Welcome to the PAH Treatment Center. This section is dedicated to reviewing the treatment
options for PAH. Once the diagnosis of PAH is confirmed via right heart catheterization, many patients go on
to initiate oral and/or intravenous treatment.In the US, treatment guidelines from the American College of Cardiology (2009) are available to guide the
care of patients with PAH. The treatment of PAH evolved greatly over the last decade as numerous treatment
options became available to treat this disease. There are several classes of drugs available for the treatment of PAH. These include calcium channel
blockers (CCBs), endothelin antagonists (ERAs), phosphodiesterase type-5 inhibitors (PDE-5s), and
prostacyclin analogs.
Goals of Therapy
There are numerous treatment goals in PAH. Most importantly, though, is an improvement in patient
symptoms and well-being. This is typically measured using assessments of functional capacity including
the 6-minute walk test (6-MWT)and assessment of NYHA functional class. Since PAH is characterized by
severe hemodynamic abnormalities, improvement in cardiac hemodynamics is often considered. Another
important clinical goal is the prevention of disease worsening. This has been measured in clinical trials using
the “time-to-clinical worsening” endpoint. Lastly, because PAH is a disease of severely impaired survival,
prevention of morbidity and mortality is a goal. To date, no therapies have been shown to improve this in
clinical trials. (McLaughlin 2009, JACC)
When evaluating treatment options for PAH, many of these parameters are considered and are often used
in determining treatment success. These assessments may also be used to determine if more aggressive
measures, such as intravenous or combination therapy, are warranted.
Treatment Approach
The treatment approach according to the ACCP is listed in figure 1. As you can see, the treatment of PAH
is complex and is based on the overall risk profile of patients. Low-risk patients are typically considered
patients without evidence of right heart failure, typically in WHO FC II or III, with relatively preserved cardiac
output and ability to function via the 6-MWT. In low-risk patients first line therapy is typically an oral PDE-5
or ERA. Higher-risk patients typically should be started on IV prostacyclin. Combination therapy is reserved
for patients who do not adequately respond to initial monotherapy. Lung transplantation is the last resort
for patients whose disease progresses despite aggressive therapy.
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The Role of Allied Health Professionals in the Treatment of PAH
The treatment of PAH is complex, often involving intravenous and multi-therapy regimens. Within the
Allied Health field, nurses and pharmacists are largely involved in the care of patients with PAH.
The ACCP has set recommendations for nurse and allied health interactions with patients (figure 2).
Frequent telephone calls are necessary during initiation of therapy, after an episode of decompensation,
and for periodic evaluation during treatments. Nurses are also heavily involved in the education of patients
regarding the disease of PAH, drug therapies, and also the overall treatment plan and approach. You will
find many tips and tools in the Resource Center to aid in the education and management of patients.
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