After being pumped around all of the arteries, veins and capillaries in the adult human body, the blood refuels with oxygen in the lungs, and then starts the cycle all over again. For patients with pulmonary arterial hypertension (PAH), however, the refuelling process becomes increasingly obstructed when thickening of the blood vessel wall reduces the volume of blood able to pass through. Eventually, unable to pump the blood through the arteries that traverse the lung, the heart fails.
PAH is responsible for 10–15 cases per million people each year; in the UK, there are currently 5,000 people with the disease. PAH has a poor prognosis, as Professor Nick Morrell, British Heart Foundation (BHF) Chair of Cardiopulmonary Medicine at Cambridge’s Department of Medicine, explained: “Patients – often women in early middle age – present with unexplained breathlessness and abnormally high blood pressure in the lungs. The only available drugs have rarely accomplished more than disease stabilisation. Without a lung transplant, the disease can prove fatal within three years.”
Now, a promising recent development in drug treatment for PAH could revolutionise the treatment of this disease. The recently completed Phase III trial was based on research from Morrell’s team and collaborators in Giessen, Germany, and has demonstrated the efficacy and safety of a drug that directly combats the narrowing of blood vessels.
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Image: Confocal microscopy image of a small pulmonary artery in a patient with pulmonary arterial hypertension
Credit: Dr Mark Southwood, Papworth Hospital
Reproduced courtesy of the University of Cambridge
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Breathtaking: new treatments for a fatal lung disease
8 March 2013
Research paves the way for new approaches in the treatment of pulmonary arterial hypertension, a progressive lung disease that can lead to heart failure within three years.