Pulmonary hypertension symptoms causes and treatment methods

Pulmonary hypertension is a serious and rare medical condition that affects the pulmonary arteries and the right side of the heart. It occurs when blood pressure in the pulmonary arteries rises abnormally, restricting blood flow and placing significant strain on the heart. Symptoms often begin with shortness of breath and severe fatigue, gradually progressing to life-threatening complications if not diagnosed and treated early. In this article from Dalili Medical, we explore everything you need to know about pulmonary hypertension — from its causes and symptoms, through modern diagnostic methods, to the latest treatments and practical tips for prevention and improving quality of life.

1. What is pulmonary hypertension?
It is an abnormal increase in blood pressure within the pulmonary arteries that carry blood from the heart to the lungs, increasing the heart’s workload and affecting breathing.

2. What is the difference between pulmonary hypertension and regular high blood pressure?
Regular high blood pressure affects the arteries throughout the body, while pulmonary hypertension is limited to the pulmonary arteries only.

3. Can pulmonary hypertension be cured?
In most cases, there is no complete cure, but early diagnosis and proper treatment can help control symptoms and slow disease progression.

4. What are the first warning signs?
Shortness of breath during exertion, rapid fatigue, fast heartbeat, and sometimes dizziness or fainting.

5. Can you exercise with pulmonary hypertension?
Light activities such as walking or stretching can be done under medical supervision, but intense exertion should be avoided.

6. Does pulmonary hypertension affect pregnancy?
Yes. Pregnancy poses significant risks to both mother and baby. Medical consultation is essential before considering pregnancy.

7. What is the life expectancy for patients?
It depends on the disease type, how early it is diagnosed, and the response to treatment, but modern therapies have improved survival rates and quality of life.

8. What causes pulmonary hypertension?
Causes include chronic heart disease, lung conditions such as pulmonary fibrosis or COPD, chronic pulmonary blood clots, and certain autoimmune diseases.

9. Can it affect young people or children?
Yes. While more common in adults, it can also occur in children and adolescents, especially if linked to congenital heart defects.

10. Is pulmonary hypertension genetic?
Some types can be hereditary, particularly the familial idiopathic type associated with specific gene mutations like BMPR2.

11. Does diet affect the condition?
Yes. Reducing salt intake, eating potassium- and magnesium-rich foods, and maintaining a healthy weight can help improve the condition.

12. Does it affect traveling or visiting high-altitude areas?
High altitudes reduce oxygen levels, which may worsen symptoms. Always consult your doctor before traveling.

13. What is the difference between primary and secondary pulmonary hypertension?
Primary pulmonary hypertension occurs without an identifiable cause, while secondary pulmonary hypertension results from another condition such as heart or lung disease.

14. Can pulmonary hypertension be prevented?
Not all types can be prevented, but treating heart and lung diseases early and preventing blood clots can lower the risk.

Types of Pulmonary Hypertension

According to the World Symposium on Pulmonary Hypertension, the disease is classified into five main groups:

1. Pulmonary Arterial Hypertension (PAH)

Occurs due to narrowing or stiffening of the small arteries in the lungs, restricting blood flow and increasing pressure.

Common causes include:

Idiopathic PAH (unknown cause)
Heritable PAH due to genetic mutations such as BMPR2
Linked to autoimmune diseases like scleroderma or systemic lupus erythematosus
Caused by certain drugs or toxins
Associated with congenital heart diseases

2. Pulmonary Hypertension due to Left Heart Disease

The most common type worldwide, occurring when the left side of the heart cannot pump blood effectively.

Causes include:

Systolic or diastolic heart failure
Heart valve diseases, especially mitral or aortic valve disorders

3. Pulmonary Hypertension due to Lung Diseases or Hypoxia

Caused by chronic lung conditions or situations that lead to low blood oxygen levels.

Causes include:

Chronic Obstructive Pulmonary Disease (COPD)
Pulmonary fibrosis
Sleep apnea
Long-term living at very high altitudes

4. Pulmonary Hypertension due to Chronic Obstruction in the Pulmonary Arteries
Known as Chronic Thromboembolic Pulmonary Hypertension (CTEPH), this condition results from previous blood clots that have not fully dissolved.

5. Pulmonary Hypertension from Rare or Multifactorial Causes
Includes less common cases such as:

Rare blood disorders.
Metabolic diseases such as Gaucher’s disease.
Complex systemic diseases.
Tumors obstructing the pulmonary arteries.

