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Early PAH diagnosis necessary for improved outcomes
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LOS ANGELES — Early diagnosis of pulmonary arterial hypertension is crucial for improving patient outcomes, according to Glenna Traiger, RN, MSN, CNS-BC from the University of California at Los Angeles, who presented at the American Association of Heart Failure Nurses Annual Conference.
PAH is a rare disease, with 15,000-25,000 patients diagnosed and treated in the United States, Traiger said. Patient populations at risk for PAH include users of anorexigens or methamphetamines, patients with scleroderma, HIV infection, portal hypertension, cirrhosis or congenital heart disease, as well as family members of patients with idiopathic or heritable PAH. Symptoms of PAH include dyspnea, fatigue, syncope or near syncope, chest pain, palpitations and leg edema.
According to Traiger, PAH is difficult to diagnose because it occurs most often among women who are overweight, so their symptoms are typically misdiagnosed at first.
“The delay in diagnosis persists even though we have had drugs for this condition since the late 1990s,” she said. “Most patients are diagnosed with late symptoms; and, when it is untreated, PAH has a very poor prognosis.”
Traiger emphasized the importance of early PAH diagnosis in order to increase survival rates and improve patient outcomes. She noted that patients who can walk 400 meters or more during a 6-minute walk test at the time of diagnosis increase their chance for survival at 7 years post-diagnosis by 71%; however, patients are generally Functional Class III or IV at diagnosis.
Treatment goals for patients with PAH include improvement to Functional Class I or II, right ventricular function near normal according to ECG scans, right atrial pressure of 8 mm Hg or less, cardiac index of 2.5-3.0 L/min/m2 or greater, walking between 380 and 440 meters during a 6-minute walk test, a peak VO2 of 15 ml/min/kg or greater on the Cardiopulmonary Exercise Test and B-type natriuretic peptide levels near normal.
“Our short-term goal is to make this a chronic, manageable disease until we can find a cure,” Traiger said. “We want people to live longer and better with this disease, and we are developing more therapies to help control it and make it a chronic disease that patients can live with.”
For more information:
Traiger G. Pulmonary Hypertension: Recognition and Diagnosis. Presented at: American Association of Heart Failure Nurses Annual Conference; June 26-28, 2014; Los Angeles.
Disclosure: Traiger serves as a consultant and on the speaker’s bureau for Actelion Pharmaceuticals, US; Gilead Sciences, Inc.; and United Therapeutics Corp.