What is pulmonary hypertension?

Scans of pulmonary hypertension in the lungs

Pulmonary hypertension (PH) means high blood pressure in the lungs. This condition leads to pulmonary vascular disease (PVD), or peripheral arterial disease (PAD): the progressive obstruction of the lung blood vessels, which results in increasing shortness of breath and severe heart failure.

This disease is incurable and often fatal. It can affect people of all ages, races and socio-economics groups; it can be seen in babies born with septal defects (a hole in their heart) and those living at high altitude.

Causes of PVD

Common causes include infection, cigarette smoking, abuse of dietary suppressants and other drugs, as well as other causes which (at least in part) induce significant oxidative stress.

These strains on the cardiovascular systems can result in endothelial cell injury, apoptosis, hyperproliferation, and vaso-occlusive disorders. Pulmonary vascular disease and pulmonary hypertension can also develop for no apparent reason in otherwise healthy people.

Pulmonary Hypertension Symptoms

Regardless of a patient’s particular cause, the effects can have devastating consequences. PVD and PH symptoms include:

  • Numbness, tingling, or weakness in the legs and/or buttocks.
  • Burning or aching pain in the legs, feet and/or toes while resting.
  • A sore/lesion on the legs or feet that will not heal.
  • One or both legs/feet feeling cold or changing colour (pale, bluish, dark reddish).
  • Loss of hair on the legs.
  • General fatigue.
  • Impotence.
  • Gangrene on the extremities (both hands and feet)

Six P’s of PVD

As you can see, many pulmonary hypertension symptoms revolve around limb ischemia and the lack of blood flow to the lower extremities, in particular. As a result, PVD and pulmonary hypertension diagnosis often follows what is commonly referred to as ‘the six P’s – they are as follows:

  • Pain
  • Pallor
  • Pulselessness
  • Poikilothermic
  • Paresthesia
  • Paralysis

PVD in numbers

While new medicines can help considerably by relieving pulmonary hypertension symptoms and prolonging life, there is no cure for pulmonary vascular disease. It is estimated that PVD affects over 70 million people worldwide, although this is an under-estimate as many patients are never diagnosed.

Beyond PVD itself, as many as 20 people per 100,000 suffer from pulmonary embolisms every year, with a mortality rate of 7-11%. A big part of this is lack of diagnosis; the median average survival rate for those who go without diagnosis/specific pulmonary hypertension treatment is just 2.8 years.

Current estimates suggest that around 200 million people in the world suffer from PVD/one or more pulmonary hypertension symptoms. Part of the Pulmonary Vascular Research Institute’s (PVRI) mission is not only to help carry out pulmonary vascular treatment, but to raise awareness around the disease, in general, across as many regions around the globe as possible.

Where does PVRI come in?

Delay in treatment and even failure to make a pulmonary hypertension diagnosis is common in both the developed and developing world. The Pulmonary Vascular Research Institute (PVRI) is a membership organisation specifically aimed at scientists and physicians who have an interest in pulmonary hypertension and PVD, rather than strictly patients and those affected.

However, all our efforts still remain focused on improving the care of patients undergoing pulmonary hypertension treatment, as well as continuing to carry out research into a cure for PVD.

Pulmonary Vascular Research

Any money raised for the PVRI will go directly towards two main purposes – education and research. Education is aimed at raising awareness around pulmonary vascular disease throughout the world by informing doctors on how to better diagnose and manage the disease, so that patients can receive proper treatment as quickly as possible.

Early accurate diagnosis is key to survival. It can often take up to 18-24 months for a patient to be accurately diagnosed, by which time, they have often reached advanced and serious stages of pulmonary hypertension and little can be done to help. However, provided accurate diagnosis has been made within the first six months from onset of the disease, PVD can be managed and patients can receive vital medicines to help them.

What we are contributing to PVD treatment

The PVRI runs large scientific meetings all over the world as well as Master Classes to educate doctors on how to diagnose and manage PVD symptoms in their patients. We have developed a comprehensive, interactive e-learning course on PVD, The PVRI Digital Clinic, which is aimed at specialists who wish to enhance their knowledge on diagnosis and management of pulmonary hypertension in their patients.

The full course, of 12 patient cases, was launched in Lima, Peru 2020 – the site of our 14th Annual World Congress on PVD – and is available to all paid PVRI members.

Pulmonary Vascular Medicines

Research is aimed at facilitating new drug development and eventually finding a cure to treat PVD. We work closely with other partnered organisations the pharmaceutical industry and drug regulatory bodies to develop new medicines.

We have an ongoing collaboration with The Dinosaur Trust, a UK charity founded by a patient's father, who supports specific research targeted at the genetically inherited form of pulmonary vascular disease. Our network spreads to over 6,300 PH professionals in 103 different countries, and our members include the most senior researchers in the field.

A global effort

We believe that our passion for pulmonary vascular treatment and our worldwide network makes it possible for our members to conduct basic, clinical and translational research at a level that no single individual or academic institution could accomplish. Together with our PVRI members, we’re combatting pulmonary vascular disease from every angle.

As well as our various symposiums and global events, we have set up Regional, Disease & Speciality Task Forces all over the world to put into research and treatment into practice. You can see the kind of impact that has been made throughout the history of the PVRI and review the work done each year in our annual reports.

Learn more about PVD

It is all of our responsibility to increase the level of treatment and awareness of pulmonary vascular disease, no matter our level of expertise. With that in mind, the PVRI is more than just a global scientific organisation, it serves as an educational resource for academics and anyone committed to learning more about the condition.

Not only do we publish the highly-respected peer-reviewed journal, Pulmonary Circulation – issues of which can be accessed through our website – our Learning page features everything from recorded and digital conference talks to academic abstracts on pulmonary vascular research.

In terms of online learning resources, be sure to also visit the PVRI E-learning portal, which includes a wealth of interactive courses – including the aforementioned PVRI Digital Clinic.

Why you should join PVRI

With tens of millions of affected by pulmonary vascular disease very year, your life may be impacted PVD whether you know it or not. You don’t have to be in the medical industry to do your bit in this global fight.

So, if you’re a pulmonary hypertension specialist, a healthcare professional, an interested academic or simply want to do your part, Join PVRI now and become one of 6000+ members in our global network.

You don’t have to be a member to make a difference either: donate to the PVRI today to fund the research and development of PVD treatment, medicines and more.

Our research platform is the world.

Through worldwide collaboration, we can begin to answer the question of a global disease.

Join the PVRI