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What is a Hypo Heart Condition?

Published in Congenital Heart Disease 2 mins read

A "hypo heart condition," more accurately referred to as hypoplastic left heart syndrome (HLHS), is a severe congenital heart defect where the left side of the heart is significantly underdeveloped.

Here's a breakdown of what that means:

  • Congenital Heart Defect: This means it's a heart problem present at birth.
  • Hypoplastic: This term signifies underdevelopment of a tissue, organ, or body part. In this case, it's the left side of the heart.
  • Left Side of the Heart: Normally, the left ventricle (the main pumping chamber on the left side) pumps oxygen-rich blood to the aorta, the body's largest artery. The aorta then distributes this blood throughout the body.

Key Characteristics of HLHS

  • The left ventricle is very small and may not be able to pump enough blood to meet the body's needs.
  • The mitral valve (between the left atrium and left ventricle) and the aortic valve (between the left ventricle and aorta) may also be small or closed.
  • The aorta itself is often smaller than normal.

Why HLHS is a Problem

Because the left side of the heart is underdeveloped, it cannot effectively pump blood to the body. Babies with HLHS need intervention shortly after birth to survive. Typically, this involves multiple surgeries to redirect blood flow and allow the right ventricle to pump blood to both the lungs and the body.

Treatment

Treatment for HLHS typically involves a series of three open-heart surgeries:

  1. Norwood Procedure: Performed shortly after birth. Creates a new aorta connected to the right ventricle.
  2. Glenn Procedure (or Hemi-Fontan): Performed at a few months of age. Connects the superior vena cava (a major vein draining blood from the upper body) directly to the pulmonary artery.
  3. Fontan Procedure: Performed between 2 and 5 years of age. Connects the inferior vena cava (a major vein draining blood from the lower body) to the pulmonary artery.

The goal of these surgeries is to allow the right ventricle to function as the main pumping chamber for both the lungs and the body. In some cases, heart transplantation may also be considered.

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