Pulmonary arterial hypertension (PAH) is a rare disease. Patients with suspected PAH should have their doctors perform diagnostic testing based on their suspicion for PAH. This will often include an echocardiogram to estimate how high their pulmonary artery pressure is. If the echocardiogram suggests PAH, or is confirmed by right heart catheterization, it is appropriate for the treating doctor to refer the patient to a doctor that regularly evaluates and treats PAH (PH specialist).
A PH specialist along with the entire PAH team has the most experience managing PAH and has expertise with PAH-specific drugs, such as calcium channel blockers (CCBs), Remodulin, Flolan, and Tracleer. The PAH team may also be participating in clinical research trials that offer new, alternative investigational PAH therapies. Because all of these therapies require expertise in PAH, it is recommended that only PH specialty treatment centers initiate these treatments.
The follow-up care of patients receiving PAH-specific therapies should include both visits to the patient's primary care physician and periodic evaluations by the PH specialist. The frequency of visits should be individualized according to the patient's specific clinical condition. It is also advisable to involve a local cardiologist or pulmonologist in the patient's care.
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