Primary Pulmonary Hypertension – Breathe in, breathe out

Pulmonary Hypertension (PH), according to the Pulmonary Hypertension Association, is high blood pressure in the arteries of the lungs that can lead to heart failure. This disease affects people of any age, race and ethnicity. Although anyone can get PH, there are risk factors that make some people more susceptible such as family history or genetics, living in high altitudes, obesity, pregnancy, gender, ingestion of certain drugs and other toxins, and other diseases that can lead to pulmonary hypertension.

Primary Pulmonary Hypertension
According to Williams and Hopper (Understanding Medical-Surgical Nursing, p. 459), Primary Pulmonary Hypertension occurs when the arteries that carry deoxygenated blood from the heart to the lungs become narrowed as a result of changes in the vascular smooth muscle. The result is elevated pressure in the pulmonary arteries, requring the right ventricle to work harder to push blood into them. Eventually the right ventricle fails. The reason for these vascular changes is not known. Primary pulmonary hypertension is more common in women between ages 20 and 40 and has a hereditary tendency.

Secondary Pulmonary Hypertension
Secondary Pulmonary Hypertension, the other type of pulmonary hypertension, results from other disorders, such as coronary artery disease or mitral valve disease, both of which increase pressures in the left side of the heart.

What does a person affected with this disease usually feel or experience?
– The most common symptoms include dyspnea and fatigue, which worsen over time.
– Crackles and decreased breath sounds are heard on auscultation.
– Cyanosis and tachypnea (rapid respiratory rate) are noted.
– If heart failure is present, peripheral edema and distended jugular veins are seen.
– Angina may result from right ventricular ischemia.
– Death usually occurs within 2 to 3 years of diagnosis, unless a lung or heart-lung transplant is done.

No cure is available for pulmonary hypertension, except for lung or heart-lung transplant. Supportive care includes a low-sodium diet and diuretics to reduce blood volume (and therefore pressure), oxygen, and cardiac monitoring. Vasodilators such as calcium channel blockers may be beneficial in some patients to reduce pulmonary artery pressure. Warfarin may be used to prevent clotting. A newer therapy is IV administration of prostacyclin, a vasodilator that may reverse some of the vascular changes but has many serious side effects. Helping the patient into a sitting position or a semi-sitting position may help reduce difficulty in breathing. Bed rest and other comfort measures are helpful in treating fatigue and anxiety.

There is no known preventive measure for this disease. The affected person should have a change in lifestyle to assist with their daily activities. Example is engaging in relaxation exercise as a stress reliever. Maintaining good health habits such as a healthy diet, not smoking and drinking alcoholic beverages, and getting plenty of rest allows the patient to cope up with the daily challenges brought about by this disease.

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