Primary Pulmonary Hypertension

Primary pulmonary hypertension is a gradual increase in blood pressure in the pulmonary artery and vein. The pulmonary artery comes out of the heart and branches out to the two lungs. PPH is a condition that is a result of lung issues. The pulmonary arteries typically have lower blood pressure than might be taken in the arm. When the blood pressure rises from constriction it becomes more difficult for the heart to pump blood to the lungs. The stress can cause the right side of the heart to enlarge, eventually causing fluid to build up in the liver and in other places such as the legs. This hypertension (elevated blood pressure) is called primary because the condition is not caused by some other bodily system failure. Secondary pulmonary hypertension, which is much more common, is the result of some contributing factor.

Typically primary pulmonary hypertension or PPH occurs in younger persons and more in females than males. There are a number of causes for this condition such as liver disease, HIV, a certain form of lupus, and a number of other conditions. Additionally sleep apnea can also contribute to this condition because the continued sleep interruption all night long contributes to low blood oxygen levels. Often blood clots and lung problems such as emphysema and COPD are also contributors to PPH. A now banned weight loss drug called Fen/Phen has recently been linked to another cause for PPH. This weight loss drug was very popular in the 1990's but has been off the market since about 1998.

Many people who have developed PPH don't have any symptoms early on, but as the condition worsen, it begins to affect breathing, with the sufferer experiencing shortness of breath that usually heightens when activity is attempted. Additionally, symptoms of primary pulmonary hypertension include a persistent cough, fatigue and/or dizziness. As the condition continues to get worse, fluid may build in the legs causing heaviness in the legs and chest may also accompany this fluid buildup. Upon being examined with a stethoscope, a health practitioner may hear louder than usual heart sounds. Having such a condition is a stark reminder of the fact that we all hang on to life by a thin thread. God has determined that at the end of life, all mankind has an appointment. "And as it is appointed unto man once to die, but after this the judgment." (Hebrews 9 :27)

Often a trained medical person will notice the chest wall is elevated called right ventricular heave. If it is suspected, a number of tests will probably be run to confirm the suspicions. A chest X-ray, elector and echo cardiogram will be run along with other tests to find out if indeed there is primary pulmonary hypertension. An intensive look at the function of the right side of the heart will be conducted. Every effort will be taken to see if there is any kind of obstruction in the pulmonary artery system.

A drug therapy will certainly be one of the main therapies that will be offered by a physician in treating this condition. There is no treatment to stop the progression of the disease but common drugs used for controlling systemic blood pressure are also used for primary pulmonary hypertension. Vasodilators which widen blood vessels may be used or diuretic drugs may be employed to restrict fluid pressure on the heart. But it is often a fishing expedition by the physician to get the right combination of drugs to lower the PPH. It is quite normal that as the disease progresses, there will be the need to recalibrate the drug therapy.

Women between the ages of twenty and forty are the most prominent demographic group affected by primary pulmonary hypertension. Part of this narrow demographic band of females may well be due to their use of the drug Fen/Phen and medical experts say there is a latency of as much as ten years after ending use of the drug before the first symptoms of PPH surface. There has been a rash of television commercials by attorneys from around the country urging those who have used the diet drug and contracting this condition to call and join in lawsuits against the manufacturer. There are others who didn't take the diet drugs of the nineties who may also be susceptible to contracting this condition. Cocaine use, HIV and pregnancy can bring PPH on and in about ten percent of the cases; medical profession thought is that PPH runs in families.

For others who have the condition, research shows that those with Reynaud's disease are more likely to get primary pulmonary hypertension. This is a disease which causes fingers and toes to turn blue when they get cold because their blood vessels appear to be highly sensitive to lower temperatures. No matter what the cause is, it is critical that those who have the condition get immediate medical attention because life expectancies can be lowered dramatically without treatment. Once the diagnosis has been made, the circumstances dictate that the patient locate a physician that has had experience in treating this condition. Since only about a thousand new cases are opened each year across the United States, this is not a run of the mill common occurrence. There are physicians in every state that have treated primary pulmonary hypertension and they can easily be found online on websites that deal with PPH issues.

