About pulmonary hypertension, secondary

What is pulmonary hypertension, secondary?

General Discussion

Secondary pulmonary hypertension is a disorder of the blood vessels in the lungs. It usually is the result of other lung diseases or related diseases in other organs. Affected individuals have high blood pressure (hypertension) of the main artery of the lungs (pulmonary artery). The disorder is characterized by breathing difficulties, especially after exertion.

What are the symptoms for pulmonary hypertension, secondary?

Pulmonary hypertension signs and symptoms include:

What are the causes for pulmonary hypertension, secondary?

Chambers and valves of the heart Open pop-up dialog box Close Chambers and valves of the heart Chambers and valves of the heart

A typical heart has two upper and two lower chambers. The upper chambers, the right and left atria, receive incoming blood. The lower chambers, the more muscular right and left ventricles, pump blood out of the heart. The heart valves are gates at the chamber openings. They keep blood flowing in the right direction.

The typical heart has two upper chambers (atria) and two lower chambers (ventricles). Each time blood passes through the heart, the lower right chamber (right ventricle) pumps blood to the lungs through a large blood vessel (pulmonary artery).

In the lungs, the blood releases carbon dioxide and picks up oxygen. The blood typically flows easily through blood vessels in the lungs (pulmonary arteries, capillaries and veins) to the left side of the heart.

However, changes in the cells that line the pulmonary arteries can cause the walls of the arteries to become stiff, swollen and thick. These changes may slow down or block blood flow through the lungs, causing pulmonary hypertension.

Pulmonary hypertension is classified into five groups, depending on the cause.

Group 1: Pulmonary arterial hypertension (PAH)

Causes include:

  • Unknown cause (idiopathic pulmonary arterial hypertension)
  • Changes in a gene passed down through families (heritable pulmonary arterial hypertension)
  • Use of certain drugs or illegal substances
  • Heart problems present at birth (congenital heart disease)
  • Other conditions such as HIV infection, chronic liver disease (cirrhosis) and connective tissue disorders (scleroderma, lupus, others)
Group 2: Pulmonary hypertension caused by left-sided heart disease

Causes include:

  • Left-sided heart valve disease such as mitral valve or aortic valve disease
  • Failure of the lower left heart chamber (left ventricle)
Group 3: Pulmonary hypertension caused by lung disease

Causes include:

  • Chronic obstructive pulmonary disease (COPD)
  • Scarring of the tissue between the lung's air sacs (pulmonary fibrosis)
  • Obstructive sleep apnea
  • Long-term exposure to high altitudes in people who may be at higher risk of pulmonary hypertension
Group 4: Pulmonary hypertension caused by chronic blood clots

Causes include:

  • Chronic blood clots in the lungs (pulmonary emboli)
  • Other clotting disorders
Group 5: Pulmonary hypertension triggered by other health conditions

Causes include:

  • Blood disorders, including polycythemia vera and essential thrombocythemia
  • Inflammatory disorders such as sarcoidosis and vasculitis
  • Metabolic disorders, including glycogen storage disease
  • Kidney disease
  • Tumors pressing against pulmonary arteries
Eisenmenger syndrome and pulmonary hypertension

Eisenmenger syndrome is a type of congenital heart disease that causes pulmonary hypertension. It's most commonly caused by a large hole in the heart between the two lower heart chambers (ventricles), called a ventricular septal defect.

This hole in the heart causes blood to flow incorrectly in the heart. Oxygen-carrying blood (red blood) mixes with oxygen-poor blood (blue blood). The blood then returns to the lungs — instead of going to the rest of the body — increasing the pressure in the pulmonary arteries and causing pulmonary hypertension.

What are the treatments for pulmonary hypertension, secondary?

There's no cure for pulmonary hypertension, but treatment is available to help improve signs and symptoms and slow the progress of the disease.

It often takes some time to find the most appropriate treatment for pulmonary hypertension. The treatments are often complex and require extensive follow-up care.

When pulmonary hypertension is caused by another condition, treatment is given for the underlying cause whenever possible.


Medications are available to help improve symptoms of pulmonary hypertension and to slow the progression of the disease.

Medications that may be prescribed to treat pulmonary hypertension symptoms or complications include:

  • Blood vessel dilators (vasodilators). This type of medicine relaxes and opens narrowed blood vessels, improving blood flow. Vasodilators may be taken by mouth, inhaled, injected or given by IV infusion. A commonly prescribed vasodilator for pulmonary hypertension is epoprostenol (Flolan, Veletri).

    This drug continuously flows through an IV attached to a small pump, which is worn in a pack on the belt or shoulder. Potential side effects of epoprostenol include jaw pain, nausea, diarrhea, leg cramps, and pain and infection at the IV site.

    Other types of vasodilators, including treprostinil (Tyvaso, Remodulin, Orenitram), can be inhaled, injected or taken by mouth. The drug iloprost (Ventavis) is given while breathing in through a nebulizer, a machine that vaporizes the medication.

    Side effects associated with treprostinil include chest pain, often with headache and nausea, and breathlessness. Possible side effects of iloprost include headache, nausea and diarrhea.

