Pulmonary hypertension is a type of high blood pressure that affects the pulmonary arteries in the lungs, which carry deoxygenated blood from the heart to the lungs. The initial sign is shortness of breath while performing regular routine work. In this condition blood vessels in the lungs are narrowed, blocked, or destroyed. This slows blood flow via the lungs and blood pressure in the lung rises. Heart disease, lung disease and hypoxia are common causes. Prompt diagnosis and treatment can improve the quality of life.

Symptoms

Generally, the signs and symptoms of pulmonary hypertension develop slowly and may be left unnoticed for several months or years. The symptoms get worse as the disease progresses. Some of the symptoms include:

• Blue lips and skin (cyanosis)
• Intense pressure or pain in the chest
• Dizziness or fainting
• Rapid pulse or pounding heartbeat
• Exhaustion
• Shortness of breath (dyspnea)
• Swelling in the ankles, legs and eventually abdomen

Also Read: Accelerated Hypertension: Causes, Symptoms And Treatment

Causes

The heart comprises two upper chambers (atria) and two lower chambers (ventricles). Every time the blood travels via the heart, the lower right chamber (right ventricle) pumps blood to the lungs via a large blood vessel (pulmonary artery). While in the lungs, the blood releases carbon dioxide and takes up oxygen. The blood typically flows easily via blood vessels in the lungs (pulmonary arteries, capillaries, and veins) to the left side of the heart.

But any changes in the cells that line the pulmonary arteries can cause the walls of the arteries to become stiff, swollen, and thick. These changes impede or slow down blood flow via the lungs, causing pulmonary hypertension.

At times the healthcare provider cannot find out the exact reason for high blood pressure in the lungs and the condition is termed idiopathic pulmonary hypertension. Genes may play a significant role in some people getting pulmonary hypertension. While, in a few cases, another condition may be causing the problem. Some of the health problems that can lead to high blood pressure in the lungs include:

• Congestive heart failure
• Blood clots in the lungs
• HIV
• Use of illegal drug use
• Cirrhosis of the liver)
• Lupus, scleroderma, rheumatoid arthritis, and other autoimmune diseases
• Congenital heart problems
• Lung conditions such as emphysema, chronic bronchitis, or pulmonary fibrosis
• Sleep apnea

Types Of Pulmonary Hypertension

According to the World Health Organization (WHO), pulmonary hypertension (PH) is categorised into five groups based on its cause.

Group 1 PH caused due to Pulmonary arterial hypertension (PAH):

This has several causes ranging from underlying diseases to the use of certain drugs. PAH makes the arteries become narrow, thick, or stiff, which results in less blood flowing, thereby raising the pressure in the pulmonary arteries.

Group 2 PH due to left-sided heart disease:

The left side of the heart pumps out blood to the entire system. Any problem on this side of the heart can affect the right side of the heart and the entire pulmonary circuit. This backs up the blood flow in the heart, increasing the pressure in the pulmonary arteries.

Group 3 PH due to lung problem:

Lung issues cause the arteries in the lungs to tighten. Very minimal blood circulation via the lungs raises the pressure in the pulmonary arteries.

Group 4 PH due to obstructions in the lungs:

Blood clots or scars avert blood from flowing normally via the lungs. This eventually puts more stress on the right side of the heart and raises pulmonary blood pressure.

Group 5 PH due to other disorders:

PH develops due to other comorbid conditions like blood disorders and metabolic problems. However, the exact mechanisms of how the condition worsens is not clear.

Also Read: Emphysema: Causes, Symptoms And Treatment

Risk Factors

Pulmonary hypertension is mostly diagnosed in people between 30 to 60 years of age. Some of the other factors that can increase the risk include:

• Hereditary
• Being overweight
• Blood-clotting disorders
• Exposure to asbestos
• Congenital heart disease
• Living at a high altitude
• Use of some weight-loss medicines and illegal drugs such as cocaine or methamphetamine
• Certain serotonin reuptake inhibitors are used for the treatment of depression and anxiety

Diagnosis

Pulmonary hypertension is tough to diagnose initially, as it’s not identified during a routine check-up. Even if the condition is advanced, its signs and symptoms are similar to those of heart and lung conditions. To confirm the diagnosis the doctor will do a complete physical examination, collect a detailed medical and family history, and review the patient's symptoms.

Some of the blood and imaging test suggested to diagnose pulmonary hypertension include:

• Blood works to determine the cause of pulmonary hypertension.
• Chest X-ray
• Electrocardiogram (ECG)
• Echocardiogram
• Right heart catheterization

Other tests that are done to determine the condition of the lungs and pulmonary arteries and help determine the cause of pulmonary hypertension include:

• Computerized tomography (CT)
• Magnetic resonance imaging (MRI)
• Lung (pulmonary) function test
• A sleep study (polysomnogram)
• Ventilation/perfusion (V/Q) scan)
• Open-lung biopsy

Treatment

Though there’s no complete cure for pulmonary hypertension, treatment is available to support ease signs and symptoms and slow the progression of the condition. Generally, the treatments are complex and need extensive follow-up care. When pulmonary hypertension is caused by another condition, then the underlying problem is first treated.

Medications are prescribed by the health care provider to ease symptoms, slow the progression, and avert complications.

Surgery

If medications do not help to manage the symptoms of pulmonary hypertension, surgery may be suggested. Surgeries to treat pulmonary hypertension include:

• Atrial septostomy
• Lung or heart-lung transplant