Pulmonary Hypertension Symptoms

Primary pulmonary hypertension (PPH) is high blood pressure in the pulmonary artery, a condition that can be treated but not cured. This artery is the one that carries oxygen in the blood to the lungs. The disease can be caused from blockages or narrowing of the arteries. When the muscles that are close by tighten up and constrict the arteries it can lead to PPH. Pulmonary hypertension symptoms include dizziness, swelling in the ankles or legs, chest pain, palpitations, fatigue, and fainting. As the disease progresses a person will experience extreme fatigue and weakness, have difficulty breathing, and may have to remain in bed most of the time. A diagnosis can be made by a physician when using several different types of tests. Treatment involves taking medication, oxygen, and for the most severe cases, lung and heart transplantation.

High blood pressure in the pulmonary artery affects the condition of the heart and the lungs. The artery is located in the right ventricle of the heart and when the pressure is high there is a strain put on the organ that can lead to other problems. In addition, the lungs may not receive enough oxygen to operate efficiently. Primary pulmonary hypertension causes changes in the small blood vessels within the lungs. The actual cause of the disease is not completely understood but some conditions that may play a role in the onset are Raynaud's syndrome, cocaine, and HIV. The beginning signs of the condition often include extreme fatigue, difficulty breathing, and fainting spells. Because of the usual slow progression of the disease many people go undiagnosed until the condition becomes much worse. Having symptoms without a diagnosis can become a burden to the person experiencing them. "Cast thy burden upon the LORD, and He shall sustain thee: He shall never suffer the righteous to be moved." (Psalm 55:22)

One of the medications that is used to treat high blood pressure in the lungs and help with the functioning of the heart include calcium channel blockers. Calcium channel blockers cause the arteries to relax thus improving blood flow. Vasodilators are also useful for treating pulmonary hypertension symptoms. Vasodilators are deliverable intravenously helping to lower the pressure within the artery and give the patient the capability of being more active. Anticoagulants are often used because it decreases the bloods ability to clot so the blood flows better through the arteries. Diuretics help to decrease the workload on the heart by reducing the fluid level in the body. Many patients require the use of oxygen when breathing becomes difficult. Before a heart or lung transplant is considered a physician will try medications first. If the patient doesn't respond to meds then transplants become a last resort option.

When the cause of pulmonary hypertension is known it is referred to as secondary pulmonary hypertension (SPH). Some of the conditions that have been known to cause SPH are emphysema, bronchitis, scleroderma, and lupus. Suffering with pulmonary hypertension symptoms may be difficult to diagnose in a person who has other medical conditions simply because the symptoms of SPH may be similar to symptoms he or she is already experiencing. A doctor will normally want to know of any new symptoms that may have surfaced or if any symptoms have worsened between office visits. Three conditions that are all related to the immune system that have been linked to the disease are Raynaud's syndrome, scleroderma, and lupus. In Raynaud's syndrome the patient experiences sensitivity to cold causing their fingers and toes to turn blue. Scleroderma is a connective tissue disorder that affects the patient's skin, blood vessels, and internal organs. Lupus attacks the cells and tissues causing damage to joints, blood vessels, and organs.

Appetite suppressants have been linked to the onset of primary pulmonary hypertension. One drug that has been linked to PPH is no longer on the market but according to medical experts patients who have taken the drug may not have symptoms for several years after the drug has been discontinued. Patients who are trying to lose weight should be very careful to use appetite suppressants that have been proven to be safe. Being overweight can aggravate PPH since extra pounds put more strain on the heart and the blood vessels. Healthy weight loss for those who have the disease should be done slowly by changing lifestyle habits to include eating healthy and moderate exercise. Exercise should only been done under the advice of a physician because patients with PPH have a problem with shortness of breath.

Some of the tests involved with diagnosing PPH usually include electrocardiogram, echocardiogram, chest x-ray, and cardiac catheterization. An electrocardiogram will show the size of the heart to see if it is enlarged. An echocardiogram is an ultrasound of the heart that will show the right ventricle and pulmonary arteries in order to see enlargement and estimate the pressure in the arteries. Primary pulmonary hypertension will show up in a cardiac catheterization because the procedure will measure the pressure on the right side of the heart as well as the blood vessels in the lungs.

Symptoms that should not be ignored with PPH include coughing up blood, breathing faster, racing pulse, not being able to do any activity without shortness of breath, and extreme fatigue. Pulmonary hypertension symptoms normally progress and worsen over time. When this happens it is extremely important that the patient lets a physician know. A physical examination that can reveal a problem with PPH is when there are distended veins in the neck, swelling of the ankles, legs, and hands, and an enlarged liver. When a physician is trying to make a diagnosis he or she will often do it by checking for other conditions simply because the symptoms can mimic other diseases.

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