Symptoms of Pulmonary Hypertension (PH)

Pulmonary Hypertension is a progressive disease whose symptoms often start mild and gradually worsen over time. Because these symptoms may resemble those of other heart or lung diseases, early diagnosis is often a challenge.

1. Early Symptoms

Shortness of breath (Dyspnea) during exertion, such as climbing stairs or walking long distances.
Persistent fatigue and tiredness even with minimal activities.
Dizziness or lightheadedness, especially during physical effort, due to reduced blood flow to the brain.

2. Advanced Symptoms

Chest pain or pressure (Angina).
Swelling of the ankles or legs (Edema) caused by fluid retention.
Heart palpitations or irregular heartbeat (Arrhythmia).
Bluish lips or skin (Cyanosis) due to low blood oxygen levels.

3. Symptoms Related to Complications

Difficulty breathing even at rest.
Enlarged liver or a feeling of fullness in the abdomen due to blood congestion.
Loss of appetite or nausea caused by high blood pressure affecting the digestive system.

???? Important Note: In the early stages, patients may mistake these symptoms for simple fatigue or poor fitness, leading to delayed diagnosis until the condition worsens.

Causes of Pulmonary Hypertension

Causes vary depending on the type, but all lead to narrowing or obstruction of blood vessels in the lungs or increased pressure on the right side of the heart.

1. Chronic Heart and Lung Diseases

Chronic lung diseases: such as COPD, pulmonary fibrosis, and sleep apnea.
Left heart diseases: such as left ventricular failure and valvular diseases (mitral or aortic valve stenosis/regurgitation).

2. Pulmonary Vascular Diseases

Idiopathic Pulmonary Arterial Hypertension (IPAH) – cause unknown.
Genetic factors (mutations in genes like BMPR2).
Autoimmune diseases such as scleroderma, lupus, or vasculitis.

3. Chronic Blood Clots

Chronic pulmonary embolism due to recurrent blood clots that did not fully dissolve (CTEPH).

4. Other Conditions

Advanced liver disease (e.g., cirrhosis or portal hypertension).
Chronic infections such as schistosomiasis or HIV.
Long-term exposure to high altitudes.
Certain drugs or toxins (diet pills, amphetamines, cocaine).

Complications of Pulmonary Hypertension

If left undiagnosed and untreated, it can lead to life-threatening complications:

Right heart failure (Cor Pulmonale) – the most common and severe complication.
Arrhythmias – such as atrial fibrillation or ventricular tachycardia.
Blood clots – increasing the risk of pulmonary embolism.
Pulmonary hemorrhage – may cause life-threatening hemoptysis (coughing up blood).
Chronic hypoxia – leading to cyanosis and muscle weakness.
Premature death – from advanced heart or lung failure.

Diagnosis of Pulmonary Hypertension

1. Medical History & Physical Exam

Asking about symptoms: shortness of breath, fatigue, chest pain, fainting.
Checking for risk factors: heart, lung, or autoimmune diseases.
Physical findings: abnormal heart sounds, distended neck veins, enlarged liver, or leg edema.

2. Laboratory Tests

Blood tests to check for autoimmune, liver, or infectious diseases.
Cardiac biomarkers such as BNP and NT-proBNP to assess heart strain.

3. Imaging Tests

Chest X-ray: shows enlarged pulmonary arteries or right ventricle.
Echocardiography: estimates pulmonary artery pressure and assesses right ventricle function.

Treatment of Pulmonary Hypertension

The goals of treatment are to:

Relieve symptoms and improve daily activity.
Slow disease progression and prevent heart/lung complications.
Improve survival and quality of life.

The treatment plan depends on the type of PH (WHO Groups 1–5), cause, and severity.

1. Treating the Underlying Cause

Left heart disease: Treat heart failure or repair/replace damaged valves.
Chronic lung diseases: Optimize oxygen levels, treat infections or fibrosis, and prevent exacerbations.
CTEPH: Surgical removal of clots (Pulmonary Endarterectomy) or balloon pulmonary angioplasty.
Autoimmune diseases: Use corticosteroids or immunosuppressants to control inflammation.

