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  • Image not available. Pulmonary arterial hypertension (PAH) may be defined as a mean pulmonary artery pressure (mPAP) ≥25 mm Hg at rest with a pulmonary wedge pressure (also known as pulmonary artery occlusion pressure) or left ventricular end diastolic pressure (LVEDP) ≤15 mm Hg measured by right cardiac catheterization.
  • Image not available. Regardless of the etiology, be it unknown or related to an associated medical condition, subgroups of PAH are based on similar clinical and pathological physiology.
  • Image not available. Diagnosis of PAH is growing due to increased awareness and knowledge of the disease state, leading to earlier and improved evaluation and identification.
  • Image not available. The underlying cause of PAH is a complicated amalgam of endothelial cell dysfunction, a procoagulant state, platelet activation, vasoconstriction, loss of relaxing factors, cellular proliferation, hypertrophy, fibrosis, and inflammation.
  • Image not available. Patients with PAH present with exertional dyspnea, fatigue, weakness, and exertion intolerance. As the disease progresses, symptoms of right heart dysfunction and failure, such as dyspnea at rest, lower extremity edema, chest pain, and syncope, are seen.
  • Image not available. The only way to make a definitive diagnosis of PAH is by right heart catheterization. The right heart catheterization provides important prognostic information and can be used to assess pulmonary vasoreactivity prior to initiating therapy.
  • Image not available. The goals of treatment are to alleviate symptoms, improve the quality of life, slow the progression of the disease, and improve survival.
  • Image not available. A general goal of PAH treatment is to correct the imbalance between vasoconstriction and vasodilation and prevent adverse thrombotic events to improve oxygenation and quality of life.
  • Image not available. Nonpharmacologic therapy is frequently used to address comorbid conditions that often accompany PAH.
  • Image not available. Conventional therapy of PAH includes oral anticoagulants, diuretics, oxygen, and digoxin.
  • Image not available.Prostacyclin analogs such as epoprostenol, treprostinil, and iloprost induce potent vasodilation of pulmonary vascular beds.
  • Image not available. Endothelin receptor antagonists, bosentan, ambrisentan, and sitaxsentan, improve exercise capacity, hemodynamics, and functional class in PAH.
  • Image not available. Phosphodiesterase-5 inhibitors, including sildenafil and tadalafil, are potent and highly specific drugs that have been shown to reduce mPAP and improve functional class.
  • Image not available. Only a very small number of patients with PAH demonstrate a favorable response to acute vasodilator testing. These are the only patients amenable to therapy with calcium channel blocker.
  • Image not available. Combination therapy in PAH may address more than one mechanism causing this disease. Combination therapy in clinical trials has provided additional benefit but more studies are needed.

Upon completion of the chapter, the reader will be able to:

  • 1. Discuss the molecular and cellular mechanisms comprising the pathophysiology of pulmonary arterial hypertension (PAH).
  • 2. Recognize the signs and symptoms associated with the clinical presentation of patients with PAH.
  • 3. List the diagnostic tests involved in the assessment of patients presenting with PAH.
  • 4. State the goals of treatment for PAH.
  • 5. Identify the nonpharmacologic treatments of PAH and their role in therapy.
  • 6. Identify the conventional therapy of patients with PAH, including the use of oral anticoagulants, diuretics, oxygen, and digoxin.
  • 7. Compare and contrast the mechanisms of action for the various classes of PAH pharmacotherapy: synthetic prostacyclin ...

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