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

Purpose of review: 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).

Recent findings: The first steps in management are to understand the mechanisms and hemodynamic profile and to exclude chronic thromboembolic disease. Recent studies in the past 5 years 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.