2. Medications

Vasodilators: e.g., Epoprostenol, Treprostinil, Iloprost, Selexipag.
Endothelin Receptor Antagonists (ERAs): e.g., Bosentan, Ambrisentan, Macitentan.
PDE-5 inhibitors: e.g., Sildenafil, Tadalafil.
sGC stimulators: e.g., Riociguat.
Diuretics: to reduce fluid retention.
Anticoagulants: e.g., Warfarin in select cases.
Oxygen therapy: for chronic hypoxemia.

3. Surgical & Interventional Procedures

Balloon Atrial Septostomy (BAS): creates a small hole between atria to reduce right heart pressure.
Pulmonary Endarterectomy (PTE): surgical removal of chronic clots in CTEPH — potentially curative if successful.

C. Lung or Heart–Lung Transplantation
When the disease reaches the end stage of lung or heart failure and the patient no longer responds to other treatments, lung or combined heart–lung transplantation may be recommended.

Challenges: Limited donor availability, risk of organ rejection, and the need for lifelong immunosuppressive medications to prevent rejection.

D. Supportive Interventional Procedures
In critical cases, right ventricular assist devices (RVAD) may be used to help the heart pump blood more effectively while preparing for definitive treatment or transplantation.

3. Supportive Therapy for Pulmonary Hypertension – Exercise & Physical Activity

Although strenuous activity should be avoided, light and regular exercise under medical supervision is an important part of the treatment plan.

Benefits of Exercise for Pulmonary Hypertension Patients:

Improves the body’s ability to use oxygen more efficiently
Strengthens muscles and reduces fatigue
Improves balance and reduces the risk of falls
Supports mental health and reduces anxiety and depression

Tip: Combining exercise with cardiopulmonary rehabilitation sessions offers the best results.

Recommended Types of Exercise for Pulmonary Hypertension Patients

Slow to Moderate Walking
- Duration: 10–20 minutes daily, gradually increasing as tolerated
- Advice: Break walking into shorter sessions (5–10 minutes) to avoid overexertion
Deep Breathing Exercises (Diaphragmatic Breathing)
- Sit or lie down, place your hand on your abdomen
- Inhale deeply through the nose until the abdomen expands, then exhale slowly through the mouth
- Repetitions: 5–10 times, several times daily
Light Stretching Exercises
- To improve muscle and joint flexibility
- Performed before and after main workouts to reduce injury risk
Light Resistance Training
- Using small weights or resistance bands, 2–3 times weekly
- Caution: Avoid holding your breath during exercises

Safety Guidelines for Exercising

Always consult your doctor before starting any activity
Stop immediately if you feel dizzy, experience chest pain, or have severe shortness of breath
Avoid strenuous exercises or extreme temperatures (very hot or very cold weather)
In some cases, oxygen therapy may be recommended during exercise

Pulmonary Hypertension and Herbal / Natural Support

Currently, there is no scientifically proven herbal cure for pulmonary hypertension. However, certain herbs and supplements may support heart and lung health and improve circulation, provided they are used under medical supervision to avoid drug interactions.

Key Herbs & Supplements:

Garlic – Improves vascular elasticity and lowers blood pressure.
Caution: Use with care if taking blood thinners.
Ginger – Enhances circulation and reduces inflammation.
Note: Avoid high doses if on anticoagulants.
Turmeric – Rich in curcumin, a strong antioxidant and anti-inflammatory compound. Best absorbed with black pepper.
Ginkgo Biloba – May improve blood flow and reduce oxidative stress.
Note: Requires medical monitoring for heart patients or those on blood thinners.
Green Tea – High in antioxidants, supports vascular health.

Important Tips for Using Herbs

Consult your doctor first: Especially if you take blood pressure or heart medications
Herbs are a supplement, not a substitute: They should support, not replace, prescribed medical treatment
Avoid random combinations: Do not mix multiple herbal supplements without ensuring safety and avoiding harmful interactions

Lifestyle Tips for Pulmonary Hypertension Patients

Following a healthy lifestyle can help reduce symptoms and improve the effectiveness of medical treatment:

Regular Medical Check-ups – Keep all appointments to monitor your condition and adjust treatment
Medication Adherence – Take medicines on time, never stop or adjust doses without your doctor’s approval
Avoid Excessive Physical Strain – Engage in light to moderate activity like walking and breathing exercises, but avoid high-intensity workouts
Healthy Diet – Reduce salt intake to prevent fluid retention, and focus on vegetables, fruits, and whole grains