Newswise — Natalie Cole’s death has been linked to pulmonary arterial hypertension. But what is the rare heart disease that took the life of the great American singer who had overcome so many other health challenges?

Pulmonary hypertension is high blood pressure in the loop of vessels connecting the heart and lungs. It often leads to heart failure as the heart works harder to pump blood to the lungs.

Dr. Vallerie McLaughlin, director of the Pulmonary Hypertension Program at the University of Michigan, is among the world’s researchers looking for ways to treat the disease and improve chances of survival. Untreated patients often die in two to three years.

The greatest number of cases of pulmonary hypertension is reported in women between ages 21 and 40 who most often experience fatigue and shortness of breath.

“The first and only drug available for treating pulmonary hypertension was approved 20 years ago and today we have a dozen treatment options,” says McLaughlin who is leading the Pulmonary Hypertension Quality Enhancement Research Initiative Extension Program, which is evaluating how often doctors follow evidence-based pulmonary hypertension guidelines.

Newer therapies have been successful at keeping patients out of the hospital and preventing worsening symptoms.

McLaughlin was a senior investigator in the GRIPHON study, the largest study ever of PH patients, and has been an integral part of every pivotal trial in PH as the spectrum of therapy has grown significantly.