Diagnosis and Treatment for PAH
Pulmonary hypertension usually is diagnosed based on personal and family medical history, physical examination, and the results of diagnostic tests. Tests that may be used to diagnose the condition include chest x-ray, echocardiogram, electrocardiogram, and right heart catheterization.
To determine the underlying cause for pulmonary hypertension, additional tests may be performed. These tests include the following:
- Blood tests
- Cardiac MRI
- CT scan
- Pulmonary function tests
- Sleep study
- Stress tests
- Ventilation/Perfusionlung scan
There is no cure for pulmonary hypertension; however, research in this area is ongoing. The goals of treatment are to control symptoms and slow progression of the disease. In some cases, treatment for the underlying cause can help reduce symptoms of pulmonary hypertension.
It is important for patients with pulmonary hypertension to have a doctor who specializes and is committed to this condition, and for patients to work with their health care provider to develop an individual treatment plan. Treatment often is based on the type and severity of the condition and the patient's age and overall health.
Current treatment options include lifestyle changes (e.g., exercise), medications (e.g., water pills, blood thinners, vasoactive pills, inhalants, infusions), oxygen therapy, and surgery (e.g., lung transplant, heart-lung transplant).
In October 2013, the U.S. Food and Drug Administration (FDA) approved riociguat (Adempas) to treat adults with two forms of pulmonary hypertension (chronic thromboembolic pulmonary hypertension [CTEPH], pulmonary arterial hypertension of unknown causes) and macitentan (Opsumit) to treat adults with PAH. Studies have shown that these drugs can help delay progression of the disease and improve the ability of patients to continue to exercise longer.
Adempas and Opsumit carry a Boxed Warning and should not be used in women who are or may be pregnant. They can be prescribed to female patients of childbearing age only through a REMS (Risk Evaluation and Mitigation Strategy) Program. Common side effects of Adempas include headache, dizziness, stomach upset, and swelling (edema) of the hands and feet. Opsumit may cause anemia (low red blood cell count), cold- and flu-like symptoms, headache, and urinary tract infection (UTI).