Advances in prostacyclin therapy for pulmonary arterial hypertension.

To purchase reprints, contact The InnoVision Group, 101 Columbia, Aliso Viejo, CA 92656. Phone, (800) 8092273 or (949) 362-2050 (ext 532); fax, (949) 362-2049; e-mail, [email protected]. ing from disorders that affect the pulmonary vasculature. Before the World Health Organization reclassification, the aforementioned categories were referred to as secondary pulmonary hypertension. Patients with PAH are further classified according to their level of functional ability. This classification is determined by patients’ exercise performance. The functional assessment grading scale shown in Table 2 has been modified according to the functional classification system of the New York Heart Association. Patients with class I disease have PAH but are symptom-free. Patients with class IV disease may have signs or Patricia L. Eells, RN, MS, CPNP Pulmonary arterial hypertension (PAH) is a progressive, lifethreatening, incurable disease. It is characterized by mean pulmonary artery pressure greater than 25 mm Hg at rest or 30 mm Hg with activity. Pulmonary hypertension was once differentiated into primary and secondary forms but was reclassified in 1998 after the World Health Organization symposium on pulmonary hypertension. This reclassification was designed to reflect clinical patterns of disease. The current classification is based on differences between primary pulmonary hypertension (PPH) and pulmonary hypertension of certain known causes. These types of hypertension differ with respect to patients’ clinical manifestations and pulmonary histopathologic findings. The reclassification allows categorization by common clinical features (Table 1). A diagnosis of PPH indicates PAH with no identifiable cause and includes the familial or the genetic form of the disease. Although most cases of PPH are sporadic, the prevalence of familial disease is at least 6%. Other forms of pulmonary hypertension as described in the World Health Organization proposals include pulmonary venous hypertension, pulmonary hypertension associated with disorders of the respiratory system and/or hypoxemia, pulmonary hypertension caused by chronic thrombotic and/or embolic disease, and pulmonary hypertension resultClinicalArticle