Pulmonary hypertension is a rare disorder characterized by abnormally high blood pressure in the arteries that supply blood to the lungs (pulmonary arteries). Pulmonary hypertension is not the same as general hypertension. The abnormally high blood pressure makes the right ventricle of the heart work harder to pump blood through the lungs. This gradually makes your heart muscle weaker and can lead to heart failure.
Pulmonary hypertension usually occurs as a result of other medical conditions such as blood clots in the lungs, heart valve disease, chronic obstructive pulmonary disease, connective tissue disorder, congenital heart defect, sickle cell anemia, chronic liver disease, AIDS, pulmonary fibrosis and left sided heart failure. This type of pulmonary hypertension is called secondary pulmonary hypertension as it results from another condition, but sometimes, the cause of pulmonary hypertension cannot be identified. In such cases, the condition is termed as idiopathic pulmonary hypertension.
Pulmonary hypertension is a life-long disease and requires complex treatment and extensive follow-up care. Medications are prescribed depending on the severity of disease, likelihood of progression and individual drug tolerance. It may include blood thinners, diuretics, potassium, inotropic agents and vasodilators. Medications to lower pulmonary hypertension and reverse the scarring in the pulmonary blood vessels are also prescribed. Oxygen therapy may be recommended if you have low oxygen levels in your blood. If pulmonary hypertension is caused by an underlying condition, your doctor will treat the underlying cause.
When pulmonary hypertension cannot be managed with medications, surgery is advised. This may involve atrial septostomy or pulmonary thromboendarterectomy. Atrial septostomy is an open heart surgery that creates an opening between the left and the right chambers of the heart to relieve pulmonary hypertension. Whereas, pulmonary thromboendarterectomy involves removal of a large blood clot to improve blood flow and reduce pulmonary hypertension. For severe pulmonary hypertension, a lung or a double organ transplant of both heart and lung may be an option.
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