An Update on Pulmonary Arterial Hypertension

• Pulmonary arterial hypertension is a chronic, debilitating disease that leads to right heart failure and premature death.
• The diagnosis of PAH is one of exclusion.
• PAH-specific treatments include: prostanoids, enothelin receptor antagonists and phosphodiesterase-5 inhibitors.

Pulmonary arterial hypertension (PAH) is a progressive disease that ultimately leads to right heart failure and death. PAH is defined as a mean pulmonary arterial pressure ≥ 25 mmHg with a pulmonary capillary wedge pressure ≤ 15 mmHg at rest. The diagnosis of PAH is one of exclusion; diagnostics include an extensive history, serology, chest radiograph, pulmonary function tests, ventilation/perfusion scan, transthoracic echocardiogram, and right heart catheterization. Treatment and care of patients with PAH can be complex. Therefore, the nurse practitioner is an integral member of the health care team caring for PAH patients, helping to ensure seamless care and support.