Understanding Pulmonary Hypertension: Symptoms, Causes and Treatments

If the blood vessels in the lungs become thickened, narrowed, blocked, or damaged, it can make blood flow difficult. This leads to increased blood pressure in the lungs, which is called pulmonary hypertension.

Pulmonary hypertension is a type of high blood pressure that impacts arteries in the lungs and the right side of the heart.

In a pulmonary hypertension disorder titled pulmonary arterial hypertension (PAH), the blood vessels in the lungs become restricted, blocked, or damaged. This reduces blood circulation through the lungs, raising the pressure of the arteries in that area. To battle this, the heart has to pump with more effort, eventually weakening the muscle and resulting in failure.

Pulmonary hypertension can be fatal if it worsens over time. While certain types can’t be cured, treatment can still ease symptoms and improve one’s quality of life.

Symptoms

Pulmonary hypertension generally has subtle, growing signs and symptoms that may take months or even years to surface. When the disease advances, the symptoms become more severe.

Signs and symptoms of pulmonary hypertension are as follows:

  • Skin and lips appear blue (cyanosis)
  • Chest pressure or pain
  • Experiencing dizziness or passing out (syncope)
  • Rapid or forceful heartbeat (palpitations)
  • Fatigue
  • Breathing difficulties, initially when exercising and eventually even when at rest.
  • Swelling of the ankles, legs, and, eventually, the abdomen (belly area) can occur.

Causes

The chambers and valves of the heart

A conventional heart is made up of two higher and two lower compartments. The special chambers, the left, and right atria take in incoming blood. The more powerful lower chambers, the left, and right ventricles push the blood out of the heart. The cardiac valves that ensure the blood flows in the correct direction are gates close to chamber entrances.

Usually, a heart has two upper chambers (atria) and two lower chambers (ventricles). The right ventricle pumps blood to the lungs via the pulmonary artery each time the blood passes through the heart.

Oxygen is taken up and the blood releases carbon dioxide as it flows through the pulmonary arteries, capillaries and veins in the lungs, whereafter it goes to the left side of the heart.

However, alterations in the cells that line the pulmonary arteries can lead to walls that are stiffer, swollen and thicker. This can make it difficult for blood to move through the lungs, resulting in pulmonary hypertension.

Pulmonary hypertension is categorized into five groups based on the underlying cause.

Group 1: High blood pressure in the arteries of the lungs (PAH)

Causes include:

  • Pulmonary arterial hypertension with an unknown cause (idiopathic)
  • Pulmonary arterial hypertension is inherited due to changes in a gene passed down within families
  • Consuming certain drugs or illicit substances
  • Heart conditions present from birth (congenital heart disease)
  • Besides the issues above, HIV infection, liver cirrhosis, and connective tissue disorders such as scleroderma and lupus are also concerns.

Pulmonary hypertension caused by heart ailments on the left side of the heart belongs to Group 2.

  • Conditions such as mitral and aortic valve disease on the left side of the heart.
  • Malfunction of the left ventricle, which is the lower left heart chamber

Group 3: Hypertension in the lungs caused by diseases of the lungs.

  • Long-term obstructive lung disease (COPD)
  • Scarring of the lung tissue in the air sacs (pulmonary fibrosis)
  • Obstructive sleep apnea
  • People more prone to pulmonary hypertension may experience adverse effects from long-term exposure to high altitudes.

Group 4: Pulmonary hypertension from chronic clotting of the blood

  • Chronic pulmonary emboli, which are blood clots in the lungs
  • Other clotting disorders

Group 5: Pulmonary hypertension caused by other health issues

  • Conditions such as polycythemia vera and essential thrombocythemia are blood disorders.
  • Sarcoidosis and vasculitis, which are inflammatory disorders
  • Metabolic conditions, such as glycogen storage disease
  • Kidney disease
  • Tumors making contact with pulmonary arteries

Pulmonary hypertension and Eisenmenger syndrome

Eisenmenger syndrome is a form of congenital heart disease that leads to pulmonary hypertension, mainly resulting from an extensive ventricular septal defect (a hole between the heart’s two lower chambers).

When this opening in the heart exists, oxygen-carrying blood (red) and oxygen-poor blood (blue) flow through. This disrupts average circulation and causes higher pressure in the pulmonary arteries due to the extra blood returning to the lungs instead of going to other parts of the body, leading to pulmonary hypertension.

Risk factors

Pulmonary hypertension is most often diagnosed in individuals aged 30 to 60. However, aging increases the risk of developing Group 1 pulmonary hypertension; idiopathic PAH is more frequently found among younger adults.

Factors that can increase the chance of pulmonary hypertension include:

  • A record of the ailment in the family
  • Being overweight
  • Disruptions to blood clotting or a history of lung clots in the family
  • Exposure to asbestos
  • Congenital heart disease
  • Residing at a high elevation
  • Use of specific drugs like some weight-loss medicines and illegal drugs like cocaine or methamphetamine
  • SSRIs, which are used to treat depression and anxiety, are utilized.

Complications

Possible risks associated with pulmonary hypertension include:

  • The exemplary region of the heart enlarges and eventually fails due to heart failure (cor pulmonale). With cor pulmonale, the right ventricle in the gut gets larger. It is forced to put forth more effort than usual to circulate blood through narrowed or impeded pulmonary arteries. Subsequently, the heart’s walls strengthen, and the right ventricle becomes bigger to accommodate more blood. Yet these modifications place extra demand on the heart, leading it to failure.

Cor pulmonale is marked by an enlargement of the heart’s lower right chamber (ventricle) needed for pushing blood across narrowed or obstructed pulmonary arteries. This causes right-sided heart enlargement and heart failure.

The heart’s walls become thicker, and the right ventricle expands to hold more blood, but this extra strain leads to eventual failure of the right ventricle.

  • Pulmonary hypertension can increase the danger of blood clots forming in the small arteries of the lungs.
  • Pulmonary hypertension can cause dangerous arrhythmias that manifest as irregular heartbeats.
  • Bleeding within the lungs is a risk with pulmonary hypertension, and it can even be fatal. Hemoptysis is another symptom associated with this condition, which involves coughing up blood.
  • Pregnancy is at risk if pulmonary hypertension is present. It can be fatal for the fetus.
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