Diagnosing and managing pulmonary hypertension

Abstract

Pulmonary hypertension (PH) is defined as an increase in mean pulmonary arterial pressure > or = 25 mmHg at rest as assessed invasively by right heart catheterisation. It can affect patients at any age and presents with non-specific symptoms. Accurate diagnosis is important as while PH is a potentially lethal disease it is treatable. Identification of the cause of PH is crucial to ensure that the patient receives appropriate management. The most common causes are heart and lung disease; specialist drug therapies for PH are contraindicated in these patients. However, PH is also associated with a diverse group of diseases. The most common symptom of PH is breathlessness on exertion. Syncope may be the presenting symptom especially in children. Other PH symptoms include: angina pectoris, palpitations, dry cough, exertional nausea and vomiting. Lack of response to conventional treatment for dyspnoea should alert the GP to search for another cause of the patient's symptoms, one of which could be PH. Patients should have an ECG, a chest radiograph and a full blood count, renal and liver function as well as thyroid function tests. When PH is suspected, referral to the local cardiology or respiratory department is recommended. Referral should be urgent if the patient is syncopal, has rapidly progressing symptoms or is in heart failure. Echocardiogram is the investigation of choice for detecting elevated pulmonary pressures. If the echocardiogram shows signs of PH then the patient should be referred to a designated PH centre if pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension are suspected or if PH is of uncertain cause.