Primary pulmonary vascular disease is an uncommonly diagnosed cause of pulmonary hypertension but left sided heart disease, parasitic lung disease, and several commonly encountered respiratory disorders can lead to this secondary complication. Clinical signs of cough, tachypnea, and exercise intolerance overlap with these cardiopulmonary diseases; however, syncope is commonly associated with and suggestive of pulmonary hypertension. Thoracic radiographs demonstrating right heart or pulmonary artery enlargement should also trigger investigation of pulmonary hypertension by use of echocardiography. Further investigation of underlying respiratory conditions can be extensive but might allow targeted therapy. Use of PDE5i and oxygen therapy can provide palliative benefit.