Pulmonary Arterial Hypertension
Pulmonary arterial hypertension (PAH) is often silent until well advanced, the presentation can be vague, and the mortality rate when untreated is high. Fortunately, PAH can now be precisely classified and better managed with drugs such as the prostanoids, the endothelin receptor blockers, and the phosphodiesterase-5 inhibitors. Older agents such as calcium channel blockers, anticoagulants, or diuretics may also remain useful as supportive care.
The new drugs—especially the oral agents—dramatically improve treatment options for patients with PAH and published algorithms can assist clinicians in using the expanded array of drugs1. However, managing patients with advanced PAH can be still be tricky and time-consuming. The Temple Lung Center, one of the busiest referral centers for PAH, can assist at several junctures.
Diagnosis
PAH can be idiopathic or associated with disorders such as thrombotic/embolic disease, left-sided cardiac disease, hypoxemia, scleroderma, or sarcoidosis. The patient's functional severity can range from no limitation of physical activity to severe dyspnea at rest. Temple pulmonologists perform comprehensive diagnostic exams aimed at distinguishing between the various PH subtypes and staging disease severity. The clinicians here are expert in administering the variety of lung function tests needed to diagnose PAH, rule out other conditions, and choose appropriate therapy.
Selection, initiation, and monitoring of therapy
The diversity of possible PAH presentations underscores the need for an up-to-date knowledge of drug safety and efficacy standards in these various settings. Temple physicians have first-hand experience in vasodilator assessment and in the proper initiation of new and investigational agents in a range of complex scenarios.
From an administrative standpoint, initiation of certain PAH therapies can be cumbersome. Temple's full-time PAH administrator is expert in getting these special drugs approved quickly by insurers and thereby assuring that your patient can begin appropriate therapy without delay.
Selecting and properly administering the most appropriate pharmacologic regimen will slow but won't necessarily halt the progression of PAH. Temple clinicians can advise on options (eg, switching therapies, transplantation, ventilator therapy) to consider when the patient worsens. See the separate section on Lung Transplantation for a description of the growing Temple transplantation program.
Research that is reshaping the standard of care
With one of the most active, innovative, and inclusive pulmonary research programs in the country, Temple respiratory specialists are continually challenged with a wide range of seriously ill patients. This constantly motivates them to pursue new avenues of diagnosis and treatment and it keeps them up to date with the latest evidence-based therapies. This research program in PAH benefits specialists near Temple because it provides them with new options for managing patients who are not improving on standard therapy. Temple is currently testing several novel agents in PAH and it is also monitoring long-term disease progression in patients with early stages of the disease. Please contact us for information on any of our ongoing PAH clinical trials and for specifics on patient eligibility criteria.
References
- Badesch DB et al. Chest 2007;131:1917-1928.
- Scientific Registry of Transplant Recipients, Program Specific and OPO Reports January 2008: www.ustransplant.org
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