Diagnosis and Treatment

How do you diagnose pulmonary hypertension?

When pulmonary hypertension is suspected, the screening test of choice is an echocardiogram, which is an ultrasound of the heart. This test will give an idea of how high the pressures on the right side of the heart are. The test which is used to confirm the echocardiogram findings and get more detail is a cardiac catheterization. Both of these tests should be done in centers with pulmonary hypertension expertise so that the data is interpreted correctly.

Once the diagnosis is made by echo and/or cardiac catheterization, additional testing will be done as indicated to investigate possible causes of pulmonary hypertension. These include lung function testing, computed tomography (CT) scans of the chest, sleep studies and bloodwork.

How do you treat pulmonary hypertension?

Until the early 1990s, the only therapy for pulmonary hypertension was lung or heart-lung transplantation. Shortly thereafter, the Food and Drug Administration approved epoprostenol as the first specific pulmonary hypertension treatment (1995). We now have 13 drug options on the market specifically to treat pulmonary hypertension; there are other drugs which are used in conjunction with these medications but they are not specific to pulmonary hypertension. Response to therapy is monitored by checking growth, activity tolerance and 6 minute walk tests.

While the advances in medical therapy are incredible and have certainly improved quality and length of life for many persons with pulmonary hypertension, the ultimate outcome remains poor for most types of pulmonary hypertension because there is no known cure for this disease. The medications only help to slow down the disease process; they do not completely reverse it. In some persons with pulmonary hypertension, lung transplantation may be an option.

At Texas Children's Hospital, we utilize oral, inhaled and continuous therapies as indicated to manage pulmonary hypertension. Our medical team is well-versed with the various treatment options and is able to work with specialty pharmacies to ensure that your child receives the treatment he/she needs.