This review will also summarize the recent advances in pharmacological therapy in Group 2–4 PH patients.

Prof. Dr. David Barnes
Guest Editor

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• current state of PH treatment
• new targeted pathway perspectives for the treatment of Group 1 PAH
• new treatment perspectives in Group 2–3
• new treatment perspectives in CTEPH (Group 4)
• overview of exercise-induced PH
• overview of Group 5 PH and potential treatment