Have you ever heard of a condition where the pressure in the tiny blood vessels inside the lungs suddenly increases? It's like something getting stuck in a water pipe and the pressure increases. That's what CTEPH is. While the name may sound a bit long, it's easy to understand. It's a rare condition, but it's an important one that we should all be aware of.
Simply put, what is CTEPH?
CTEPH is high blood pressure in the lungs. Medically, we call this `(Pulmonary Hypertension)`. But this is different from normal high blood pressure. There is a specific reason why CTEPH occurs. That is, blood clots that have previously been stuck in the blood vessels of the lungs `(Pulmonary Embolism)` do not dissolve properly .
Imagine a person has a blood clot in their lungs. Normally, our body's own mechanisms dissolve these blood clots over time. But in some people, these blood clots don't dissolve completely. Instead, they become old, scar-like tissue that lodges in the blood vessels. Over time, this scar tissue causes the blood vessels to narrow.
When blood vessels narrow, the heart has to work harder to pump blood through those narrowed vessels. This causes the pressure inside the blood vessels in the lungs to rise too high. That's what we call CTEPH (Chronic Thromboembolic Pulmonary Hypertension) .
The important thing is that this disease only occurs in people who have a history of pulmonary embolism. If the pressure in the lungs is high without such a history, it could be another medical condition.
How does CTEPH affect your body?
The right side of our heart pumps oxygen-poor, impure blood to the lungs for purification. Now, when the blood vessels in the lungs become narrowed due to CTEPH and the pressure increases, the right side of the heart has to face a major obstacle in pushing this blood to the lungs.
This causes two main problems:
1. Overloading the heart: When you work hard like this all the time, the right side of the heart starts to weaken. This is like a water pump working hard to fill a blocked space. Over time, the pump weakens.
2. Decreased oxygen levels in the body: The blood is unable to properly pass through the lungs and pick up oxygen. This causes the oxygen levels in the body to drop. This is what we experience as symptoms like fatigue and shortness of breath.
What factors increase the risk of developing CTEPH?
As mentioned earlier, the main cause is that the blood clots in the lungs do not completely dissolve. However, some people are more likely to develop this condition. Let's take a look at what these risk factors are.
| Risk factor | Description |
|---|---|
| Blood type | Having a blood type other than 'O'. |
| Blood clotting diseases | If you personally or someone in your family has a blood clotting disorder (e.g. Antiphospholipid Syndrome ). |
| Spleen removal | Splenectomy (surgical removal of the spleen) . |
| Long-term infections | Long-term infections such as bone infections (osteomyelitis) . |
| Other medical conditions | Some types of cancer and diseases such as Inflammatory Bowel Disease . |
| Other factors | Being a woman, having a pacemaker in the heart, and taking thyroid hormone treatment are risk factors for infections. |
What could be the symptoms of this disease?
The symptoms of CTEPH may not be obvious at first. They develop gradually. You may think that this is just normal fatigue. But it is very important to pay attention to these symptoms.
| Symptom | More information |
|---|---|
| Difficulty breathing | It occurs especially when you exert yourself, such as when walking or climbing stairs. As the disease progresses, this discomfort can occur even when you are just standing still. |
| Extreme fatigue and exhaustion | Feeling tired all the time even without doing any work. |
| Chest pain or tightness | This pain can occur because the heart has to work harder. |
| Swelling of the legs and ankles (Edema) | Due to impaired heart function, fluid accumulates in the body, causing swelling in the legs. |
| Dizziness and fainting (Syncope) | This can happen when the brain does not receive enough oxygen. |
| Heart palpitations | Feeling like you can hear your own heartbeat. |
| Blue discoloration (Cyanosis) | Lips and fingertips turn blue due to low oxygen in the blood. |
If you have these symptoms, especially if you have a history of previous blood clots in the lungs, please see your doctor immediately.
How is CTEPH diagnosed?
When you see a doctor, he or she will ask about your symptoms and examine you. If there is any suspicion that you have CTEPH, they will recommend several tests to confirm the diagnosis.
- V/Q scan: This looks at how blood flows to the lungs and how air moves through them. This scan can detect areas where blood clots are present, as blood flow is reduced.
- Transthoracic Echocardiogram (TTE): This can check the pressure on the right side of the heart and how weak the heart is.
- CT Pulmonary Angiogram: This is a special CT scan that can clearly see old blood clots and narrowing of the blood vessels in the lungs.
Further tests to confirm
Once the disease is confirmed, several more tests may be performed to determine its severity and plan treatment.
- Right Heart Catheterization: This is the most accurate way to measure pulmonary pressure. A small tube (catheter) is inserted through a blood vessel in the body into the right side of the heart and the main blood vessel that leads to the lungs, and the pressure is measured.
- Exercise Stress Test: This test is done to see how your heart and lungs work when you exercise.
What are the treatments for this?
The good news about CTEPH is that it is a curable disease. Most patients can be relieved with surgery or other treatments. There are two main treatment options.
1. Pulmonary Endarterectomy (PTE):
This is an open-heart surgery. In this procedure, surgeons open the main blood vessels in the lungs and scrape out any old blood clots and scar tissue that may have built up inside. Although this is a very complex surgery, if successful, it has a high chance of completely curing the disease.
2. Pulmonary Balloon Angioplasty (BPA):
This treatment is used for people who have blood clots in veins that are too small to be operated on. In this treatment, a small tube (catheter) is inserted into a vein in the leg and a small balloon at the end is used to inflate and widen the narrowed vein. This is not as major as surgery.
Either of these treatments can almost completely cure CTEPH. After treatment, most people can live a normal life.
Sometimes, for those who cannot undergo surgery or who still have somewhat high pressure in their lungs after surgery, special medications (e.g., Riociguat) are given to control that pressure.
Things to know when living with CTEPH
Your life will improve a lot after treatment, but there are a few things you need to be aware of.
- Lifelong blood thinners: You will need to take blood thinners for the rest of your life to prevent further blood clots. It is important to follow your doctor's instructions exactly.
- Diet: If you are taking a medication like `Warfarin`, you should be careful about foods rich in vitamin K (such as cabbage, spinach, broccoli). Talk to your doctor about this.
- Avoid smoking: Smoking is very harmful to your lungs. If you are a smoker, it is imperative to quit completely.
- Light exercise: Light exercise, such as walking, can help increase your strength. However, be sure to consult your doctor before starting any exercise program. You may be referred to a medically supervised rehabilitation program called ``Pulmonary Rehabilitation.''
- Mental health: Living with a long-term illness like this can lead to feelings of stress, anxiety, or depression. If you're feeling this way, don't be ashamed to talk to your doctor or someone close to you.
Take-Home Message
- CTEPH is a high blood pressure condition caused by old blood clots getting stuck in the blood vessels of the lungs.
- Do not confuse this with normal high blood pressure.
- If you have symptoms such as shortness of breath, extreme fatigue, or swelling in your legs, especially if you have a history of blood clots, see a doctor immediately.
- This disease can often be completely cured with surgery or other methods.
- Even after treatment, follow your doctor's instructions exactly, especially regarding taking blood-thinning medications.
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