What is Primary Pulmonary Hypertension (Definition)

Primary pulmonary hypertension (PPH) is a rare disease. It consists of high blood pressure in the blood vessels of the lungs.

People with PPH have more muscle than normal in the walls of these blood vessels. That extra muscle makes it harder for the blood to flow through it. As a result, the right side of the heart must work harder to bring blood to the lungs. This extra effort can eventually lead to heart failure.

HPP is a serious condition. It requires medical attention.

Primary Pulmonary Hypertension Causes

The cause of PPH is unknown. There are several factors that can contribute to the development of the disease, including:

• Immune system disease
• Exposure to certain drugs or chemicals
• Genetic defects

Primary Pulmonary Hypertension Risk factors

The following factors increase your chances of developing PPH:

• Women between the age of 30 and 40 years
• Liver cirrhosis
• Portal hypertension
• HIV infection
• Family history of HPP
• Use of appetite suppressants: diet drugs (eg, fenfluramine)
• Cocaine use

Primary Pulmonary Hypertension  Symptoms

The Early symptoms of PPH may be minor. They get worse over time. If you experience any of these symptoms do not assume it is due to PPH. These symptoms may be caused by other less serious health conditions. If you experience any of them, check with your doctor.

• Shortness of breath, especially when active or at rest
• Hyperventilation: abnormally fast and deep breathing
• Fatigue: tiredness
• Progressive weakness
• Fainting periods
• Dizziness
• Cough bleeding
• Cyanosis: bluish tinge to the lips and skin
• Inflammation of the legs and hands
• Chest pain
• Lack of appetite
• Cold hands and feet
• Low blood pressure

A physical examination by your doctor may show:

• Inflammation of the veins in your neck
• Enlarged liver and abdomen inflamed
• A heart murmur: an abnormal sound in the heart

Primary Pulmonary Hypertension Diagnosis

The diagnosis of PPH may be delayed because it is difficult to detect until symptoms worsen. The evaluation must be carried out in a medical center with experience in pulmonary hypertension.

The doctor will ask about your symptoms and medical history. You will have a physical exam. Tests may include:

• Blood test: liver activity, coagulation tests, HIV test, autoantibodies
  Blood gases to determine oxygenation
• Electrocardiogram (ECG): analysis that records the electrical activity of the heart
• Chest x-ray: test that uses radiation to take an image of structures inside the body and can show if the heart is enlarged
• Chest CT scan: a type of x-ray that uses a computer to take pictures of structures inside the chest
• Echocardiogram: test that uses high-frequency sound waves (ultrasound) to examine the size, shape and movement of the heart
• Pulmonary activity tests: noninvasive tests, such as blowing inside a tube, that measure how well your lungs are working
• Cardiac catheterization: detects heart problems and blood supply
• Pulmonary arteriogram: type of radiography that detects blood clots and other obstructions
• Lung scan: a special camera takes images of the lungs, to detect blockages in the blood vessels
  Six-minute walk to determine the degree of shortness of breath, as an indirect measure of the severity of PPH

Primary Pulmonary Hypertension Treatment

There is no cure for PPH. The treatment is used to help relieve and control the symptoms. Ask your doctor what is the best plan for you. Treatment options include:

Medications

• Calcium channel blockers (Nifedipine, diltiazem and amlodipine): to relax the muscles of the blood vessels of the lungs
  Prostacilin (epoprostenol, treprostinil) and synthetic prostacycline (iloprost, cisaprost) : to relax the muscles of the blood vessels of the lungs
  Digoxin: to improve the pumping capacity of the heart
  Anticoagulants: to reduce the likelihood of having blood clots in the lungs
  Diuretics: to reduce the amount of fluid in the body and , in turn, reduce the effort in the heart
  Vasodilators: to help reduce blood pressure in the blood vessels of the lungs
  Endothelin receptor antagonists (Bosentan): to relax the muscles of the blood vessels of the lungs
  Inhibitors of phosphodiesterase 5 (sildenafil): to relax the blood vessels of the lungs

Complementary oxygen 

If breathing difficulties occur, oxygen can be provided. It can be supplied with a mask or with tubes inserted in the nostrils.

Lung transplant or heart-lung transplant

Lungs or hearts with defects are replaced with donor organs. This option is only used in severe cases of PPH.

Primary Pulmonary Hypertension Prevention

There are no guidelines to prevent PPH because the cause is unknown.