Pulmonary Arterial Hypertension - Types, Causes, and Diagnosis

What Is Pulmonary Arterial Hypertension?

Pulmonary arterial hypertension (PAH) refers to high blood pressure in the heart that passes down to the lungs. PAH is responsible for delivering fresh or oxygenated blood to the heart and returning deoxygenated or oxygen-depleted blood to the lungs. Systemic blood pressure is a reading of the pressure exerted by the continuous pumping of the heart. The heart pumps blood and gushes blood within the arteries of the body. On the other hand, pulmonary hypertension refers to the pressure exerted by the blood vessels that connect the lungs.

What Are the Types of Pulmonary Arterial Hypertension?

The World Health Organization (WHO) has categorized pulmonary arterial hypertension into five groups or disorders that present similar symptoms as that of PHT.

• Group 1 is a kind of pulmonary arterial hypertension with no cause and may be inherited. At times, this kind of pulmonary arterial hypertension may result from connective tissue disorders, body infections, such as the human immune virus, sickle cell disease (inherited blood disorder), liver abnormality, congenital heart disease, and the influence of certain medications.

• Group 2 is a kind of pulmonary arterial hypertension that develops due to a disease in the heart's mitral valve. The left side of the heart is most commonly affected by group 2 pulmonary arterial hypertension.

• Group 3 is a kind of pulmonary arterial hypertension that leads to certain issues of the lungs, for instance, chronic obstructive pulmonary disease, known as COPD, as well as sleep apnea (serious sleep-related breathing disorder) and interstitial lung diseases (a group of diseases causing lung scarring). Several other naso-oral conditions may also be developed over time.

• Group 4 is a kind of pulmonary arterial hypertension that directly results from blood clots within the lungs.

• Group 5 is a kind of pulmonary arterial hypertension triggered by an underlying disease.

What Causes Pulmonary Arterial Hypertension?

An increase in the blood pressure of the arteries connecting the heart and the lungs may result from a range of activities as well as physiological disturbances.

Below are a few possible reasons that may result in pulmonary arterial hypertension.

• Aging.

• Stress.

• Obesity.

• Drinking coffee.

• Smoking.

• Pregnancy.

• Standing up all of a sudden.

• Intense physical activity.

• Drinking alcohol.

• Living in a tropical country.

• Certain medications.

• Drugs.

• Anxiety.

• Issues with the heart's conduction system.

• Fear.

• Anemia (lack of healthy red blood cells in the blood).

• Infection.

• Elevated thyroid hormone.

• Dehydration.

• Fever.

What Are the Trigger Factors of Pulmonary Arterial Hypertension?

• Polycythemia vera (disorder causing increased red blood cells).

• Thrombocytopenia (disorder causing too low platelet count).

• Thrombocythemia (disorder causing the bone marrow to make too many platelets).

• Vasculitis (inflammation of the blood vessels).

• Sarcoidosis (growth and collection of tiny inflammatory cells).

• Thyroid.

• Kidney diseases.

• Tumor.

• Glycogen storage disorders.

What Are the Signs and Symptoms of Pulmonary Arterial Hypertension?

The clinical manifestations of pulmonary arterial hypertension can be understood if the patient reveals a few or all of the below-mentioned signs and symptoms:

• Shortness of breath.

• Fatigue.

• Chest pain.

• Increase in heart rate.

• Pain in the right upper side of the abdomen.

• Loss of appetite.

• Feeling light-headed.

• Dizziness during physical activities.

• Fainting.

• Swelling of the ankles or legs.

• Bluish discoloration of the lips.

• Cyanosis (bluish-purple discoloration of nails, skin, lips, and around the eyes).

• Asthma (a lung condition that affects airways).

What Are the Factors That Influence Pulmonary Arterial Hypertension?

Pulmonary arterial hypertension is a condition where the blood pressure of the arteries that connect the lungs and the heart is met. A few of the factors that influence pulmonary arterial hypertension are mentioned below.

• Age.

• Fitness level.

• Physical activeness.

• Smoking.

• Presence of cardiovascular disease.

• High cholesterol.

• Diabetes mellitus (abnormal blood sugar levels).

• Air temperature.

• Standing up.

• Lying down.

• Emotions.

• Body size.

• Medications.

How Is the Diagnosis of Pulmonary Hypertension Made?

To diagnose a case of pulmonary hypertension, the healthcare provider may ask about the signs and symptoms and the risk factors, including medical conditions. Being aware of family history is also necessary.

Mentioned below are a few diagnostic measures that the health care professional may conduct to confirm the diagnosis of pulmonary arterial blood pressure.

• Medical evaluation is done to look for signs and symptoms of PAH and the patient's past medical history.

• Echocardiography - To evaluate the complete structure and function of the heart.

• Chest X-ray -To check the size and shape of pulmonary arteries and heart.

• Catheterization of the Heart - To evaluate pulmonary artery pressures.

• Chest CT (Computed Tomography) Scan - To rule out kidney diseases.

• Chest MRI (Magnetic Resonance Imaging) - To evaluate the right ventricle that pumps blood to the pulmonary arteries.

• Lung Function Tests - To evaluate lung function.

• Polysomnogram or PSG -An overnight sleep test to check for sleep apnea.

• Lung Ventilation or Perfusion Scan -To check for any blood clots in the lungs.

• Complete Blood Picture - To evaluate hormone levels, organ function, and any underlying disorders.

What Is the Management and Treatment of Pulmonary Arterial Hypertension?

Pulmonary arterial hypertension is treated in the following ways:

• Medications - The medications for treating PAH can be given orally, intravenously (IV), and through inhalation or a portable infusion pump. The medications that can be given orally include Bosentan, Selexipag, Ambrisentan, Macitentan, Tadalafil, and Riociguat. Intravenous drugs including Epoprostenol and Iloprost can be given through inhalation. In addition, Treprostinil can be taken orally, IV, infusion pump, or can be inhaled.

• Oxygen Therapy - If one does not have enough blood oxygen level, this therapy is indicated as a supplementation of oxygen during sleep, rest, or exercise.

• Balloon Atrial Septostomy (BAS) - This procedure is primarily used for congenital defects in young children but can also be used in adults to treat pulmonary arterial hypertension. It removes pressure from the left side of the heart to allow the circulation of oxygen in the blood.

• Lung Transplant - This is the last and final option for a person suffering from severe pulmonary arterial hypertension. In this surgery, a person can get one or both of the lungs replaced or transplanted.

How Pulmonary Arterial Hypertension Be Prevented?

Pulmonary arterial hypertension can be prevented by avoiding risk factors, such as street drugs; one must consult the doctor before taking any medication, including diet pills. However, certain risk factors, such as genetic mutations, are beyond one's control, so preventing PAH becomes difficult. Thus, one must try to avoid the risk factors that may cause this condition, such as dietary and lifestyle habits, and must follow up regularly with their doctor.

Conclusion

Pulmonary arterial hypertension has no definite cure. However, individuals can have a better prognosis or outcome of the disease and a fulfilled life with this condition, provided the prescribed medications and lifestyle habits are within limits. Pulmonary arterial hypertension requires strict management. An individual who is suffering from pulmonary arterial hypertension must quit smoking. They must eat a diet rich in fruits and vegetables and low in cholesterol, sugar, and sodium. Maintaining the ideal weight is an added key factor to optimum levels of pulmonary arterial hypertension.