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Testing, treatment help patients with rare condition breathe easier

One of the most difficult parts of living with pulmonary hypertension is not knowing you have it.

In 2018, Sylvia Goulet of East Hampton found herself out of breath after walking up stairs and while working as a certified dental assistant.

“I kept brushing it off,” said Goulet, 49. “I thought I was just out of shape.” 

Her symptoms, which also included nausea and slight heaviness in her chest, finally prompted Goulet to visit a walk-in clinic. She was prescribed medication for asthma and gastroesophageal reflux disease, but her condition worsened.

“Pulmonary hypertension is rare, and its symptoms are similar to those of other conditions,” said Karelys Adorno-Loveless, RN, nurse coordinator with the Pulmonary Vascular Disease (PVD) program, which treats pulmonary hypertension and other conditions. “A lot of patients with pulmonary hypertension are undiagnosed or misdiagnosed for years.”

Normally, blood flows from the body into the right side of the heart, which pumps it through pulmonary arteries into the lungs. The lungs add oxygen to the blood, which the left side of the heart pumps out to the body. Pulmonary hypertension (PH) occurs when blood pressure builds up in the lungs, forcing the right side of the heart to work harder. Untreated, PH can lead to heart failure and death.

After fainting in her workplace parking lot one day, Goulet was taken by ambulance to Middlesex Hospital. Doctors there referred her to Yale New Haven Hospital, where she saw Inderjit Singh, MBChB, PVD program director. He ordered a series of tests, including a number performed by YNHH’s Pulmonary Function Testing program (see sidebar). 

When these tests ruled out other causes of Goulet’s shortness of breath, Dr. Singh ordered a right-heart catheterization. The “gold standard” for diagnosing PH, the procedure involves inserting a catheter into the right side of the heart to measure blood pressure in the heart and lungs. 

Goulet was “shocked” to learn she has pulmonary arterial hypertension due to narrowed pulmonary arteries. Her case is “idiopathic,” meaning it is not secondary to other causes, such as lung or liver disease, connective tissue disease, blood clots or other conditions that can lead to PH. 

There’s no cure for PH, but Goulet now takes medications to lower the blood pressure. One of these, Remodulin (treprostinil), is delivered continuously into a vein via a pump Goulet carries. She’ll be on the medications for the rest of her life, but she doesn’t mind. She considers herself lucky to feel as good as she does.

“It was actually a blessing that I ended up in the hospital and got the tests and treatment I need,” Goulet said. “I feel fortunate that I can live my life.”