Everything You Need To Know About Primary Pulmonary Hypertension

Posted on: Aug 27, 2024 | 3 mins | Written by: HDFC ERGO Team

Everything You Need To Know About Primary Pulmonary Hypertension

Do you feel dizzy and extremely tired often? Do you experience severe pain in the chest from time to time and find it difficult to breathe after a strenuous activity? You may be suffering from primary pulmonary arterial hypertension or primary pulmonary hypertension (PPH). This is a rare type of high blood pressure related to the narrowing of blood vessels in the lungs. Let’s talk about PPH in detail.

What is Primary Pulmonary Hypertension?

Primary pulmonary arterial hypertension, also known as idiopathic pulmonary arterial hypertension, pulmonary arterial hypertension (PAH) or primary pulmonary hypertension (PPH), is a type of high blood pressure which affects the arteries and capillaries in the lungs. It happens when the pressure in the pulmonary arteries increases significantly due to the constriction of blood vessels in the lungs.

Pulmonary arteries and capillaries carry blood from your heart to the lungs. When the blood vessels become narrow, the heart works harder to ensure blood reaches the lungs. This excessive pressure weakens the heart muscle over time and increases the risk of heart failure and death.

Causes and Risk Factors of PPH

While the exact causes of PPH are unknown, experts believe that the following factors can increase the risk of developing the condition:

• High blood pressure

• Chronic diseases related to the heart, lungs, and liver

• Sickle cell anaemia

• Certain connective tissue disorders, such as systemic sclerosis, mixed connective tissue disorders, and systematic lupus erythematosus

• Infections such as HIV or schistosomiasis (a chronic parasitic disease)

• Genetic mutations:

PPH can be inherited from close family members. It can happen due to a gene defect that may run in your family. Studies have shown that gene mutation makes blood vessels sensitive to certain factors and this causes them to become constricted.

Signs and Symptoms of Primary Pulmonary Hypertension

Primary pulmonary arterial hypertension may not show many signs and symptoms in its early stages. However, as the condition worsens, you may experience the following health issues:

• Dizziness and fatigue

• Difficulty breathing or shortness of breath

• Fainting

• Pressure and pain in the chest

• Heart palpitations

• Racing pulse

• Dry cough

• Swelling of the legs or ankles

• Swelling inside the abdomen due to fluid accumulation

The symptoms of the condition worsen over time, and patients may experience them even when they are resting.

Diagnosis of Primary Pulmonary Arterial Hypertension

Doctors may prescribe the following tests to diagnose primary pulmonary arterial hypertension —

1. Chest X-ray:

This is done to take images of the heart and other internal tissues.

2. Chest CT scan:

This can help check if there are any injuries and blood clots in the chest. A CT scan can help identify the narrowing of the pulmonary arteries and the blood flow.

3. Echocardiogram:

This test can help check the functioning of the heart and measure pulmonary artery pressure. It can also identify heart damage and enlargement.

4. Electrocardiogram (ECG):

This test is done to check for abnormal heart rhythms.

5. Lung perfusion scan:

This is a nuclear radiology test done to check lung function.

6. Pulmonary function tests:

These tests help measure the functioning of the lungs and their ability to inhale oxygen and exhale carbon dioxide.

7. Cardiac catheterisation of the right side of the heart:

This test is done to measure pressure inside the pulmonary arteries.

8. Blood tests:

These tests can help check oxygen levels in blood, strain on the heart, liver and kidney function, and other conditions.

Treatment Options for PPH

The treatment options for primary pulmonary arterial hypertension include the following:

1. Medications

• Anticoagulants:

These are blood thinners that support easy flow of blood in the arteries. They also help prevent the clotting of blood.

• Diuretics:

Also called water pills, diuretics reduce the amount of fluid in the body. The absence of extra fluid makes it easier for the heart to pump blood.

• Vasodilators:

These medications help lower blood pressure in the lungs and prevent the weakening of the arteries and capillaries.

