| Risk Factors
Pulmonary hypertension high blood pressure in the blood vessels of the lungs. Pressure helps move blood through blood vessels.
Normal pressure for blood flow through the lungs is one-sixth of the pressure for the rest of the body. Pressure may rise for any number of reasons. It creates a potentially serious condition. The right side of the heart needs to pump harder to move blood against the pressure. If left untreated the right side of the heart can eventually fail.
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Pulmonary hypertension can happen for many reasons.
- Since blood vessels in the lungs are very sensitive to oxygen, lower levels of oxygen in the body or lungs may narrow the vessels. Higher pressure will be needed to push blood through the narrow blood vessels.
- Pulmonary pressure will also increase when there are abnormalities of the lungs, such as fluid or scarring, heart, or blood vessels.
The increase may be caused by:
Chronic obstructive pulmonary disease (COPD) is the most common cause. This condition causes destruction of lung tissue. This decreases the number of blood vessels. It also lowers the amount of oxygen available.
COPD is a combination of chronic bronchitis
Heart abnormalities can result in extra blood in the lungs. Some are conditions people are born with. Others may develop over time. Some of these conditions are:
- Septal defects
(holes between the right and left sides of the heart, present at birth)
- Tight (stenotic) or leaky heart valves
Lung diseases that scar the lungs:
(systemic sclerosis)—stiffening blood vessels, effectively preventing them from opening which raises the pressure
- Patients who have large sections of lung removed surgically
Systemic lupus erythematous
obstructive sleep apnea
breathing stops at times through the night. This lowers available oxygen. As a result, pulmonary blood pressure will also increase.
Muscle weakness can make it difficult to breathe. This will lower the available oxygen. This weakness is common in neuromuscular disorders such as:
- Mountain climbers all develop the condition. It is the natural result of breathing thin air. This is one cause of high altitude sickness.
is a build up of blood clots in the lungs. These clots plug up the blood vessels.
- A type of chest wall deformity is known as pectus excavatum. Rarely, severe scarring of the chest wall can prevent chest expansion. It has the same effect as scarring of the lungs themselves.
- Idiopathic pulmonary arterial hypertension is due to defects in the arteries of the lungs. There is no known cause. It is likely due to genetic factors.
Other factors and conditions that can create pulmonary hypertension include:
- Exposure to certain substances, such as cocaine and amphetamines
- Liver disease
Factors that may increase your chance of pulmonary hypertension include:
or other chronic lung disease
- Recurring pulmonary emboli
- Obstructive sleep apnea
- Low thyroid (myxedema)
- Certain congenital and valvular heart conditions
- Muscle weakness diseases
- Home at high altitude (over 10,000 feet)
- Pectus excavatum or other severe chest deformity, such as kyphoscoliosis
Pulmonary hypertension may cause:
- Progressive shortness of breath
- Chronic cough
- Chronic fatigue
- Fainting spells
- Ankle swelling from fluid retention
- Chest pain—angina
Your doctor will ask about your symptoms and medical history. A physical exam will be done. You may be referred to a specialist in heart or lung diseases. A cardiologist focuses on the heart. A pulmonologist focuses on the lungs.
Tests may include the following:
Most cases of pulmonary hypertension are due to another disease. In this case the underlying cause is treated.
For some, no treatable cause is found. In either case, there are several medications that might be of benefit. Talk with your doctor about the best plan for you. Treatment options include the following:
Pulmonary hypertension may be treated with:
- Calcium channel blockers
- Phosphodiesterase inhibitors
- Endothelin receptor blockers
A lung transplant may be necessary if the disease has progressed or is advanced. It is generally not considered unless other treatment methods fail.
To help reduce your chances of getting pulmonary hypertension, take the following steps:
- Maintain proper weight
- Treat conditions that can lead to pulmonary hypertension
Pulmonary hypertension classification and treatment. EBSCO DynaMed Plus website. Available at: http://www.dynamed.com/topics/dmp~AN~T905845/Pulmonary-hypertension-classification-and-treatment. Updated May 27, 2016. Accessed September 29, 2016.
Rubin LJ, Badesch DB. Evaluation and management of the patient with pulmonary arterial hypertension.
Annals of Internal Medicine. 2005;143(4):282-292.
Last reviewed September 2016 by
Michael J. Fucci, DO, FACC
Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.
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