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Revision as of 23:06, 25 May 2004 by PFHLai (talk | contribs)(diff) ← Previous revision | Latest revision (diff) | Newer revision → (diff)Pulmonary hypertension is an increase in arterial blood pressure in the pulmonary or lung vasculature.
Causes
Pulmonary hypertension can be primary (occurring without an obvious cause) or secondary (a result of other disease processes.)
Primary pulmonary hypertension (PPH) is considered a genetic disorder. Patients usually have no symptoms until they reach their late twenties or early thirties. PPH is very rare but often fatal. It is characterized by elevated pulmonary vascular resistance attributable to the abnormal thickening of the vessel wall and narrowing of the lumen of arterioles in the lungs.
A very common cause of secondary pulmonary hypertension (SPH) is chronic obstructive pulmonary disease (COPD). Many other disease processes such as pulmonary embolism, fibrosis in the lungs and asthma are also known to result in SPH.
Signs, symptoms and examination findings
Normal pulmonary arterial pressure in a person living at sea level has a mean value of 12-16mmHg. Definite pulmonary hypertension is present when mean pressures exceed 30-20mmHg. A history usually reveals gradual onset of shortness of breath, fatigue, angina pectoris, syncope (fainting) and peripheral edema.
A common association with chronic pulmonary hypertension is cor pulmonale (right sided heart failure) believed to be caused by the increased load on the right ventricle and atrium of the heart. A chest radiograph (x-ray) will often reveal an enlarged right atrium and ventricle, and prominent pulmonary arteries. An ECG will often demonstrate right ventricular hypertrophy.
Risk factors
Women are almost twice as likely to present with PPH than men. Obesity and the use of appetite suppressants (e.g. Fen-phen) are among the risk factors.
Treatment
Treatment is determined by the condition underlying the cause of the hypertension. For instance, long term oxygen therapy can be useful in patients with obstructive lung disease. In PPH, lifestyle changes, digoxin, diuretic, oral anticoagulants, oxygen therapy and vasodilators are the mainstays of treatment.
Prognosis
Several studies have reported a mean survival of 2-3 years from time of diagnosis with the cause of death usually being right ventricular failure (cor pulmonale).
External links
- Facts About Primary Pulmonary Hypertension from the National Heart, Lung, and Blood Institute (NHLBI)
- Appetite-Suppressant Drugs and the Risk of Primary Pulmonary Hypertension, in NEJM, by Abenhaim, L. et al., 1996.