Understanding Pulmonary Hypertension: Symptoms, Causes, and Hope
Know what is pulmonary hypertension, 5 groups of pulmonary hypertension, symptoms, diagnosis and treatment options and more.
Introduction
Have you ever felt unusually short of breath after a simple activity, like walking to the mailbox or climbing a few stairs? While it's easy to attribute this to being out of shape, it can sometimes signal a more serious condition affecting the heart and lungs. Pulmonary hypertension (PH) is a complex and often misunderstood disorder characterised by high blood pressure in the arteries of your lungs. Unlike the common systemic high blood pressure, PH specifically targets the pulmonary arteries, forcing the right side of your heart to work much harder to pump blood through your lungs. This guide will walk you through everything you need to know, from recognising the early warning signs and understanding the different types to exploring modern treatment options that offer real hope for managing this condition.
What is Pulmonary Hypertension? It’s Not What You Think
Many people hear "hypertension" and think of the common condition measured with a cuff on your arm. Pulmonary hypertension is fundamentally different. It refers to high blood pressure that occurs specifically in the pulmonary arteries—the vessels responsible for carrying blood from the right side of your heart to your lungs to pick up oxygen.
In PH, these arteries become narrowed, blocked, or stiff. This forces your heart's right ventricle to pump harder to push blood through, which, over time, causes the heart muscle to weaken and potentially fail. It's a serious, progressive disease, but with advancements in treatment, it can be managed effectively.
Consult Top Pulmonologist for Personalised Advice
Pulmonary vs. Systemic Hypertension: A Crucial Difference
Understanding this distinction is key:
Systemic Hypertension: This is common "high blood pressure." It affects arteries throughout your entire body (systemic circulation) and is easily measured with an arm cuff.
Pulmonary Hypertension: This is high blood pressure only within the lung arteries (pulmonary circulation). It cannot be detected with a standard blood pressure check and requires specialised tests for diagnosis.
Recognising the Signs: Symptoms of Pulmonary Hypertension
The symptoms of PH are often subtle in the early stages and can be mistaken for asthma, being out of shape, or other common conditions. This is why awareness is critical.
Early Symptoms Often Overlooked
Shortness of breath (dyspnea): Initially during physical activity, but eventually at rest.
Fatigue: A pervasive feeling of tiredness and lack of energy.
Chest pain or pressure: Often felt during increased activity.
Heart palpitations: A racing heartbeat or a sensation of fluttering in the chest.
Symptoms as the Condition Progresses
As the strain on the heart increases, more severe symptoms may develop:
Dizziness or fainting spells (syncope): Especially during exertion.
Swelling (oedema) in the ankles, legs, and eventually the abdomen.
Bluish colour to the lips and skin (cyanosis): A sign of low oxygen levels in the blood.
A feeling of fullness in the upper abdomen due to an enlarged liver.
If you experience persistent and unexplained shortness of breath, it's crucial to consult a doctor. You can book a convenient online consultation with a specialist on Apollo24|7 to discuss your symptoms and determine the necessary next steps.
Why Does It Happen? The 5 Groups of Pulmonary Hypertension
PH isn't a single disease; it's a condition with multiple potential causes. The World Health Organisation (WHO) classifies it into five groups based on its underlying cause, which is essential for determining the correct treatment for pulmonary arterial hypertension.
Group 1: Pulmonary Arterial Hypertension (PAH)
This group includes PH with no clear cause (idiopathic), heritable causes, and PH caused by conditions like connective tissue diseases (scleroderma, lupus), HIV infection, or congenital heart disease.
Group 2: PH Due to Left Heart Disease
This is the most common form of PH. It results from issues with the left side of the heart, such as heart valve disease or heart failure with preserved ejection fraction (HFpEF), which causes pressure to back up into the lungs.
Group 3: PH Due to Lung Disease
This form is caused by lung conditions that lead to low oxygen levels, such as Chronic Obstructive Pulmonary Disease (COPD), interstitial lung disease, and sleep apnea.
Group 4: PH Due to Chronic Blood Clots
Known as Chronic Thromboembolic Pulmonary Hypertension (CTEPH), this type occurs when old blood clots (emboli) obstruct the pulmonary arteries. Unlike acute clots, these don't dissolve and cause persistent blockages.
Group 5: PH with Unclear and/or Multifactorial Causes
This group includes PH with various rare triggers, such as certain blood disorders, metabolic disorders, or systemic conditions like sarcoidosis.
Getting a Diagnosis: How Doctors Detect Pulmonary Hypertension
Because its symptoms mimic other conditions, diagnosing PH requires a multi-step process. It often begins with an echocardiogram (an ultrasound of the heart) that can estimate the pressure in the pulmonary arteries and assess heart function.
The Gold Standard Test: Right Heart Catheterisation
While an echocardiogram can suggest PH, a right heart catheterisation is the only definitive test for diagnosis. In this procedure, a thin, flexible tube (catheter) is inserted into a vein and guided into the right side of the heart and pulmonary arteries. It directly measures the blood pressure within these chambers and vessels, confirming the diagnosis and helping to guide treatment decisions. Other tests, like CT scans, pulmonary function tests, and a 6-minute walk test, are also used to assess severity and underlying causes.
Treatment Options for Pulmonary Hypertension
Treatment is highly personalised and depends on the WHO group classification. The goals are to treat the underlying cause, improve symptoms, and slow the disease's progression.
