What are the first symptoms of pulmonary hypertension?
The first symptom of pulmonary hypertension is shortness of breath during your daily activities. These may include climbing stairs or grocery shopping. You may also feel short of breath when you exercise.
At the start of pulmonary hypertension, you may not have any symptoms. When you do start to notice symptoms, they may be mild. But PH symptoms get worse over time, making it harder for you to do your usual activities.
What are the later symptoms of pulmonary hypertension?
As PH progresses, you’ll feel shortness of breath even when you’re not moving around. Other symptoms include:
- Bluish color on your skin or lips.
- Chest pain or pressure.
- Dizziness or fainting.
- Fatigue.
- Feeling less hungry than usual.
- Pain in the upper right side of your tummy.
- Racing heartbeat.
- Swelling (edema) in your ankles, legs or tummy.
PH symptoms make it hard for you to exercise or carry out your normal daily routine.
What are the stages of pulmonary hypertension?
There are four main stages of pulmonary hypertension. The World Health Organization (WHO) calls these “functional classes.” They’re based on the symptoms you feel and refer to how well you can carry out your daily activities. As PH gets worse, the symptoms become more noticeable and more disruptive to your daily life.
- Class 1: You don’t have any symptoms.
- Class 2: You don’t have symptoms when you’re resting. But you feel some discomfort or shortness of breath during some routine activities. These include household chores and climbing stairs.
- Class 3: You may still feel fine when you’re resting. But it’s now much harder to do normal tasks because you feel tired or short of breath.
- Class 4: You have symptoms even when you’re resting. The symptoms get worse when you try to do any normal task.
What causes pulmonary hypertension?
Causes of pulmonary hypertension vary widely depending on the type of PH you have. They include a range of diseases and underlying conditions as well as environmental exposures (toxins and drugs).
Group 1 PH due to pulmonary arterial hypertension (PAH)
There are many causes of pulmonary arterial hypertension. They include:
- Congenital heart disease.
- Diet pills like “fen-phen” (which can cause PAH years after use).
- Genetic mutations.
- Glycogen storage diseases.
- HIV.
- Liver disease.
- Lupus.
- Portal hypertension.
- Pulmonary capillary hemangiomatosis.
- Pulmonary veno-occlusive disease.
- Schistosomiasis.
- Scleroderma.
- Recreational drugs like methamphetamine.
Some people develop PAH without any clear cause. These cases are known as “idiopathic.”
Group 2 PH due to left-sided heart disease
Heart problems are a common cause of pulmonary hypertension. Because the left and right sides of your heart work together, a problem with the left side also affects the right side. The right side of your heart is responsible for pumping blood into your pulmonary arteries. So, left-sided heart problems cause a chain reaction that affects the rest of your heart, your pulmonary arteries and your lungs.
Left-sided heart problems that can cause pulmonary hypertension include:
- Aortic valve disease. Your aortic valve connects your left ventricle (your heart’s main pumping chamber) to your aorta. Your aorta is the largest blood vessel in your body, and it’s responsible for sending oxygen-rich blood out to the rest of your body. With aortic valve disease, your valve may be leaky (regurgitation) or narrow (stenosis). Other problems like congenital heart disease and endocarditis can also affect your aortic valve.
- Left-sided heart failure. This means the left side of your heart can’t pump blood as it should. Your left ventricle may be too weak, or it may be too stiff. Either way, not enough oxygen-rich blood can reach your body’s organs and tissues. Over time, left-sided heart failure can lead to right-sided heart failure and other complications.
- Left ventricular hypertrophy. This is a thickening of the muscular walls of your left ventricle that makes your heart less efficient at pumping blood. It’s usually caused by long-term hypertension or other heart problems like arrhythmias.
- Mitral valve disease. Your mitral valve is the door that lets blood flow from your left atrium to your left ventricle. It may become too stretchy (prolapse), leading to regurgitation. It may also become too narrow. Over time, mitral valve disease strains and damages your heart.
Group 3 PH due to lung disease or hypoxia
Lung problems are another common cause of pulmonary hypertension. Some people with left-sided heart disease also have lung disease or a lack of oxygen (hypoxia).
Lung issues that can cause pulmonary hypertension include:
- Chronic obstructive pulmonary disease (COPD). This is a group of diseases that includes chronic bronchitis and emphysema. COPD makes it harder for you to breathe.
- Interstitial lung disease. This means you have fibrosis, or scarring, in your lung tissue. The scarring leads to breathing problems and coughing.
- Obstructive sleep apnea. This condition causes your airway to become partly or completely blocked, over and over again, as you sleep. As a result, less oxygen reaches your organs.
Group 4 PH due to blockages in your lungs
This form of PH is usually caused by chronic thromboembolic pulmonary hypertension (CTEPH). CTEPH is a condition that involves blood clots and scarring in the arteries in your lungs.
A pulmonary embolism is a blood clot that travels from somewhere else in your body and gets stuck in a blood vessel in your lungs. These blood clots are often treatable, but they may leave behind scar tissue.
These scars can make it harder for blood to flow through your pulmonary arteries. As a result, the blood pressure in your pulmonary arteries goes up.
Group 5 PH due to other disorders
Many other conditions cause pulmonary hypertension in ways that scientists don’t yet understand. Scientists don’t know the exact mechanisms that cause these conditions to trigger PH. What they do know is that there’s an association between these conditions and PH. That means people with these conditions may face a higher risk of developing PH. Such conditions include:
- Gaucher disease: A metabolic disorder that affects your organs and bones.
- Kidney disease: Problems with the function of your kidneys.
- Langerhans cell histiocytosis: A disorder that, in adults, causes scarring and cysts in your lungs.
- Sarcoidosis: An inflammatory disease that usually affects your lungs and lymph glands.
- Thyroid disease: Disorders that affect your thyroid’s production of hormones.
- Tumors: Cancerous or benign tumors may put pressure on blood vessels in your lungs.
