Pulmonary Hypertension - Diagnosis and Treatment

Diagnosis

Evaluating with Medical Examinations and Tests

Doctors usually start identifying pulmonary hypertension by reviewing symptoms like shortness of breath, chest pain, dizziness, palpitations, or tiredness. Many symptoms are similar to other heart or lung diseases.

Because of this, doctors often order several tests to confirm the diagnosis and look for other health problems.

Assessment Steps:

Physical Check: Doctors listen to the heart and lungs, look for leg swelling, and check for signs of heart failure.
Medical & Family History: Doctors gather information about risk factors such as chronic lung diseases, congenital heart disease, liver disease, or family history of pulmonary hypertension.

Laboratory and Imaging Tests:

Test Name	Purpose
Blood Tests	Check for anemia, sickle cell disease, liver problems, risk of scleroderma, and signs of inflammation. Detect causes or complications related to pulmonary hypertension.
Chest X-ray	Shows heart and lung structure. Can reveal causes like emphysema or interstitial lung disease.
Electrocardiogram (EKG/ECG)	Tracks the heart’s electrical signals to detect abnormal rhythms or strain on the right side of the heart.
Echocardiogram	Uses sound waves to make images of the heart. Measures function, the size of heart chambers, and detects blood flow issues. Important for screening and monitoring. Sometimes performed with exercise to see effects of activity.
Right Heart Catheterization	Directly measures blood pressure in the pulmonary arteries using a thin tube. This is the most accurate test for confirming diagnosis.
CT Scan	Shows detailed images of the heart, lungs, and blood vessels. Helps spot blood clots (pulmonary embolism), abnormal blood vessels, or blockages.
Cardiac MRI	Offers detailed pictures and blood flow analysis. Helpful in checking the function of the right ventricle and blood flow in pulmonary arteries.
Pulmonary Function (Spirometry)	Assesses how well lungs are working. Can find signs of chronic obstructive pulmonary disease (COPD) or interstitial lung disease.
Exercise Tests	Examines how exercise affects heart and lung function, often by using a treadmill or stationary bike. Assesses severity and tracking progress.
Six-Minute Walk Test	Measures exercise capacity by recording how far a person can walk in six minutes. Indicates everyday physical limits.
Sleep Study (Polysomnography)	Checks for sleep apnea by monitoring breathing, oxygen levels, and heart activity during sleep.
Ventilation/Perfusion (V/Q) Scan	Detects blood clots by showing how well air and blood move through the lungs. Helpful for diagnosing pulmonary embolism as a cause.
Lung Biopsy	Rare test to examine lung tissue for diseases affecting blood vessels or tissue. Used when other tests are unclear.

These tests can show underlying causes, such as chronic heart failure, connective tissue diseases, anemia, or previous blood clots.

Key Points:

Doctors often use right heart catheterization to confirm the diagnosis.
Echocardiography helps with initial screening.
Lung function tests help find breathing problems like COPD or interstitial lung disease.
Blood tests can show genetic risks, anemia, or other diseases that make pulmonary hypertension worse.

Checking for Inherited Risk Factors

Sometimes, people develop pulmonary hypertension because of changes in their genes. When doctors suspect this, they may order genetic tests.

Genetic analysis looks for gene variations linked to pulmonary arterial hypertension (PAH). Results can help doctors decide if other family members need screening.

Families with histories of connective tissue disease, heart defects, or blood disorders such as sickle cell disease may benefit from these tests.

Assessing How Pulmonary Hypertension Affects Everyday Life

After confirming the diagnosis, doctors classify how much the disease limits daily activity. This method is called “functional grouping.”

The categories help track changes over time and guide treatment choices.

Functional Group Table:

Group	Symptoms	Impact on Activity
Group 1	No symptoms with activity or rest.	No limits
Group 2	No symptoms at rest; mild symptoms with normal activities.	Slight limits (“ordinary” activities can cause mild shortness of breath or chest pain.)
Group 3	Comfortable at rest; symptoms with small tasks.	Simple daily chores like dressing or cooking can lead to tiredness, shortness of breath, or chest discomfort. Activity is much more limited.
Group 4	Symptoms at rest and with any activity.	Even basic movements or rest bring increasing discomfort.

Doctors might use checklists or calculators that combine symptoms, walking test results, imaging studies, and blood test numbers to personalize care.

Treatment

Medication Choices

Doctors use several types of medicine to treat pulmonary hypertension. Each medicine works differently to lower pressure in the lungs, make breathing easier, and improve quality of life.

The main medicines include:

Vasodilators: These drugs open up narrow blood vessels. This helps blood flow more easily through the lungs. Some are given through a vein, inhaled, or taken as a pill. Examples are epoprostenol, treprostinil, iloprost, and selexipag.
Soluble Guanylate Cyclase (sGC) Stimulators: These medicines relax blood vessels in the lungs and lower pressure inside the arteries. Riociguat is one medicine in this group. Pregnant people should not use these medicines.
Endothelin Receptor Antagonists: These medicines block substances that tighten blood vessels. Examples are bosentan, macitentan, and ambrisentan. They can help increase energy and reduce symptoms. Pregnant people should avoid this type of medicine.
Phosphodiesterase 5 (PDE5) Inhibitors: These medicines increase blood flow through the lungs. They are also used for other health issues like erectile dysfunction. Examples include sildenafil and tadalafil.
Calcium Channel Blockers: These relax the blood vessel walls. Only certain people benefit from them. Examples include amlodipine, diltiazem, and nifedipine.
Anticoagulants (Blood Thinners): These help prevent blood clots but can make bleeding more likely. Warfarin is a common option. Doctors take extra care with these medicines, especially before surgeries or treatments that break the skin.
Cardiac Glycoside Therapy: This type of medication helps the heart pump more effectively and supports a regular heartbeat.
Diuretics (Water Pills): These help the body get rid of extra fluid. Removing extra fluid makes it easier for the heart to work and helps reduce swelling in the legs, belly, or lungs.
Oxygen Therapy: Using extra oxygen helps people who live at higher altitudes or have trouble due to sleep apnea. Some people may need oxygen all day to feel better.

