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Pulmonary Hypertension

What is pulmonary hypertension?

Pulmonary hypertension occurs when high blood pressure affects the arteries in the lungs and the right side of your heart. To start, tiny arteries in your lungs, called pulmonary arteries, and capillaries narrow, become blocked or  are destroyed. Blood flow through your lungs becomes more difficult, and pressure within your lungs’ arteries increases. As the pressure builds, your heart’s lower right chamber (right ventricle) is forced to work harder to pump blood through your lungs. This weakens your heart muscle, eventually causing it to fail.

Pulmonary hypertension, a serious illness, sometimes becomes increasingly worse over time and can be fatal. While not curable, there are treatments available that can help decrease symptoms thus improving the quality of your life.

What are the common symptoms of pulmonary hypertension?

In their early stages, the symptoms of pulmonary hypertension may not be apparent for months and, in some cases, not for years. With the progression of the disease, symptoms worsen. Pulmonary hypertension symptoms include: fatigue; a bluish tinge to your lips and skin; shortness of breath, initially while exercising and eventually while at rest; dizziness or fainting (syncope); chest pain or pressure; swelling in your ankles, legs and eventually in your abdomen; racing pulse or heart palpitations.

What causes pulmonary hypertension?

As pulmonary hypertension causes changes in the cells that line your pulmonary arteries, an unhealthy increase in your blood pressure takes place. These changes bring about the formation of extra tissue, eventually narrowing or even entirely blocking  blood vessels. This makes the arteries narrow and stiff. Blood flows with more difficulty and blood pressure in the pulmonary arteries rises.

Without the identification of an underlying cause for high blood pressure in the lungs, the condition is called idiopathic pulmonary hypertension (IPH). Some IPH patients may have a gene which causes a propensity for developing pulmonary hypertension. But for the majority of idiopathic pulmonary hypertension patients, there is no recognized cause. Secondary pulmonary hypertension is diagnosed when another medical problem causes the hypertension. Secondary pulmonary hypertension is more common than idiopathic pulmonary hypertension.

Causes of secondary pulmonary hypertension include: sleep apnea and other sleep disorders; sickle-cell anemia; blood clots in the lungs; chronic liver disease (cirrhosis ); chronic obstructive pulmonary diseases, such as emphysema; AIDS; connective tissue disorders, such as scleroderma or lupus; heart abnormalities you’re born with; diseases such as pulmonary fibrosis, a condition which causes tissue scarring between the lungs’ air sacs; left-sided heart failure; climbing or hiking to altitudes higher than 8,000 feet without acclimating first; living at altitudes over 8,000 feet above sea level; and the use of certain stimulant drugs, such as cocaine.

How is pulmonary hypertension tested and diagnosed?

Because it is rarely detected in a routine physical examination, pulmonary hypertension is rarely diagnosed early on. Even in cases where the disease is more advanced, many of its more obvious signs and symptoms are similar to those of other lung and heart conditions. In either event, one or more tests to rule out other possible reasons for your condition are available.

The first level of tests used to diagnose pulmonary hypertension include: chest X-rays; blood tests; Doppler echocardiogram to estimate pressures in the lung; and heart catheterization to measure directly the pressure in the right ventricle and the main pulmonary arteries. Additional tests to check the condition of your pulmonary arteries and your lungs include: a perfusion lung scan, a pulmonary function test,  computerized tomography (CT) scan; magnetic resonance imaging (MRI; and an open-lung biopsy.

How is pulmonary hypertension treated?

Identifying and implementing the best treatment for pulmonary hypertension cannot be achieved immediately. Often the treatments are complex and call for extensive follow-up care. Also, your doctor will need to revise your treatment if and when it ceases to be effective. Additionally, when pulmonary hypertension is brought on by another condition, treatment for the underlying cause will be employed first if possible.

Various medications are used to treat pulmonary hypertension. These include: blood vessel dilators (vasodilators);

endothelin receptor antagonists which are meant to reverse the effects of endothelin, a component in the walls of blood vessels that causes them to narrow;  tadalafil  and sildenafil to open the lung’s blood vessels and allow blood to flow through more easily; and high-dose calcium channel blockers. Other medications used stop the narrowing of your blood vessels, are anticoagulants, and act as diuretics.

Sometimes doctors recommend breathing pure oxygen, a treatment known as oxygen therapy, particularly if you live at a high altitude or suffer from sleep apnea. Some pulmonary hypertension patients eventually require oxygen therapy constantly. Surgery is another option for treating some forms of pulmonary hypertension, including a type of open-heart surgery called atrial septostomy.

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