Medications to Manage Pulmonary Arterial Pressure
For Group 1 PAH, several advanced drug classes are available:
Vasodilators: These drugs relax and open narrowed blood vessels. They are often delivered via inhalation, IV, or subcutaneous pump.
Endothelin Receptor Antagonists: These block the action of endothelin, a substance that narrows blood vessels.
Phosphodiesterase-5 Inhibitors: These help relax the pulmonary artery muscles and increase blood flow.
Anticoagulants (blood thinners) may be used to reduce the risk of clots.
Oxygen Therapy and Lifestyle Changes
Supplemental oxygen can help if blood oxygen levels are low. Doctors also recommend lifestyle adjustments like a low-sodium diet, fluid restriction, supervised physical activity, and avoiding pregnancy, which puts extreme stress on the heart.
Surgical Procedures and Interventions
For advanced cases or specific types like CTEPH (Group 4), surgery may be an option:
Pulmonary Thromboendarterectomy: A complex surgery to remove old clots from the lungs.
Atrial Septostomy: A procedure to create an opening between the heart's upper chambers to relieve pressure.
Lung or Heart-Lung Transplant: Considered for severe cases when other treatments have failed.
Living with Pulmonary Hypertension: Management and Outlook
A PH diagnosis is life-changing, but it's not a life sentence. With proper treatment, many people can manage their symptoms and lead active lives.
Daily Life and Coping Strategies
Staying active within limits set by your doctor is important for maintaining strength. Pacing yourself, eating a heart-healthy diet, getting plenty of rest, and getting vaccinated against flu and pneumonia are all key strategies. Apollo24|7 offers convenient home collection for tests like NT-proBNP, which can help monitor heart strain, allowing you to track your health from the comfort of your home.
The Importance of a Support System
Connecting with others who understand your journey is invaluable. Consider joining a patient support group. Emotional health is just as important as physical health, so don't hesitate to seek counselling if you're feeling overwhelmed, anxious, or depressed.
Conclusion
Understanding pulmonary hypertension is the first powerful step toward managing it. While it's a complex and serious condition, significant medical advancements have transformed its outlook. From precise diagnostic tools like right heart catheterisation to targeted therapies that relax lung arteries and reduce strain on the heart, there is more hope than ever before. Remember, you are not alone on this journey. By working closely with a dedicated healthcare team, making informed lifestyle choices, and leveraging a strong support network, you can actively manage your health and maintain your quality of life. If you or a loved one is experiencing potential symptoms, take that crucial first step: seek expert medical advice to get the answers and care you deserve.
Consult Top Pulmonologist for Personalised Advice
Consult Top Pulmonologist for Personalised Advice
Dr Rakesh Bilagi
Pulmonology Respiratory Medicine Specialist
10 Years • MBBS MD PULMONOLOGIST
Bengaluru
Apollo Clinic, JP nagar, Bengaluru
Dr. Naseeha Mohammed S V
Pulmonology Respiratory Medicine Specialist
6 Years • MBBS, MD ,DNB Respiratory Medicine
Bengaluru
Apollo Clinic, Sarjapur Road, Bengaluru
Dr Haripriya S G
Family Physician
22 Years • MBBS, PGD (Family Medicine)
Bengaluru
Apollo Clinic, JP nagar, Bengaluru
Dr. Sumara Maqbool
Pulmonology Respiratory Medicine Specialist
12 Years • MBBS, DNB Respiratory, critical care and sleep medicine, DrNB superspeciality Critical care, IDCCM, IFCCM, EDIC
Delhi
Apollo Hospitals Indraprastha, Delhi
(25+ Patients)
Dr. Chandrakant Tarke
Pulmonology Respiratory Medicine Specialist
11 Years • DM Pul. , MD, DNB, MNAMS, EDRM
Hyderabad
Apollo Hospitals Jubilee Hills, Hyderabad
(225+ Patients)
More articles from Pulmonary Hypertension
Frequently Asked Questions
1. Is pulmonary hypertension curable?
While there is currently no universal cure for all types of pulmonary hypertension, many forms are highly treatable. The goal of treatment is to effectively manage symptoms, slow disease progression, and improve quality of life. For some types, like CTEPH, surgery can be potentially curative.
2. What is the life expectancy for someone with pulmonary hypertension?
Life expectancy with pulmonary hypertension has improved dramatically over the past two decades due to new treatments. Prognosis varies greatly depending on the type of PH, its cause, how early it's diagnosed, and how well a patient responds to therapy. Many patients now live well over a decade after diagnosis.
3. Can you have pulmonary hypertension with a normal echocardiogram?
Yes, it is possible, especially in the very early stages. An echocardiogram is an excellent screening tool, but it is an estimate. A normal result does not completely rule out PH. If clinical suspicion remains high, a doctor will recommend a right heart catheterisation for a definitive answer.
4. What is the difference between PAH and pulmonary hypertension?
Pulmonary Hypertension (PH) is the broad umbrella term for high blood pressure in the lungs. Pulmonary Arterial Hypertension (PAH) is a specific subtype (WHO Group 1) where the small arteries in the lungs become narrowed. All PAH is PH, but not all PH is PAH.
5. Does pulmonary hypertension run in families?
In most cases, it does not. However, a small percentage (5-10%) of patients with idiopathic PAH have a heritable form caused by a genetic mutation that can be passed down in families. Genetic counselling may be recommended in these cases.