Idiopathic Pulmonary Arterial Hypertension - Types, Symptoms, Diagnosis, and Management

Introduction:

Idiopathic pulmonary hypertension or IPHT refers to high blood pressure in the heart that passes down to the lung that 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. The word idiopathic means not known. Thus, idiopathic pulmonary arterial hypertension is a kind of pulmonary hypertension that does not have a definite cause or etiological factor behind it.

What Are the Types of Pulmonary Hypertension?

The World Health Organization (WHO) has developed four categories of pulmonary arterial hypertension. Mentioned below are the types of pulmonary hypertension.

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

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

• Group 3 is a type 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 and interstitial lung diseases. Several other naso-oral conditions may also be developed over time.

• Group 4 is a type of pulmonary arterial hypertension that is a direct result of blood clots within the lungs.

• Group 5 is a type of pulmonary arterial hypertension that is simply triggered by an underlying disease.

How Does Blood Flow Between the Heart and the Lungs During Circulation?

The heart has four chambers- two upper sections called the right atrium and left atrium, and two lower sections called the right ventricle and left ventricles. Parallel to four chambers, the heart has four valves- mitral valve, tricuspid valve, aortic valve, and pulmonary valve. The mitral valve or the bicuspid valve allows blood to flow smoothly from the left atrium into the left ventricle. It has two leaflets. The tricuspid valve allows blood to flow easily from the right atrium into the right ventricle. It has three leaflets. The pulmonary valve has the primary function of allowing smooth blood flow from the right ventricle into the pulmonary artery. It has three leaflets.

Mentioned below are the steps of blood flow from the heart to the lungs.

• The lower right section of the heart receives deoxygenated blood and pumps it to the pulmonary arteries.

• This blood moves to the lungs in order to receive a sufficient amount of oxygen.

• It then moves to the left atrium or the upper left section of the heart.

• The blood is now rich in oxygen and moves into the left ventricle or the lower-left section of the heart.

• The blood is then pumped to the rest of the body via the aorta.

• The aortic valve controls smooth blood flow from the left ventricle to the aorta. Aorta is known to be the largest blood vessel of the heart as well as the entire body. It is highly flexible, and is why blood flows out of the heart into the rest of the body. It has three leaflets.

What Are the Signs and Symptoms of Idiopathic Pulmonary Arterial Hypertension?

The clinical manifestations of idiopathic pulmonary arterial hypertension can be understood if the patient is revealing a few or all of the below-mentioned signs and symptoms of pulmonary hypertension.

• 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.

• Asthma

What Are the Influencing Factors That Underline Idiopathic Pulmonary Arterial Hypertension?

Idiopathic pulmonary arterial hypertension is a condition where the blood pressure of the arteries that connect the lungs and the heart is increased for no particulate reason behind the elevation. Mentioned below are a few of the factors that influence idiopathic pulmonary arterial hypertension.

• Age.

• Fitness level.

• Physical activeness.

• Smoking.

• Presence of cardiovascular disease.

• High cholesterol.

• Diabetes mellitus.

• Polycythemia vera.

• Thrombocytopenia.

• Thrombocythemia.

• Vasculitis.

• Sarcoidosis (a multisystem inflammatory disease characterized by the formation of granulomas in various organs, often affecting the lungs and lymph nodes).

• Thyroid.

• Kidney diseases.

• Tumor.

• Glycogen storage disorders.

• Air temperature.

• Standing up.

• Lying down.

• Emotions.

• Body size.

• Medications.

How to Diagnose Idiopathic Pulmonary Arterial Hypertension?

To diagnose a case of idiopathic pulmonary arterial hypertension, the healthcare provider may ask about the signs and symptoms as well as the risk factors that include medical conditions. Being aware of the family history is also necessary in addition to the drug history of the patient. Mentioned below are a few of the diagnostic measures that may be conducted by the health care professional in order to confirm the diagnosis of idiopathic pulmonary arterial blood pressure.

• Medical evaluation.

• Echocardiography.

• Chest X-ray.

• Electrocardiogram.

• Catheterization of the heart.

• Chest CT (computed tomography) scan.

• Chest MRI (magnetic resonance imaging).

• Lung function tests.

• Polysomnogram or PSG.

• Lung ventilation.

• Perfusion scan.

• Complete blood picture.

How to Correct Idiopathic Pulmonary Arterial Hypertension?

It should be noted that pulmonary high blood pressure or idiopathic pulmonary arterial hypertension is a disease that involves the cardiovascular system and thus there are only a few strategies to help drop the blood pressure, but none of these work for a longer time frame since medications and drug therapy is a must. Mentioned below are a few of the ways to correct idiopathic pulmonary arterial hypertension.

• Maintaining body mass index.

• Eating a diet low in sodium.

• Regular exercise.

• Brisk walking.

• Reducing stress.

• Antihypertensive drugs such as diuretics, angiotensin inhibitors, etc.

Conclusion:

Idiopathic pulmonary arterial hypertension is the increase in the blood pressure of the pulmonary artery or the blood vessels that transfer blood between the heart and the lungs. There is no causative agent behind the development of this condition, and thus the name idiopathic is assigned to it. There are, on the other hand, certain factors that influence the condition and have the potential to lead to it. Treatment is done after a definitive diagnosis and medications are prescribed by a healthcare provider, which works successfully. The patient is able to live a life with a positive outlook after the correction of idiopathic pulmonary arterial hypertension.