Pulmonary arterial hypertension occurs when there is abnormally elevated pressure in the blood vessels leading from the heart to the lungs. It is a rare but critical disease which causes the pulmonary arteries to become narrow and stiff. It can lead to enlargement and weakening of the heart, ultimately causing right heart failure or other life-threatening consequences. It is difficult to determine the causes of pulmonary arterial hypertension but research has identified certain key factors.

Causes of Idiopathic Pulmonary Arterial Hypertension

The medical term “idiopathic” means the cause of the disease is unknown. Doctors and physicians use “idiopathic pulmonary arterial hypertension” term when they have no explanation of its cause. This condition is exceedingly rare and difficult to treat since the underlying cause remains concealed.

Causes of Inherited Pulmonary Arterial Hypertension

Pulmonary arterial hypertension can be hereditary In this case, physicians conduct a genetic test to search for the defective gene. Early and routine screening is important for individuals with a family history of the condition as the disease tends to get more severe with each generation.

Causes of Persistent Pulmonary Arterial Hypertension Related to Newborn Babies

This type of hypertension occurs in about two in every thousand infants or young children. It is caused due to the underdevelopment of lungs known as pulmonary hypoplasia, abnormally low level of glucose in the blood known as hypoglycemia, sepsis, meconium aspiration syndrome when the baby breathes in a mixture of amniotic fluid and feces, or genetic mutations.

Left Heart Disease as a Cause of Pulmonary Arterial Hypertension

Pulmonary hypertension is most commonly related to left heart disease. About 60% of patients that suffer from severe left ventricular dysfunction, such as a mitral valve or long-term high blood pressure end up developing this condition.

Lung-related Disease as a Cause of Pulmonary Arterial Hypertension

Hypoxia lung disease, which occurs due to a shortage of oxygen is one of the leading causes of pulmonary arterial hypertension. Other illnesses that can lead to the condition include chronic obstructive pulmonary disease (COPD), interstitial lung disease, and sleep-disordered breathing. It is necessary to treat the primary disease first and then pulmonary arterial hypertension.

Blood Clots as Causes of Pulmonary Arterial Hypertension

When there is an injury or cut, blood clots are the body’s natural way to stop internal bleeding. However, when clots appear due to no reason, it narrows or blocks the pulmonary arteries. This eventually leads to pulmonary arterial hypertension or pulmonary embolism.

Other Causes of Pulmonary Arterial Hypertension

Less common reasons and conditions for the condition include:

• Sarcoidosis — an inflammation of different organs like lungs and lymph nodes
• Histiocytosis X — a rare disease caused by scarring, granulomas, and air-filled cysts in the lungs
• Rare blood conditions like pulmonary veno-occlusive disease or pulmonary capillary hemangiomatosis
• Scleroderma
• Congenital heart problems
• HIV
• Certain medications or drugs
• Thyroid gland disorder
• Sickle cell disease
• Glycogen storage disorders
• Compression of blood vessels in the lungs, which may occur due to tumors.