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Old 02-19-2009, 08:16 PM
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Thumbs up Pulmonary Hypertension: Causes, Symptoms, Diagnosis, and Treatment ...

Pulmonary Hypertension : Causes, Symptoms, Diagnosis, and Treatment


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What are pulmonary arteries?

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The human body has two major sets of blood vessels that distribute blood from the heart to the body. One set pumps blood from the right heart to the lungs and the other from the left heart to the rest of the body.

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  • The portion of the circulation that distributes oxygen-rich blood from the left side of the heart, throughout the body, is referred to as the systemic circulation.

  • The blood then returns from the body to the right side of the heart and passes through the lungs to replenish oxygen.

  • It then returns to the left side of the heart for another round through the systemic circulation.

  • The portion of the circulation that distributes the blood from the right side of the heart to the lungs is referred to as the pulmonary (lung) circulation.

  • The pulmonary arteries are the major blood vessels that carry blood from the right side of the heart to the lungs.
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The left ventricle of the heart pumps oxygenated blood (blood that has been reloaded with oxygen in the lungs) from the lungs into the systemic circulation. When a doctor or a nurse measures the blood pressure on a person's arm, he/she is measuring the pressures in the systemic circulation. When these pressures are abnormally high, the person is diagnosed as having high blood pressure (hypertension).
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What is pulmonary hypertension?

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The right ventricle pumps blood returning from the body into the pulmonary arteries to the lungs to receive oxygen. The pressures in the lung arteries (pulmonary arteries) are normally significantly lower than the pressures in the systemic circulation. When pressure in the pulmonary circulation becomes abnormally elevated, it is referred to as pulmonary hypertension, pulmonary artery hypertension, or PAH.

Pulmonary hypertension generally results from constriction, or stiffening, of the pulmonary arteries that supply blood to the lungs. Consequently, it becomes more difficult for the heart to pump blood forward through the lungs. This stress on the heart leads to enlargement of the right heart and eventually fluid can build up in the liver and other tissues, such as the in the legs.

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What are primary and secondary pulmonary hypertension?

In the conventional classification, pulmonary hypertension, which is also called pulmonary arterial hypertension, is divided into two main categories:

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1) primary pulmonary hypertension (not caused by any other disease or condition); and

2) secondary pulmonary hypertension (caused by another underlying condition). Secondary pulmonary hypertension is much more common than primary pulmonary hypertension.
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A newer classification of this condition is based on the main underlying cause of pulmonary hypertension. This system classifies the condition based on whether it is due to:

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  • left sided heart disease,
  • lung disease,
  • blood clots,
  • constriction of arteries due to any reasons (including primary pulmonary hypertension), and
  • obstruction from outside of blood vessel (for example from diseases of the chest wall compressing the blood vessels).


Causes

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Chambers and valves of the heart



The heart has two upper chambers (the right and left atria) and two lower chambers (the right and left ventricles).

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Pulmonary hypertension


When the tiny arteries in your lungs become narrow or blocked, or destroyed as in emphysema, there's increased resistance to blood flow through the lungs. This, in turn, causes increased pressure within these arteries, a condition known as pulmonary hypertension.
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Another view (of picture in Media File 1) of gross pathology on a patient who died from severe pulmonary hypertension secondary to persistent patent ductus arteriosus.

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Your heart has two upper and two lower chambers. Each time blood passes through your heart, the lower right chamber (right ventricle) pumps blood to your lungs through a large blood vessel (pulmonary artery). In your lungs, the blood releases carbon dioxide and picks up oxygen. The oxygen-rich blood then flows through the pulmonary veins to the left side of your heart. From there, it's pumped by the left ventricle to the rest of your body through another large blood vessel, the aorta.

Ordinarily, the blood flows easily through the vessels in your lungs, so your blood pressure is usually much lower in your lungs. With pulmonary hypertension, the rise in blood pressure is the end result of a process that begins with changes in the cells that line your lungs' arteries. These changes cause the formation of extra tissue that eventually narrows or completely blocks the blood vessels. Scarring (fibrosis) usually also occurs, making the arteries stiff and narrow. This makes it harder for blood to flow, raising the pressure in the pulmonary arteries.
Idiopathic pulmonary hypertension

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When an underlying cause for high blood pressure in the lungs can't be found, the condition is called idiopathic pulmonary hypertension (IPH) or primary pulmonary hypertension (PPH).

Some people with IPH have an inherited predisposition for the disease, which may be confirmed by a genetic test. But in most people with idiopathic pulmonary hypertension, there is no recognized cause of their pulmonary hypertension.
Secondary pulmonary hypertension

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Pulmonary hypertension resulting directly from another medical problem is called secondary pulmonary hypertension. This type of pulmonary hypertension is more common than idiopathic pulmonary hypertension.
Medical conditions that may lead to secondary pulmonary hypertension include:
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• Blood clots in the lungs (pulmonary emboli)
• Chronic obstructive pulmonary diseases, such as emphysema
• Connective tissue disorders, such as scleroderma
• Sleep apnea
• Congenital heart disease
• Sickle cell anemia
• Chronic liver disease (cirrhosis)
• AIDS
• Lupus
• Lung diseases such as pulmonary fibrosis, a condition that causes scarring in the tissue between the lungs' air sacs (interstitium)
• Left-sided heart failure
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If you live above an altitude of 8,000 feet you can develop pulmonary hypertension as a result of low blood oxygen (hypoxemia) or pulmonary edema (a condition in which the air sacs in the lungs fill with fluid instead of air), which constricts the small pulmonary arteries. People who climb to high elevations without first becoming acclimated are especially at risk of developing a temporary form of pulmonary hypertension known as transient reversible pulmonary hypertension.

Although the exact cause of secondary pulmonary hypertension isn't known, it's possible that some who develop the disorder are especially sensitive to substances that cause the blood vessels to constrict. Cocaine and the diet drug fenfluramine, which was withdrawn from the market in 1997, are two of the substances that may contribute to IPH in some people.
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Pulmonary hypertension

Pulmonary hypertension is the narrowing of the pulmonary arterioles within the lung. The narrowing of the arteries creates resistance and an increased work load for the heart. The heart becomes enlarged from pumping blood against the resistance. Some symptoms include chest pain, weakness, shortness of breath, and fatigue. The goal of treatment is control of the symptoms, although the disease usually develops into congestive heart failure.
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