Symptoms and clinical signs of PH
There is no pathognomonic clinical sign of PH. Clinical presentation is related either to right heart failure or to associated diseases. Persistant dyspnea on exertion is the most frequent symptom; and it is present in almost patients even in the presence of mild hemodynamic abnormalities [1,182]. Dyspnea usually starts insiduously and is often neglected by patients which explain the delay of around two years in establishing the diagnosis of PH. The New York Heart Association (NYHA) provides a classification system for the clinicial evaluation of dyspnoea. Four categories are proposed to classify patients in functional classes (FC) based on how much they are limited during physical activity; the limitations/symptoms are in regard to normal breathing (Table 3).
Table 3  Modified New York Heart Association (NYHA) classification for pulmonary hypertension   However, at time of diagnosis, 70% of patients are in NYHA FC III or IV. Chest pain, light-headedness and syncope may occur, particularly during physical efforts and are major signs of disease severity. Palpitations are frequent during physical efforts and may reveal true cardiac arrhythmias. Other symptoms of PAH include fatigue and weakness. Hemoptysis may complicate PAH and could be life-threatening, justifying embolization of dilated bronchial arteries. Hoarseness of the voice may occasionally be noted and is due to compression of the left laryngeal nerve by the dilated pulmonary artery (Ortner’s syndrome).
Signs of right heart failure may be observed in the most severe patients, including venous jugular distension, hepato-jugular reflux, hepatomegaly and hepatalgia. Lower limb edema, ascitis and generalized edema underscore the severity of right heart failure. Cardiac auscultation shows usually a prominent pulmonary component of S2, a systolic murmur of tricuspid regurgitation and more rarely a diastolic murmur of pulmonary regurgitation. Pulmonary auscultation is usually normal and contrasts with the importance of dyspnea. History and clinical examination should also screen for manifestations of extra thoracic diseases, particularly Raynaud’s syndrome which can be found in PAH associated with CTD and particularly in systemic sclerosis.