Pulmonary Hypertension

When you have pulmonary hypertension, it means you have one of a group of conditions caused by high blood pressure in your pulmonary arteries (blood vessels in your lungs). These arteries carry oxygen-poor blood from your heart to your lungs. But when you have PH, it means the flow of blood through your heart and lungs doesn’t move smoothly.

This can narrow the arteries and make your heart pump harder to move blood to your lungs. Over time this can damage your heart and cause other serious health conditions throughout your body. These include blood clots, anemia, irregular heartbeats and pericardial effusion.

There are five types of pulmonary hypertension:

• Pulmonary arterial hypertension (PAH): Starts in the pulmonary arteries. A rare type of PH.
• Pulmonary venous hypertension: Starts in the heart.
• Pulmonary hypertension from lung conditions: This starts from damaged lung tissue or low oxygen levels (hypoxia). PH may also be due to interstitial lung disease, which we can treat directly.

• Pulmonary hypertension from lung blockages: Blood clots or scars from blood clots in the pulmonary arteries (chronic thromboembolic pulmonary hypertension or CTEPH) cause this.

• Pulmonary hypertension from other conditions: This type starts from unknown (idiopathic) causes, metabolic or blood disorders, immune conditions or cancer.

Often, PH first shows up as shortness of breath — and it can be easy to brush off as low fitness or a run-of-the-mill cold. But then you start getting other symptoms like fatigue, dizziness, chest pain, swelling and a racing heartbeat. And starts getting worse until you have symptoms even when you’re resting.

What to expect at your first visit

PH can be difficult to diagnose because its symptoms are similar to those of other conditions. Understanding how you’re feeling and what kind of symptoms you’re having is important to making a correct diagnosis. So, when you come to your first visit, your provider will ask you to share your story. They’ll listen, ask questions and give you the chance to ask any questions you have. You’ll talk about:

• What kind of symptoms you have.
• How long you’ve had these symptoms.
• How your symptoms started.
• If your symptoms are getting worse.
• What other health conditions you may have.
• If you smoke.

Your provider will do a physical exam and blood tests to help us learn more about your overall health. You may have a comprehensive metabolic panel (CMP) and a complete blood count (CBC). They’ll likely order other tests, too, to help them rule out other possible conditions and identify any underlying problems that may cause PH.

Tests for pulmonary hypertension

There are many tests you can have to see how your lungs are working, if there’s any damage in them and what kind of blood pressure they have. Your provider may order:

• Pulmonary function testing.
• Polysomnogram (PSG).
• CT scan.
• Chest X-ray.
• MRI.
• Pulmonary ventilation/perfusion (VQ) scan.
• Electrocardiogram (EKG).
• Doppler echocardiogram.
• Right heart cardiac catheterization.

You may also do a six-minute walk test to pinpoint how severe your PH is. This test can show us how much exercise you can handle and how much oxygen is carried in your blood during exercise.

Meet our Pulmonary Hypertension Team

Because there are different types of pulmonary hypertension — and different causes — we make sure you see the best healthcare providers for your unique needs. Our team-based approach to treatment means providers from different specialties work together to get a correct diagnosis and craft a highly personalized treatment plan. This team could include:

• Pulmonologists.
• Cardiologists.
• Cardiothoracic surgeons.
• Radiologists.
• Endocrinologists.
• Nephrologists.
• Rheumatologists.
• Hepatologists (liver specialists).
• Nurse practitioners.

Your care team builds your treatment on many considerations, like the type of PH, any underlying conditions, your symptoms and your recovery goals. Right now, only PAH and CTEPH can be treated directly. Depending on these factors, your treatment could include:

Lifestyle changes

When PH is caused by other health conditions, we may recommend you make lifestyle changes to manage them. This could mean changing your diet, starting an exercise plan and quitting smoking.

Pulmonary hypertension medications

We may prescribe medications to treat PH. Some may widen your pulmonary arteries and reduce high blood pressure. Some also work to prevent blood clots and control your heart rate. You may receive medication in pill form, directly into your veins (intravenously), under your skin (subcutaneously) or as an inhaled medication. You may take only one medication, or we may recommend a combination of them. Medications include:

• Eproprostenol injections (Flolan^® and Veletri^®).
• Treprostinil (Remodulin^®, Tyvaso^® or Orenitram^®).
• Bosentan (Tracleer^®).
• Macitentan (Opsumit^®).
• Ambrisentan (Letairis^®).
• Sildenafil (Revatio^®).
• Tadalafil (Adcirca^®).
• Selexipag (Uptravi^®).
• Riociguat (Adempas^®).

Oxygen therapy

When you have pulmonary hypertension, your blood doesn’t flow as well through your lungs, where it goes to get oxygen. This means it doesn’t get enough oxygen to carry throughout your body. Your provider may recommend oxygen therapy to increase the amount of oxygen in your blood — and help relax the pulmonary arteries.

Surgery for CTEPH

Our skilled cardiothoracic surgeons may recommend surgery to treat some types of PH, particularly chronic thromboembolic pulmonary hypertension. We’re one of the few hospitals with expertise in treating CTEPH with pulmonary thromboendarterectomy (PTE).

Other surgery options may include balloon pulmonary angioplasty (BPA). This interventional treatment uses a balloon to open up blockages in your pulmonary arteries. This is most often done if PTE isn’t an option.

We take a wide-ranging, multidisciplinary approach to CTEPH, possibly including a combination of these treatments — from PTE to BPA to medical therapy.

Lung transplant for pulmonary hypertension

If pulmonary hypertension becomes severe or your body doesn’t respond to other treatment options, we may recommend a lung transplant or heart-lung transplants as a last resort. If so, you’ll undergo a lung transplant screening and evaluation with our expert team. We’ll go over what to expect and support you through every step of transplant surgery, recovery and follow-up.

Taking the Next Step

It might seem easier to ignore that huffing and puffing you keep having, even after doing light activities. But frequent shortness of breath can be one of the first signs of pulmonary hypertension. And getting checked out soon as possible is important because PH will keep getting worse without treatment. Our healthcare providers understand it can be stressful to find out what’s going on. And they’re here to support you with compassionate support and expert, personalized care. We want you to feel confident about moving forward with treatment — and a more active life.