Primary Pulmonary Hypertension

(Pulmonary hypertension occurs when pulmonary artery pressure (PAP) rises above normal and isn't attributable to the effects of aging or altitude. There is no definitive set of values used to diagnose pulmonary hypertension, but the National Institutes of Health requires that the resting mean PAP measures 25 mm Hg or more.

Primary, or idiopathic, pulmonary hypertension is rare, occurring most of ten in women between the ages of 20 and 40; pregnant women have the highest mortality. Secondary pulmonary hypertension results from existing cardiac or pulmonary disease. The prognosis depends on the severity of the underlying disorder.

Causes

Many things can cause pulmonary hypertension. However, sometimes the cause is not known. When the cause of pulmonary hypertension isn't known, the disorder is called "primary" pulmonary hypertension.

"Secondary" pulmonary hypertension has a known cause. Common causes are breathing problems called emphysema and chronic bronchitis. Other causes are congestive heart failure, birth defects in the heart, chronic pulmonary thromboembolism (old blood clots in the pulmonary arteries), human immunodeficiency virus (HIV) infection, and certain medicines.

Both primary and secondary pulmonary hypertension are usually permanent conditions, although treatment can improve symptoms and long-term outcomes in some people.

Signs and symptoms

The following are the most common symptoms for pulmonary hypertension. However, each individual may experience symptoms differently. Symptoms may include:

• fatigue
• difficulty in breathing (dyspnea)
• dizziness
• fainting spells (syncope)
• swelling in the ankles or legs (edema)
• bluish lips and skin (cyanosis)
• chest pain (angina)
• racing pulse
• trouble getting enough air
• palpitations, strong throbbing sensations brought on by increased heart rate

Diagnosis

Characteristic diagnostic findings in patients with pulmonary hypertension include the following:

• Auscultation
• Pulmonary fimction tests
• Arterial blood gas (ABG) analysis
• Electrocardiography
• Pulmonary angiography

Treatment

Appropriate treatment usually includes oxygen therapy to decrease hypoxemia and resulting pulmonary vascular resistance. For patients with right heart failure, treatment also includes fluid restriction, digitalis glycosides to increase cardiac output, and diuretics to decrease intravascular volume and extravascular fluid accumulation. An important goal of treatment is correction of the underlying cause.

In some cases, a lung or heart-lung transplant may be an option, especially for younger people who have primary pulmonary hypertension. The single-lung transplant is the most common transplant used for people with PPH. It has fewer complications than do double-lung or heart-lung transplants and often leads to considerable improvement in the functioning of the right ventricle. Major risks of any type of transplantation include rejection of the transplanted organ and serious infection, and you must take immunosuppressant drugs for life to help reduce the chance of rejection.

Prevention

• Stay as active as possible. Even the mildest forms of activity may be too exhausting for some people with pulmonary hypertension. For others, moderate exercise such as walking may be of great benefit, and using oxygen during exercise may be especially helpful. Your doctor can help you plan an appropriate exercise program.
• Get plenty of rest