Pulmonary Hypertension

Definition
Pulmonary hypertension is a rare blood vessel disorder of the lung in which the pressure in the pulmonary artery (the blood vessel that leads from the heart to the lungs) rises above normal levels and may become life-threatening. Symptoms of pulmonary hypertension include shortness of breath with minimal exertion, fatigue, chest pain, dizzy spells and fainting. When pulmonary hypertension occurs in the absence of a known cause, it is called primary pulmonary hypertension (PPH). This term should not be construed to mean that, because it has a single name, it is a single disease. There are many unknown causes of PPH.  PPH is rare, occurring in about two persons per million population per year.

Secondary pulmonary hypertension (SPH) means the cause is known. Common causes of SPH are the breathing disorders emphysema and bronchitis. Other less frequent causes are the inflammatory or collagen vascular diseases such as scleroderma, CREST syndrome, or systemic lupus erythematosus (SLE). Congenital heart diseases that cause shunting of extra blood through the lungs like ventricular and atrial septal defects, chronic pulmonary thromboembolism (old blood clots in the pulmonary artery), HIV infection, liver disease and diet drugs like fenfluramine (phen-fen) and dexfenfluramine are also causes of pulmonary hypertension.