Medical Management of Pulmonary Hypertension with Unclear and/or Multifactorial Mechanisms (Group 5): Is There a Role for Pulmonary Arterial Hypertension Medications?

Abstract

The purpose of this review was to outline the mechanisms and to review recent literature on pulmonary arterial hypertension (PAH) medications in group 5 pulmonary hypertension (PH). The first steps in management are to understand the mechanisms and hemodynamic profile and to exclude chronic thromboembolic disease. Recent studies in the past 5years have found that PAH medications may improve hemodynamics in patients with pre-capillary pulmonary hypertension due to sarcoidosis, pulmonary Langerhans cell histiocytosis, lymphangioleiomyomatosis, and myeloproliferative disorders with dasatinib-induced PH. Improvements in exercise capacity are uncommon, and no survival benefit has been demonstrated. There is a risk of pulmonary edema in patients with pulmonary venous involvement or fibrosing mediastinitis when treated with PAH therapies. There is limited evidence supporting the use of PAH medications in group 5 patients, and they may be harmful in certain cases. In most patients with group 5 PH, treatment should be directed to the underlying disease with PAH therapies reserved for patients with severe pre-capillary PH.