Definition

Cor pulmonale is a structural and functional change in the right ventricle of the heart due to diseases affecting the lungs' function and/or structure, excluding congenital heart disease or left heart disorders. Right heart failure occurs due to prolonged pressure increase in the pulmonary arteries and the right ventricle.

The primary cause of cor pulmonale is pulmonary hypertension, which is an increase in blood pressure in the pulmonary arteries and the right side of the heart. Right ventricular disease caused by left-sided heart problems is not classified as cor pulmonale. Acute cor pulmonale occurs suddenly due to a lung problem that also arises abruptly, such as in pulmonary embolism or acute respiratory distress syndrome (ARDS). This condition can be life-threatening.

Causes

Circulation of Blood in the Body

The heart is the organ responsible for supplying oxygen-rich blood to the entire body and receiving oxygen-poor blood from the body to deliver it to the lungs. The heart and lungs work closely together in the oxygenation of blood that will be circulated throughout the body. In the lungs, oxygen and carbon dioxide exchange takes place. The oxygen that is inhaled is absorbed into the blood to be distributed to the body.

Right Heart Failure

In newborns, the muscle mass of the right ventricle is equal to that of the left ventricle. This is different from adults, where the left ventricle has more muscle mass than the right ventricle. Causes of cor pulmonale, which include right heart failure due to pulmonary hypertension, can occur but rarely persist in newborns. Typically, right heart failure in newborns occurs due to:

• Below-normal oxygen levels in the blood (hypoxemia)
• Lack of oxygen in body tissues due to blocked blood flow to those tissues (ischemia)
• Acid-base imbalance in the body due to electrolyte disorders (metabolic acidosis)
• Early closure or blockage of the ductus arteriosus in the womb, a blood vessel that connects the aorta and pulmonary arteries.

If there is a lung disorder, blood flow to the left side of the heart may be disrupted, requiring more force from the right ventricle to push blood into the left side. This condition increases the workload of the right ventricle. When the right ventricle can no longer compensate, right heart failure may occur.

Pulmonary Embolism

Acute cor pulmonale is caused by pulmonary vascular disorders, such as a blockage in the blood vessels. The most common blockage is caused by blood clots that travel to and block the pulmonary arteries. The damage can be more severe if the clot is larger.

Causes of pulmonary embolism in children include the insertion of a central venous catheter, which is used to treat certain diseases. In this case, blood clots may form around the catheter. When the catheter is removed, the clot may travel and block blood vessels. If the clot blocks a blood vessel in an organ, it can lead to reduced blood and oxygen flow to that organ, causing organ damage and even death. Other causes of pulmonary embolism include a family history of blood clotting disorders.

Acute Respiratory Distress Syndrome (ARDS)

Acute cor pulmonale can also occur as a complication of ARDS, which is the accumulation of fluid in the air sacs of the lungs. This fluid comes from small pulmonary blood vessels that leak due to disease. ARDS often occurs in children who are already hospitalized for other health issues.

When filled with fluid, the child's lungs become stiff and unable to expand during breathing. Oxygen levels in the blood will drop dramatically, affecting vital organs. If this condition persists, tissue and organs will be damaged, potentially leading to death.

ARDS in children can be directly caused by chest injury, severe lung infection, inhaling harmful substances, burns, drowning, or foreign objects entering the airway, such as food, liquids, or vomit. Indirect causes include blood transfusions, inflammation of the pancreas (pancreatitis), injury leading to low blood pressure, or widespread infection through the bloodstream (sepsis)

Risk Factor

Cor pulmonale can occur at any age. However, in cases of increased blood pressure in the pulmonary arteries in newborns, severe damage to the air sacs (bronchopulmonary dysplasia) is increasingly found as a cause of cor pulmonale in children.

Other factors that may increase the risk of acute cor pulmonale in children include:

• Inhalation of lung-irritating substances
• Head, chest, or other severe injuries that directly affect the lungs or the part of the brain controlling breathing
• Family history of a tendency for blood clots in the lungs
• History of blood clotting
• Severe acute lung infections, such as severe pneumonia, including COVID-19
• Obesity
• Obstructive sleep apnea, where the upper airway collapses repeatedly during sleep
• Genetic factors such as chromosomal abnormalities

Symptoms

Common symptoms of acute cor pulmonale include:

• Difficulty breathing
• Fatigue
• Increased heart rate
• Chest pain or discomfort
• Dizziness
• Swollen legs
• Fainting
• Severe cough
• Coughing up blood
• Wheezing (a high-pitched sound during breathing)

One of the causes of cor pulmonale, ARDS, can present the following symptoms in children:

• Cough, which may produce pink or white sputum
• Confusion and fatigue
• Fever
• Difficulty breathing
• Abdominal pain (in cases of pancreatitis)

Diagnosis

Doctors diagnose cor pulmonale through an evaluation of symptoms, medical history, physical examination, and additional tests. A physical examination aims to detect abnormalities in the heart and lungs.

Additional tests to confirm the diagnosis include:

• Echocardiography (Heart Ultrasound) uses sound waves to create images of the heart
• Electrocardiogram (ECG) provides information about the heart's electrical activity
• Chest X-ray to view the condition of the lungs and heart using X-rays
• Cardiac MRI for a more detailed assessment of the heart, including size, shape, structure, and signs of inflammation
• Cardiac catheterization to assess blockages in blood vessels by inserting a catheter through a major blood vessel in the neck or thigh into the heart

Management

The initial treatment for cor pulmonale is addressing the lung problem causing the acute condition. Treatment for acute cor pulmonale focuses on improving lung oxygenation and restoring right heart function.

Treatment options include medications to lower blood pressure, increase oxygen flow to the lungs, prevent blood clots, remove excess fluid from the body, and oxygen therapy. In severe cases, surgical options such as heart or lung transplantation may be recommended.

Complications

Complications of acute cor pulmonale include:

• Pulmonary artery enlargement
• Thickening of the right ventricular wall
• Excess fluid in the body
• Severe shortness of breath
• Death if not treated promptly

Prevention

Steps to prevent acute cor pulmonale in children include:

• Promptly treating lung infections to prevent worsening
• Avoiding exposure to cigarette smoke and harmful lung irritants
• Maintaining an ideal body weight for the child
• Vaccinating against respiratory infections, such as influenza and pneumonia
• Regular doctor visits if the child has conditions that may trigger acute cor pulmonale

When to See a Doctor?

If your child is at risk or has a condition that could lead to acute cor pulmonale, see a doctor for proper treatment before symptoms worsen. Call emergency services or take your child to the emergency department if they exhibit signs or symptoms of acute cor pulmonale, as mentioned above.

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