Introduction

Pulmonary hypertension (PH) means you have high blood pressure in your pulmonary arteries. These blood vessels transport oxygen-poor blood from your heart to your lungs. Pulmonary hypertension poses a danger because it interferes with blood flow through your heart and lungs. High blood pressure in your pulmonary arteries makes these arteries narrow. As a result, your heart needs to work harder to pump oxygen-poor blood to your lungs. Pulmonary hypertension damages your heart and causes problems throughout your body over time. Without treatment, it can lead to death.

What are the Different kinds of Pulmonary Hypertension?

The World Health Organisation (WHO) splits pulmonary hypertension into five categories based on the reasons behind it: Group 1 - Pulmonary Hypertension because of pulmonary arterial hypertension (PAH): Diseases, certain drugs, or unknown causes make lung arteries stiff or narrow, which raises blood pressure. Group 2 - PH because of left-sided heart disease: Problems in the left side of the heart lead to blood backing up, which raises pressure in the arteries of the lungs. Group 3 - Lung disease or hypoxia causing PH: Some lung problems make the arteries in your lungs squeeze tight. This means less blood can move through your lungs, which bumps up the pressure in your pulmonary arteries. Group 4 - Blockages in your lungs causing PH: Blood flow in the lungs is blocked by leftover clots or scar tissue. This puts extra strain on the right side of your heart and raises the blood pressure in your lungs. Group 5 - PH due to other disorders: PH happens along with other conditions like blood disorders and metabolic disorders.

Symptoms and Causes

The first sign of pulmonary hypertension is difficulty breathing during your everyday tasks. When pulmonary hypertension begins, you might not notice any signs. As symptoms start to appear, they may seem minor. But PH symptoms worsen with time making your usual activities more challenging. As PH advances, you will experience shortness of breath even when you are not active. Other signs include:

• Blue tint on your skin or lips.
• Pain or tightness in your chest.
• Feeling lightheaded or passing out.
• Tiredness.
• Less appetite than normal.
• Ache in the top right part of your belly.
• Heart beating fast.
• Puffiness (oedema) in your ankles, legs or stomach.
• PH signs make it tough to work out or do your everyday tasks.

What Causes Pulmonary Hypertension?

The causes of pulmonary hypertension differ based on the type of PH you have. These causes include various diseases and underlying conditions as well as exposure to environmental factors (toxins & drugs). Group 1 Pulmonary arterial hypertension has many causes. These include:

• Congenital heart disease.
• Diet pills
• Genetic mutations.
• Glycogen storage diseases.
• HIV.
• Liver disease.
• Lupus.
• Portal hypertension.
• Pulmonary capillary hemangiomatosis.
• Pulmonary veno-occlusive disease.
• Schistosomiasis.
• Scleroderma.
• Recreational drugs like methamphetamine.
• Some people get PAH without any obvious reason (idiopathic PH)

Group 2- PH Left-sided heart issues that can bring about pulmonary hypertension include:

• Aortic valve disease.

• Left-sided heart failure.

• Left ventricular hypertrophy.

• Mitral valve disease. Group 3 - pulmonary hypertension Lung problems that can result in pulmonary hypertension include:

• Chronic obstructive pulmonary disease (COPD)

• Interstitial lung disease

• Obstructive sleep apnea.

Group 4 PH Chronic thromboembolic pulmonary hypertension (CTEPH) typically causes this form of PH. CTEPH has an impact on the arteries in your lungs resulting in blood clots and scarring.

Group 5 PH These conditions include:

• Gaucher disease.
• Kidney disease.
• Langerhans cell histiocytosis.
• Sarcoidosis.
• Thyroid disease
• Cancerous or benign tumours

How is Pulmonary Hypertension Diagnosed?

• To diagnose pulmonary hypertension, your doctor will examine you and run tests.
• These tests check the blood pressure in your lung arteries
• Right heart catheterisation: Doctors also call this test pulmonary artery catheterisation. It checks the pressure inside your lung arteries and measures how much blood your heart pumps each minute.
• Doppler echocardiogram: A Doppler echo measures blood circulation through your heart valves. This test helps your doctor figure out your systolic pulmonary artery pressure.
• These tests look for what's causing pulmonary hypertension:
• Blood tests: Doctors use these tests to find infections hidden illnesses, or signs of stress on the heart.
• Chest CT scan: This produces clear images of the heart and lungs to identify clots or problems in their structure.
• Chest X-ray: It shows the size of the heart, any fluid buildup or changes in the lungs.
• Pulmonary ventilation/perfusion (VQ) scan: This test helps spot blood clots or detect uneven airflow and blood circulation in the lungs.

Management and Treatment

The type of pulmonary hypertension you have and your other health issues determine your treatment. Your doctors will create a plan that fits your specific needs. A. Treatment for pulmonary arterial hypertension (PAH) includes:

Medications

Calcium channel blockers reduce the blood pressure in your pulmonary arteries and throughout your body. Diuretics are used to get rid of excess fluid. Pulmonary vasodilators help your pulmonary arteries relax & open up more. Oxygen therapy: You might need this treatment if your blood lacks sufficient oxygen.

B. CTEPH treatment includes:

Medication:

• A soluble guanylate cyclase stimulator (SGCS) slows down how the disease progresses.
• Anticoagulants help prevent blood clots.
• Balloon atrial septostomy (BAS): Doctors use this procedure for babies with critical heart defects. However, it also has an application for adults with pulmonary hypertension. It serves as a bridge to keep you stable while you wait to get a lung transplant.
• Balloon pulmonary angioplasty (BPA): This catheter-based procedure uses a balloon to widen your pulmonary artery.
• Pulmonary endarterectomy (PEA): This operation takes out blood clots from your lungs.
• Doctors treat PH from heart or lung issues by addressing the root causes. Since many heart and lung problems lead to PH, each person's treatment plan differs. Discuss with your doctor to find out what's right for you. Your doctor might suggest:
• Changes to your diet.
• Adjustments to your lifestyle.
• Medicine to control high blood pressure or heart failure.
• Therapy with oxygen.
• Operations like repairing or replacing heart valves.
• For some people with severe PH, a lung transplant is the final option.