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What Is Pulmonary Arterial Hypertension

Pulmonary hypertension--or PH--refers to an elevation in pulmonary pressure that can occur in any part of the pulmonary vascular bed. Pulmonary hypertension can be seen in association with increased pressure as a result of a decrease in the luminal diameters of small pulmonary arteries.

Pulmonary arterial hypertension--or PAH--is a progressive disease defined by elevated pulmonary artery pressure and pulmonary vascular resistance leading to right ventricular failure and premature death. PH is divided into five WHO groups. Developed in by the 5th World. Pulmonary thromboendarterectomy: If present, blood clots in the pulmonary artery may be surgically removed to improve blood flow and lung function.


Lung transplantation: Currently, this is the only cure for pulmonary hypertension. Transplantation is reserved for advanced pulmonary hypertension that does not respond to medical therapy. About 1, lung transplants are performed every year in the United States.

Women are up to four times more likely than men to be diagnosed with pulmonary arterial hypertension. Pulmonary hypertension is more common in women.

It often appears in otherwise healthy young people. Hoping to change the uncertainty surrounding diagnosis and therapy, Everett and his colleagues developed a new test to measure levels of HDGF in blood, which, Everett says, is a growth factor protein important for the formation of new blood vessels in the lung — a process known to readily occur in the lungs of patients with pulmonary hypertension.

His research group compared blood samples from 39 patients with severe pulmonary arterial hypertension who had failed treatment for pulmonary arterial hypertension and were waiting for lung transplants, and a control group of 39 age, gender and race-matched healthy volunteers.

What is pulmonary hypertension?

They found median protein levels were about seven times higher than in controls, with a median of 1. When the team followed up the new observation with blood tests of HDGF levels in an additional 73 patients over five years, the results were dramatic, Everett says.

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Patients with an HDGF level greater than 0. HDGF was also lower in survivors than nonsurvivors 0. These findings, Everett says, suggest that HDGF has a distinct advantage to current clinical measures for predicting survival in patients with pulmonary hypertension.

Although the biochemical details of why there is a link between pulmonary arterial hypertension and HDGF remain unknown, Everett suspects levels of the growth factor might increase to spur blood vessel healing when arteries stretch in the lungs due to pulmonary arterial hypertension.

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HDGF is unique to current clinical measures of pulmonary hypertension, as it does not come from the heart and reflects more specifically how the disease affects the lungs, he says. More research is needed to reveal whether levels change as drug therapy eases symptoms during the course of a disease, whether the results hold true in children with pulmonary arterial hypertension and whether HDGF can be used to predict patients at risk of developing pulmonary arterial hypertension in the future. Drug treatment is designed to thin the blood, relax arterial walls, and dilate or open up blood vessels.

People with pulmonary arterial hypertension also can benefit from oxygen therapy and surgery to reduce pressure on the right side of the heart. Treatments are complicated and carry numerous serious side effects, Everett says, so finding measures that allow clinicians to minimize drug therapy will reduce side effects and cost.

Health disparities and pulmonary arterial hypertension | CHEST Physician

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