On Air Archive

Research by PHA-UK (Pulmonary Hypertension Association UK), the patient charity for Pulmonary Hypertension (PH) patients indicates that over 50% of PH patient’s are having to endure a wait of up to two years, with 41% having to see up to four different doctors, before receiving a correct diagnosis and access to the specialist care and treatments they need to control their condition.

Due to its similarities to the more common asthma and lack of awareness of the condition, health professionals often mistakenly diagnose PH.  With correct treatment and a faster diagnosis almost two thirds of Pulmonary Hypertension patients will survive longer than 5 years, however if not treated, approximately half of PH patients die within two years.

Pulmonary Hypertension (PH) is a rare lung disorder in which the blood pressure in the pulmonary artery rises far above normal levels, usually with no apparent reason.

Symptoms: include chronic fatigue, shortness of breath (dyspnoea), chest pain (angina), fainting, swollen ankles and legs (oedema), and fluid in the abdomen (ascites). These are also symptoms for other diseases such as congestive heart failure; therefore physicians should rule out other diseases before making a diagnosis of Pulmonary Hypertension.

Causes: Pulmonary hypertension may develop after pregnancy, hypothyroidism, pulmonary embolism, autoimmune disorders, or human immunodeficiency virus (HIV) infection or result from the use of drugs such as appetite suppressants, oral contraceptives, and cocaine.
Incidence: Although there is no accurate record of the incidence of PH there are approximately 4,000 PH sufferers in the UK and it is estimated that there are an additional 4,000 undiagnosed sufferers.
Can PH Be Cured?: PH has been historically chronic and incurable with a poor survival rate. However, new treatments are available which have significantly improved prognosis.
Primary Pulmonary Hypertension: When Pulmonary Hypertension occurs in the absence of a known cause, it is referred to as primary pulmonary hypertension (PPH). There are likely many unknown causes of PPH. PPH is extremely rare, occurring in about two persons per million population per year.
Secondary Pulmonary Hypertension (SPH): This refers to PH when the cause is known. Common causes: breathing disorders, emphysema, bronchitis.  Less common disorders: inflammatory or collagen vascular diseases such as scleroderma, CREST syndrome or systemic lupus erythematosus (SLE), Congenital heart diseases that cause shunting of extra blood through the lungs like ventricular and atrial septal defects, chronic pulmonary thromboembolism (old blood clots in the pulmonary artery),HIV infection, liver disease, allegedly 'diet drugs like fenfluramine and dexfenfluramine.

Specialist PH Centres: There are 8 specialist PH centres in the UK: Western Infirmary, Glasgow, Freeman Hospital, Newcastle, Royal Hallamshire, Sheffield, Papworth Hospital, Cambridge, Great Ormond Street Hospital, London, Hammersmith Hospital, London, Royal Brompton Hospital, London, Royal Free Hospital, London.