Pulmonary hypertension - a disease of heart and lung

Our pulmonary hypertension (PH) service uniquely brings together specialists in both cardiac and respiratory medicine, as PH is one of the few conditions that cuts across both disciplines equally. 

Pulmonary hypertension is a serious condition that causes high blood pressure in the pulmonary arteries – the blood vessels that connect the heart and lungs. Treatable forms of PH are rare – affecting around 4,000 to 5,000 people in the UK. 

The Trust’s specialist PH service brings together experts from a range of different disciplines to manage the condition which, in its most serious sub-type, has a life expectancy without treatment of just three years following diagnosis – a poorer prognosis than many cancers.

Dr John Wort, consultant in pulmonary hypertension, said: “Diagnosing pulmonary hypertension involves a lot of detective work, as the symptoms can be typical of many more common conditions. Once these are ruled out, we can start to make a diagnosis.”

The PH team has a close relationship with imaging colleagues, as highly detailed imaging is needed to make the correct diagnosis. The team also collaborates closely with psychological and palliative care services, as the personal toll of living with PH can be significant.

But perhaps one of the most important partnerships is with the obstetrics department at Chelsea and Westminster Hospital.

Dr Wort explains: “For reasons we don’t yet understand, PH is more common in women than in men, and the single biggest risk for women with the condition is getting pregnant. The strain that childbirth places on the heart means that up to 30 per cent of pregnant PH patients are at risk of dying.

“We know how important motherhood is for many women, so we have a dedicated service that looks after them during pregnancy and childbirth, run by Dr Lorna Swan and Professor Michael Gatzoulis. We arrange for the birth to take place at Royal Brompton, so that when the child is born, the mother is taken straight to our intensive care unit, while the baby goes to Chelsea and Westminster to be checked out before mother and baby are reunited.”

Research is another strong theme for the service, trying to understand more about the causes of this rare condition and making it possible to identify risk factors. For example, a faulty gene has been identified, which when present gives a one in five chance of developing a rare form of the condition; families of such diagnosed patients are all offered follow up if they wish, to pinpoint their chances of developing the condition.

All PH patients are encouraged to be involved in research whenever possible. The Trust’s research partners include Imperial College and Cambridge University Hospitals NHS Foundation Trust.

The strain that childbirth places on the heart means that up to 30 percent of pregnant PH patients are at risk of dying. We know how important motherhood is for many women, so we have a dedicated service that looks after them during pregnancy and childbirth. 

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