  • Guanylate cyclase (GSC) stimulators. This type of medicine increases nitric oxide in the body, which relaxes the pulmonary arteries and lowers pressure in the lungs. GSC stimulators include riociguat (Adempas). Side effects include nausea, dizziness and fainting. You should not take GSC stimulators if you're pregnant.
  • Medications to widen blood vessels. Medications called endothelin receptor antagonists reverse the effect of a substance in the walls of blood vessels that causes them to narrow. Such drugs include bosentan (Tracleer), macitentan (Opsumit) and ambrisentan (Letairis). They may improve energy level and symptoms. However, they can damage the liver. Monthly blood tests may be done to check liver function. Don't take these drugs during pregnancy.
  • Medications to increase blood flow. Drugs called phosphodiesterase 5 (PDE5) inhibitors may be used to increase blood flow through the lungs. These medicines are also used to treat erectile dysfunction. They include sildenafil (Revatio, Viagra) and tadalafil (Adcirca, Cialis, Alyq).
  • High-dose calcium channel blockers. These drugs help relax the muscles in the walls of blood vessels. They include amlodipine (Norvasc), diltiazem (Cardizem, Tiazac, others) and nifedipine (Procardia). Although calcium channel blockers can be effective, only a small number of people with pulmonary hypertension improve while taking them.
  • Blood thinners (anticoagulants). Warfarin (Jantoven) may be prescribed to help prevent blood clots. Blood thinners increase the risk of bleeding, especially in those who are having surgery or an invasive procedure. If you take blood thinners, you'll need occasional blood tests to see if the medicine is working as it should. Don't stop taking blood thinners without first talking to your care provider.

    Many other drugs, herbal supplements and foods can interact with warfarin. Always tell your provider about your diet and all the medications you take, including those bought without a prescription.

  • Digoxin (Lanoxin). This medicine helps the heart beat stronger and pump more blood. It can help control irregular heartbeats (arrhythmias).
  • Water pills (diuretics). These medications help the kidneys remove excess fluid from the body. This reduces the amount of work the heart has to do. Diuretics may also be used to reduce fluid buildup in the lungs, legs and abdomen.
  • Oxygen therapy. Breathing pure oxygen is sometimes recommended as a treatment for pulmonary hypertension, especially for those who live at a high altitude or have sleep apnea. Continuous oxygen therapy may be needed.

Surgery and other procedures

If medications do not help control the signs and symptoms of pulmonary hypertension, surgery may be recommended. Surgeries to treat pulmonary hypertension include:

  • Atrial septostomy. This open-heart surgery may be recommended if medications don't control pulmonary hypertension signs and symptoms. In an atrial septostomy, a surgeon creates an opening between the upper left and right chambers of the heart (atria) to relieve the pressure on the right side of the heart. Potential complications include heart rhythm problems (arrhythmias).
  • Lung or heart-lung transplant. Sometimes, a lung or heart-lung transplant may be recommended, especially for younger people who have idiopathic pulmonary arterial hypertension.

    Major risks of any type of transplantation include rejection of the transplanted organ and serious infection. Immunosuppressant drugs must be taken for life to help reduce the chance of rejection.

What are the risk factors for pulmonary hypertension, secondary?

Pulmonary hypertension is more often diagnosed in people ages 30 to 60. Growing older can increase the risk of developing Group 1 pulmonary hypertension, called pulmonary arterial hypertension (PAH). However, PAH from an unknown cause (idiopathic PAH) is more common in younger adults.

Other things that can raise the risk of pulmonary hypertension include:

  • A family history of the condition
  • Being overweight
  • Blood-clotting disorders or a family history of blood clots in the lungs
  • Exposure to asbestos
  • Congenital heart disease
  • Living at a high altitude
  • Use of certain drugs, including some weight-loss medicines and illegal drugs such as cocaine or methamphetamine
  • Use of selective serotonin reuptake inhibitors (SSRIs), used to treat depression and anxiety

Is there a cure/medications for pulmonary hypertension, secondary?

There is no cure or medication that can be used to treat pulmonary hypertension, secondary. The only treatment available is surgery, and even then, it's not guaranteed to work.

  • Pulmonary hypertension occurs when the blood vessels in your lungs get narrow and hardened. This makes it difficult for your heart to pump blood through your body and can result in serious complications, including death.
  • There are two forms of pulmonary hypertension: primary and secondary. Primary pulmonary hypertension is caused by other conditions, such as high blood pressure or an infection like HIV/AIDS, while secondary pulmonary hypertension is caused by another condition that affects the lungs, such as COPD (chronic obstructive pulmonary disease).
  • Beta-blockers
  • Calcium channel blockers
  • Diuretics (water pills)
  • Angiotensin receptor blockers
  • Vasodilators include Nitric oxide donors (e.g., nitroglycerin), Alpha-adrenergic agonists (e.g., epoprostenol).
  • Endothelin receptor blockers include Endothelin receptor agonists (e.g., bosentan).
Shortness of breath,Rapid heartbeat (tachycardia),Fatigue,Chest pain,Heart failure
Heart failure,Pulmonary embolism (blood clots in the lungs),Obstructive sleep apnea, which is a condition in which the person has trouble breathing while they're sleeping,Uncontrolled high blood pressure
Amlodipine (Norvasc), Diltiazem (Cardizem, Tiazac, others),Nifedipine (Procardia)

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