Pulmonary Hypertension

What is pulmonary hypertension?

Pulmonary hypertension (PH) is when there is high blood pressure in the blood vessels going from the heart to the lungs. This is a different disease from hypertension (high blood pressure), which affects your whole body.

Pulmonary hypertension leads to low oxygen in your body. It can cause shortness of breath and fatigue.

Pulmonary arterial hypertension (PAH) is a specific type of pulmonary hypertension that affects the small arteries in the lungs. In PAH, these arteries become narrowed, thickened, or blocked, making it harder for blood to flow through them. This increased resistance causes high blood pressure in the lungs and puts extra strain on the right side of the heart. One form of PAH is called idiopathic pulmonary arterial hypertension (IPAH), which means the exact cause is unknown. IPAH is a rare condition that can affect people of any age but is more common in women. While the underlying cause of IPAH remains a mystery, researchers have identified certain genetic factors that may play a role in its development. Both PAH and IPAH are serious conditions that require specialized care and treatment to manage symptoms and slow disease progression.

High blood pressure in the lungs can be caused by a range of diseases and conditions. They include problems with the heart pumping blood, blood clots, low oxygen in the lungs (which causes blood vessels to constrict), or from thickening of the arteries (called pulmonary arterial hypertension or PAH).

It can get worse over time. However, if you can diagnose its underlying cause and treat it, then symptoms can improve, or even disappear.

Most common PH symptoms

Low oxygen levels in the blood cause general fatigue and shortness of breath when you’re physically active. It can also lead to lightheadedness and fainting.

When the heart isn’t receiving enough oxygen, you may feel pain in the chest.

Pulmonary hypertension increases pressure in the blood vessels between the heart and the lungs. Over time, this stresses the heart, making it harder for the heart to pump. It may lead to other symptoms, such as fluid build-up in the legs and belly and a lack of appetite.

Main symptoms

• Shortness of breath; difficulty breathing. At first, you may notice it during physical activity, such as running. But it may start to happen when walking around the house or even at rest.
• Fatigue

As it progresses, symptoms can include:

• Lightheadedness or fainting, especially with activity
• Chest pain with physical activity
• Swelling in the legs and belly
• Weight gain (from the fluid build-up)
• Loss of appetite

Risk factors for pulmonary hypertension

A range of conditions, each with their own risk factors, can cause pulmonary hypertension. But these are some common risk factors.

• Family history of pulmonary arterial hypertension (PAH)
• Obesity combined with obstructive sleep apnea
• Female
• Pregnancy
• Living at a high altitude for years can increase your risk
• Having other diseases, including congenital heart disease, lung disease, liver disease, scleroderma, and lupus
• Certain drugs, such as methamphetamines

How serious is pulmonary hypertension?

If you have new or sudden shortness of breath or chest pain, go to the ER. It could be a sign of something more serious.

If you have been experiencing shortness of breath or difficulty breathing during physical activity for a few weeks or months, see your doctor.

The symptoms of pulmonary hypertension (especially fatigue and feeling lightheaded) can seem vague and may be caused by other problems. It is important to see your doctor.

What is the main cause of pulmonary hypertension?

Pulmonary hypertension is a form of high blood pressure in the vessels that connect the heart to the lungs. There are many reasons that it may develop.

• Left heart disease. This is the most common cause of pulmonary hypertension in the United States. Blood travels from the right side of the heart to the lungs and out to the left side of the heart and then to the body. Problems with the left heart such as a weak squeeze can cause blood to back up, leading to high blood pressure in the lungs.
• PAH (thickening of the vessels). Blood pressure rises as blood tries to move through the narrowed vessels.
• Lung disease and low oxygen
• Blockage of the pulmonary (lung) artery

What is the best treatment for pulmonary hypertension?

How pulmonary hypertension is treated depends on the cause. Whether it’s an underlying heart disease, lung disease, low oxygen, blood clots, or bodywide diseases. For instance, if it’s from blood clots, a blood thinner may be recommended. If you have sleep apnea, that would be addressed.

The one condition that is treated directly with specific medications is PAH. They include calcium channel blockers, endothelin receptor antagonists, prostacyclin pathway agonists and nitric oxide enhancers. are a group of medications that dilate blood vessels. The medications may be pills, inhaled, or intravenous.

Pulmonary hypertension may go away if the underlying cause can be treated. For example, if the cause is weak pumping of the left side of the heart (called heart failure), treating that can improve pulmonary hypertension.

Many causes of pulmonary hypertension are chronic conditions that require long-term care. If you have severe pulmonary hypertension or it is not easily reversible by treating the underlying issue, follow up with a cardiologist (heart specialist) or pulmonologist (lung specialist).