Doctors schedule regular checkups to find the best mix of medicines. They may change the dose or type of drug if symptoms change.

Surgical Options and Other Interventions

If medicine does not help enough, doctors may recommend other steps. Certain procedures or surgeries can lower symptoms and improve life for people with pulmonary hypertension.

Creating an Opening in the Heart (Atrial Septostomy): Doctors make a small hole between the heart’s top chambers. This can ease pressure on the right side of the heart. There may be risks, such as irregular heartbeats.
Lung or Heart-Lung Transplants: If medicine and other treatments do not work, doctors may recommend a transplant. This is more common for young patients with certain forms of the disease. After a transplant, people must take medicine every day to protect the new organ.

Some people receive infusion therapy, where medicine goes directly into the blood by a small pump. This helps those who need extra support from their medications.

Day-to-Day Steps and Home Strategies

People can manage pulmonary hypertension better by making practical changes to their daily routines. A healthy diet plays an important role. Eating more fruits, vegetables, whole grains, lean meats, and low-fat dairy helps support health.

Choosing foods low in saturated fat, trans fat, and cholesterol benefits the heart. Reducing salt in meals helps control fluid in the body.

Physical activity, when safe, can help. Simple exercises like walking are useful, but only if they do not cause exhaustion. Sometimes, people exercise while using oxygen. Always talk to a healthcare team before starting any exercise plan to ensure safety.

Stopping smoking is a key step. Smoking damages the lungs and heart, and quitting improves health. Avoiding secondhand smoke also protects the lungs. Support from healthcare professionals can make quitting easier.

Proper rest is necessary. Getting enough sleep and taking breaks when tired help reduce symptoms. People should avoid high elevations, since mountain locations can make symptoms worse. If possible, living at a lower elevation helps those who are sensitive.

Some activities can lower blood pressure too much. Using hot tubs, saunas, or taking very hot baths or showers should be avoided. People should also skip heavy lifting or actions that require a lot of straining.

Open communication with a healthcare team helps track progress. Patients should mention new symptoms or any troubling side effects from medication. Bringing a list of medicines to each appointment is a helpful practice.

Healthcare providers recommend staying up to date on vaccinations. Lung infections can be more serious for people with pulmonary hypertension, so staying current on immunizations helps protect against common illnesses.

Certain life events, like pregnancy, need special care. People who want to become pregnant should talk to a healthcare provider about the risks.

Birth control options may need to change, since some types are not recommended for people with pulmonary hypertension.

The table below summarizes some key strategies:

Strategy	Purpose
Eat low-salt, nutritious foods.	Supports heart and lung function.
Moderate, doctor-approved exercise.	Helps with overall fitness.
Avoid smoking and secondhand smoke.	Protects lung and heart health.
Get rest and avoid exhaustion.	Reduces extreme tiredness.
Stay away from high altitudes.	Prevents worsening symptoms.
Avoid overheated environments.	Reduces risk of low blood pressure and fainting.
Stay in regular contact with doctors.	Helps manage health changes.
Keep up with recommended vaccines.	Lowers the risk of serious infections.
Review pregnancy or birth control plans with a provider.	Ensures safer health choices.

Finding Comfort and Encouragement

Connecting with others who have pulmonary hypertension can help improve quality of life. People can join local or online support groups to share experiences and find emotional support.

Talking with healthcare providers about available resources may also help reduce feelings of isolation.

Getting Ready for Your Visit

Steps You Can Take Before the Visit

Ask About Pre-visit Instructions: Check with the clinic about any special steps to follow before your visit. Some tests might require not eating or drinking for a set period.
Track Your Symptoms: Write down all symptoms you have noticed, even if they do not seem connected to pulmonary hypertension. Note when each symptom started and how long it has lasted.
Gather Personal and Family Health Details: List any close family members with lung or heart problems, stroke, high blood pressure, or diabetes. Include any stressful events or major life changes.
Record All Medicines and Supplements: Bring a complete list of every prescription drug, over-the-counter medicine, vitamin, and supplement you use.
Bring a Support Person: If possible, bring someone with you to help remember what the doctor says and provide support.
Review Your Diet and Exercise Habits: Be ready to discuss what you eat and your physical activity. If you struggle with diet or exercise, prepare to talk about your challenges.
Prepare Questions: Write out questions you want to ask the health team. Put the most important ones first.

Possible questions include:

Topic	Sample Question
Cause of symptoms	What could be causing my symptoms?
Diagnosis	What tests do I need?
Treatment options	Are there other treatments I should consider?
Medication	Is there a generic version of this medicine?
Physical activity	What kind of activity is safe for me?
Follow-up	How often should I return for checkups?
Specialist care	Should I see a specialist?
Self-care information	Where can I find more reliable information?

Add any other concerns or questions you may have.

What Your Doctor Might Ask

Your healthcare team will likely ask about your medical history and symptoms.

Being ready for these questions can help your visit go smoothly.

They may ask:

When your symptoms began
Whether you experience symptoms all the time or only sometimes
How severe your symptoms feel, using a scale from 1 to 10
What tends to improve or worsen your symptoms
Your past health problems and history with similar conditions
Any recent changes in your health or stress levels

Having notes ready can help you answer more accurately.

Pulmonary Hypertension – Diagnosis and Treatment