PAH most commonly presents with progressive breathlessness. As right ventricular dysfunction develops patients can experience exeional dizziness and syncope. Oedema and ascites occur late in the disease. Anginal chest pain may occur due to exeional myocardial hypoperfusion in the setting of right ventricular hyperophy, left main coronary aery compression by the pulmonary aery, or ischaemic hea disease. Patients are prone to tachyarrhythmias, most commonly atrial flutter, which can cause sudden decompensation. Haemoptysis, although uncommon, can occur in Eisenmenger's syndrome and CTEPH, where bronchial aeries may be enlarged. Signs suggestive of pulmonary hypeension include a loud pulmonary second hea sound, systolic murmur of tricuspid regurgitation, raised jugular venous pressure, peripheral oedema and ascites, but these signs may be subtle or absent in early disease. Ref: 1. Rich S, Dantzker DR, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, et al. Primary pulmonary hypeension. A national prospective study. Ann Intern Med1987;107:216-23.MedlineWeb of Science 2. Bonderman D, Fleischmann D, Prokop M, Klepetko W, Lang IM. Images in cardiovascular medicine. Left main coronary aery compression by the pulmonary trunk in pulmonary hypeension. Circulation2002;105:265.