• Calcium channel blockers:

These medicines are prescribed to lower blood pressure and prevent excessive strain on the heart and lungs.

In addition to these, doctors may prescribe other medications to lower blood pressure in the lungs. These medications can also support heart function and help it pump blood easily.

2. Oxygen therapy

A person with severe primary pulmonary arterial hypertension may experience difficulty in breathing. They are then given oxygen therapy through a mask or nasal prongs.

3. Surgery

Severe cases of PPH may require surgical treatment. Depending on the condition of the patient, doctors may recommend the following procedures —

• Atrial septostomy:

This surgery is done to reduce pressure on the right side of the heart.

• Lung or heart transplant:

If the heart or lung gets damaged, the doctor may recommend a transplant. For example, if you are suffering from a severe case of PPH due to a lung disease, your doctor may suggest a lung transplant. If PPH causes damage to your heart as well, or you have severe heart disease or heart failure, you may have to undergo a heart transplant along with a lung transplant.

• Pulmonary endarterectomy:

This medical procedure removes old blood clots from the arteries in the lungs. Pulmonary endarterectomy is suggested for people with chronic thromboembolic pulmonary hypertension.

• Balloon pulmonary angioplasty:

This procedure uses a small balloon to remove the blockage in the arteries and restore blood flow to the lungs. Balloon pulmonary angioplasty is recommended when pulmonary endarterectomy is not suitable for the patient.

4. Healthy lifestyle habits

A healthy lifestyle can also ease the symptoms of the conditions and alleviate the risk of PPH complications. The following are some tips that can help:

• Consume a nutritious diet.

• Stay active and exercise regularly.

• Keep your body weight in check.

• Stay away from tobacco.

• Limit your alcohol intake.

• Get 7-8 hours of restful sleep at night.

• Limit your intake of processed and oily foods.

If you are suffering from the condition, you must ask your doctor about lifestyle changes that can help manage the condition and prevent it from deteriorating further. Since the condition may require regular monitoring and timely treatment, it is wise to keep adequate health insurance or critical illness insurance coverage handy. Having the right health insurance cover will save you from worrying about expenses and help you concentrate on your treatment and recovery.

FAQs

1. What is primary pulmonary arterial hypertension?

Primary pulmonary arterial hypertension is a rare condition that affects the lungs and heart. It happens due to the constriction of the blood vessels in the lungs and increased arterial pressure. PPAH or PPH has no cure and is managed with the prescribed medications.

2. What are the early symptoms of primary pulmonary arterial hypertension?

The early signs and symptoms of primary pulmonary hypertension are shortness of breath while doing daily activities, fatigue, chest pain, and dizziness. However, the symptoms may vary from one person to another. Hence, it is best to talk to your doctor and get the required tests done.

3. What is the difference between high blood pressure and primary pulmonary arterial hypertension?

High blood pressure or hypertension causes constriction of the arteries throughout the body. On the other hand, primary pulmonary arterial hypertension affects the blood vessels in the lungs.

4. Can primary pulmonary hypertension (PPH) run in the family?

Yes, PPH can run in the family, but it is relatively uncommon. Most people who carry the PPH gene may not develop the condition. Only a small number of PPH carriers get diagnosed with the disease.

5. What is the outlook for a person with PPH?

In the past few years, the availability of different treatment options has improved the outlook for PPH patients. However, early diagnosis and timely treatment are important to prolong the life of people with PPH.

Conclusion

Primary pulmonary arterial hypertension is a rare but severe condition that can lead to heart failure if left untreated. Although there is no definite cure, the right treatment and medication can help manage symptoms. PPH is a life-threatening condition, so it is important to opt for timely treatment and care. This is when having a critical illness insurance policy can help. If you get diagnosed with the condition, critical illness insurance for primary pulmonary arterial hypertension will help you cover your medical bills and lessen your financial worries.

Disclaimer: The above information is for illustrative purposes only. For more details, please refer to the policy wordings and prospectus before concluding the sales.

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