by Lisa Chiplaskey

Created on: June 11, 2008

Pulmonary Hypertension: A Literature Review

Primary Pulmonary Hypertension is a rare, often fatal disease that not only affects the health status of individuals, but their quality of life as well. A rise in occurrence of the disease has prompted increased interest in research of Pulmonary Hypertension. The research to date has awarded new insights into the disease process as well as treatment modalities to better manage the outcome of afflicted individuals.

Pulmonary Hypertension (PH) is described in two forms. Primary Pulmonary Hypertension (PPH), which is the focus of this discussion, occurs in the absence of a known cause, and is thought to be a connection with certain triggers that are not well understood. Secondary Pulmonary Hypertension (SPH) means the cause is known, and is more directly related to another disease process. Some causes of SPH are: emphysema, bronchitis, inflammatory diseases, pulmonary collagen vascular diseases such as scleroderma, CREST syndrome, systemic lupus erythematosis, Eisenmeyer's Syndrome and sickle cell anemia, (Heimann & Bernstein, 2003).
Pulmonary Hypertension is a blood vessel disorder of the lung in which the pressure in the pulmonary artery rises above normal levels and may become life threatening, (Heimann & Bernstein, 2003). Normal pulmonary artery pressure is approximately 14 mmHg at rest. Phillip (2000) states that in patients with PH, the average blood pressure in the pulmonary artery is greater than 25 mmHg at rest and greater than 30 mmHg during exercise. According to Rounds (1998) and De La Mata, Gomez-Sanchez, et. al. (1994), the three main cellular changes noted that occur in the pulmonary vessels that increase pressure from stimuli causing vasoconstriction are: thromboembolic lesions, pulmonary interstitual fibrosis and vascular remolding. The present accepted New York Heart Association (NYHA) Functional Classes of the disease progress from mild to severe and are rated as Class I, Class II, Class III and Class IV respectively, (Rich, 1998).
The prevalence of PPH is estimated at 1-2 million people worldwide, with 500 to 1,000 new cases diagnosed in the United States each year, (American Heart Association, 2003). The true number of cases has eluded researchers since accurate diagnosing of PPH is very complex. Depending on the definition used by clinicians identifying PPH in their practice, Abenhaim, Moride, et. al. (1994), in a case control study of 450 patients from 35 centers, found the annual incidence of PPH

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