Hypoplastic Left Heart Syndrome (HLHS)

What is Hypoplastic Left Heart Syndrome (HLHS)?

Hypoplastic Left Heart Syndrome (HLHS) is a congenital heart defect where the left side of the heart does not develop fully during pregnancy.

This condition affects normal blood flow through the heart, leading to severe complications shortly after birth if not treated promptly.

Side Effects of Hypoplastic Left Heart Syndrome (HLHS)

The underdevelopment of the left side of the heart results in several significant side effects:

• Cyanosis: Bluish discoloration of the skin and lips due to reduced oxygen levels in the blood.
• Breathing difficulties: Infants may experience rapid or labored breathing.
• Poor feeding: Difficulty in feeding due to limited oxygen supply to the body.
• Heart failure: Inadequate pumping of blood can lead to heart failure and other complications.

How is Hypoplastic Left Heart Syndrome (HLHS) Diagnosed?

HLHS is often diagnosed during pregnancy through fetal echocardiography, a specialized ultrasound that examines the baby’s heart. After birth, symptoms such as cyanosis and breathing difficulties may prompt further diagnostic tests including:

• Echocardiogram: Detailed ultrasound of the heart to assess its structure and function.
• Electrocardiogram (ECG): Records the heart's electrical activity to detect abnormalities.
• Cardiac catheterization: Invasive procedure to evaluate the heart's chambers and blood vessels.

Potential Treatment of Hypoplastic Left Heart Syndrome (HLHS)

Treatment for Hypoplastic Left Heart Syndrome typically involves a series of surgical procedures and ongoing medical management:

• Norwood procedure: The first stage involves restructuring the heart to allow the right ventricle to perform the work of the left ventricle.
• Bidirectional Glenn procedure: The second stage connects the veins carrying oxygen-poor blood directly to the lungs.
• Fontan procedure: The final stage directs all blood returning from the body to the lungs without passing through the heart.

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