Primary pulmonary hypertension is the condition in which the pressure in pulmonary arteries becomes abnormally elevated. It is a type of pulmonary hypertension. This condition is nowadays referred to as Pulmonary Arterial Hypertension(PAH).
The pulmonary artery carries the blood from the right ventricle to the lungs to get oxygenated. After oxygenation, the blood flows to the heart and is then pumped to the rest of the body via the aorta. However, for no apparent reason, at times the pulmonary artery pressure elevates far above normal levels.
The symptoms may initially appear to be of minor concern and tend to worsen over time. In such an instance, the diagnosis is often delayed. Symptoms include dizziness, shortness of breath, fatigue, fainting and weakness,and swelling around the ankles and feet. In the advanced stage, patients may experience chest pain and their lips and skin may take on a bluish tint.
Primary pulmonary hypertension is also referred to as unexplained pulmonary hypertension or idiopathic pulmonary arterial hypertension, since it is a disease of unknown causes. Primary pulmonary hypertension is an often fatal form of pulmonary hypertension and found commonly to affect youngsters. The building up of smooth muscle cells that line the arteries causes the arterial obstruction but the underlying cause has been elusive to physicians. Researchers think that the blood vessels are sensitive to particular internal or external factors and they constrict when exposed to these factors. A genetic cause has been discovered recently though and the fault lies with mutations in a gene called BMPR2. This might provide a means of genetic diagnosis and narrowing down a potential sample of patients for therapy.
Diagnosis and risk factors
The diagnosis of primary pulmonary hypertension requires several tests since many of the symptoms are similar to other disease conditions of the heart and lung. PPH cannot be diagnosed until all types of secondary pulmonary hypertension have been excluded. The maximum number of reported patients have been found to be women aged between 20 and 40. But, men and women in all age ranges as well as young people are susceptible to PPH.
Diet drugs such as Fen Phen, fenfluramine and dexfenfluramine have been associated with PPH. Even though the patient may have stopped taking the drugs years ago, he has the potential to be diagnosed with PPH after symptoms worsen over time. Certain studies conducted have revealed that the diet drug patients have normal pulmonary artery pressure when they are relaxed, but abnormal levels when they exercise. In their case, an exercise test would be adequate.
There is no known treatment that can halt the progress of the disease. The treatments available aim to slow down disease progression thus improving the patients’ quality of life. In the absence of such a treatment the life expectancy of the patient dwindles to less than five years.
The treatment of PPH is similar to that of systemic hypertension. Individual patients may have dramatically different reactions to a specific drug, and so different drugs have to be tried before a long term treatment is done with a specific drug. Vasodilators are used to tackle PPH. Calcium channel blocking drugs or Prostacyclin is used. Tracleer is another potent vasodilator. Oxygen treatment is another method to counter PPH, thereby increasing the oxygen in the blood in order to alleviate PPH symptoms. Combination therapy is being increasingly used leading to a rise in the life expectancy in recent times. Ultimately, in order to counter PPH, replacement of the lung via lung transplant may end up in saving the patient’s life. And if the heart is affected also, a combined heart-lung transplant procedure is to be done