Primary Pulmonary Hypertension

Primary Pulmonary Hypertension

Pulmonary Hypertension is one of the rare diseases being characterised by increased level of blood pressure in the pulmonary arteries. The condition can be classified into two types namely Primary Pulmonary Hypertension and Secondary Pulmonary Hypertension.

Primary Pulmonary Hypertension is shortly termed as PPH. It is also referred as Precapillary Pulmonary Hypertension – PPH, Primary Pulmonary Artery Hypertension – PPAH, unexplained pulmonary hypertension and Idiopathic Pulmonary Artery Hypertension – IPAH. Idiopathic pulmonary artery hypertension refers to the condition of PPH with no apparent and evident cause. It can occur at any time which does not even involve any family history, genetic disorder or inheritance. Elevated levels of pressure in artery damages the lungs and can lead towards failure of right ventricle.

Causes of Primary Pulmonary Hypertension

Primary Pulmonary Hypertension generally occurs when there is an elevation in the pressure of the pulmonary artery. Heart pumps the impure blood to the lungs for purification. It is the responsibility of the lungs to supply the oxygenated and purified blood and remove the carbon dioxide from the blood. The purified blood is then sent back to heart, which is supplied to the whole body through left ventricles. Blood vessels that are connected with lungs are responsible for connecting the heart’s right ventricles to carry the blood with oxygen to the arteries. Eventually, Primary Pulmonary Hypertension also occurs when elevated blood pressure levels in the major blood vessels, responsible for connecting the lungs as well the right ventricle of the heart. A very high level of pressure in the pulmonary artery makes it difficult for the heart to pump adequate amount of blood to get purified by the lungs. Eventually, it works much harder to pump the blood and gradually lead towards weakening of heart muscles and heart failure.

As stated above, there are no apparent causes of primary pulmonary hypertension. Even though the condition is associated with arteries and the heart, it is likely to be considered a lung disorder. Even though Primary Pulmonary Hypertension is an idiopathic condition, medical experts have discovered that genetic mutations can result towards Primary Pulmonary Hypertension.

Risk factors of Primary Pulmonary Hypertension

Women are more prone to develop the condition than men. Men can develop primary pulmonary hypertension at any age and is more common in women in the age group of 20 years to 45 years. More than 500 cases affected by primary pulmonary hypertension are diagnosed every year.

Symptoms of Primary Pulmonary Hypertension

There are no symptoms of primary pulmonary hypertension in the initial stages and the signs are likely to develop gradually. Common signs of Primary Pulmonary Hypertension include shortness of breath, difficulty in breathing, chest pain and cough. As the condition aggravates, the symptoms also get aggravated.

Treatment of Primary Pulmonary Hypertension

As the condition is an idiopathic disorder with no causes, there is no effective cure or treatment for primary pulmonary hypertension. However, few medicines are prescribed to reduce the effect. The drugs that are prescribed to treat the primary pulmonary hypertension are endothelium antagonists, prostaglandins and phosphodiesterase – type 5 inhibitors. There are quite a few new therapies has been introduce to treat the condition, where the effectiveness of the treatment is still unknown. Fortunately, they are little effective in alleviating the symptoms.

Primary pulmonary hypertension is quite severe and aggressive over the other form of pulmonary hypertension. In some cases, it is found to be a fatal condition.

Primary pulmonary hypertension prognosis state that more than 50% of have the mortality rate of maximum of three years. Life expectancy of primary pulmonary hypertension is found around 3 years when the condition is left untreated after diagnosis. Fortunately, the new medical therapies found to increase the survival rate of the patients by 5 years. More than 65% of patients are likely to respond to therapies. Patients who do have positive respond to new medical therapies obviously develop heart failure, where their life can be saved only by heart transplantation.

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