What is Pulmonary Hypertension?

Pulmonary hypertension is a type of high blood pressure that affects the arteries in the lungs and on the left side of the heart.

There are multiple forms of pulmonary hypertension, some of which can be serious and even fatal. Although some forms of pulmonary hypertension are not curable, treatment can help relieve symptoms and improve quality of life.

 

Symptoms and Complications

In its early stages, signs and symptoms of pulmonary hypertension may not be noticeable for months or even years. As the condition progresses, symptoms may worsen and may include:

• Shortness of breath, first experienced while exercising but later felt even while at rest
• Fatigue
• Dizziness or fainting spells
• Chest pressure or pain
• Swelling in the ankles, legs and eventually the abdomen
• Bluish color to the lips and skin
• Racing pulse or heart palpitations

Pulmonary hypertension can lead to several complications:

• Right-sided heart enlargement and heart failure: Also called cor pulmonale, this is a condition in which the heart’s right ventricle becomes enlarged and has to pump harder than normal to move blood around. The heart will try to compensate by thickening its walls and expanding the right ventricle, but this only works temporarily—eventually the ventricle will fail.
• Blood clots: Clots help you stop bleeding after an injury, but they can sometimes form unnecessarily. Blood clots can lead to a type of pulmonary hypertension that is usually reversible with time and treatment.
• Arrhythmia: Irregular heartbeats from the upper or lower chambers of the heart can result from pulmonary hypertension. These can lead to palpitations, dizziness, fainting and even fatalities in some cases.
• Bleeding: Pulmonary hypertension can lead to bleeding into the lungs and coughing up blood, which can also be a fatal complication

Causes and Risk Factors

In people with pulmonary hypertension, the rise in blood pressure is brought on by changes in cells that line the pulmonary artery. It is classified into five groups, based on specific causes:

• Group I: Pulmonary arterial hypertension—When the cause is unknown or caused by a specific gene mutation, certain drugs, congenital heart disease or other conditions.
• Group 2: Pulmonary hypertension caused by left-side heart disease—Caused by left-sided valvular heart disease or failure of the lower left heart ventricle.
• Group 3: Pulmonary hypertension caused by lung disease—Caused by chronic obstructive pulmonary disease, lung disease, sleep apnea or other sleep disorders, or long-term high altitude exposure.
• Group 4: Pulmonary hypertension caused by chronic blood clots—Caused by chronic clots in the lungs.
• Group 5: Pulmonary hypertension associated with other conditions—Cases in this group often have unclear causes. Examples of conditions associated with pulmonary hypertension include blood disorders, disorders that affect multiple organs, metabolic disorders and tumors pressing against pulmonary arteries

In addition, a condition called Eisenmenger syndrome, a type of congenital heart disease can cause pulmonary hypertension. It’s most commonly caused by a hole in the heart

Your risk of developing pulmonary hypertension may be increased if:

• You’re a young adult
• You’re overweight
• You have a family history of the condition
• You have a condition that increases risk, as listed above
• You use illegal drugs such as cocaine
• You take certain medications to suppress the appetite
• You have an existing risk, such as a family history or living at high altitude

 

Diagnosis and Treatment

Pulmonary hypertension can be tough to diagnose because it’s often not found during basic physical exams. Your doctor will examine your physical history and may run one or more tests to check for pulmonary hypertension.

Although pulmonary hypertension cannot be cured, you can manage the condition and improve symptoms. Treatments may take some time to find and may require follow-up appointments. If the hypertension is caused by an underlying condition, your doctor will treat this underlying cause if possible.

Medications that may be used for treatment include:

• Vasodilators: To help open narrowed blood vessels
• Endothelin receptor antagonists: To remove the effects of endothelin, a substance that causes blood vessel walls to narrow
• Sildenafil and tadalafil: To open blood vessels
• High-dose calcium channel blockers: To help relax muscles in blood vessel walls
• SGC stimulators: Relax the arteries and lower pressure
• Anticoagulants: To help prevent the formation of blood clots in affected areas
• Digoxin: Helps the heart beat stronger
• Diuretics: Water pills that help eliminate excess fluids
• Oxygen: Common for people who live at high altitude or have sleep apnea

In some cases, surgery might be needed for pulmonary hypertension. If so, options include:

• Atrial septostomy: Open-heart surgery in which an opening is created between the chambers of your heart to relieve pressure on the left side
• Transplantation: A lung or heart-lung transplant might be an option, though there are risks here, including infection

 

Sources:

“Pulmonary hypertension.” The Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/pulmonary-hypertension/symptoms-causes/syc-20350697

“About Pulmonary Hypertension.” Pulmonary Hypertension Association. https://phassociation.org/patients/aboutph/