Hypoplastic Left Heart Syndrome (HLHS) in Newborns

June 27, 2024
Janelle Thomas MSN, RN
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Having a loved one diagnosed with Hypoplastic Left Heart Syndrome (HLHS) can cause feelings of overwhelm, fear, and uncertainty. We know you love your child and want the best for them. The good news is that learning about HLHS is half the battle. Understanding the ins and outs of this condition gives you the power to help your child through this difficult time. Let’s get started by learning more about HLHS. 

What is Hypoplastic Left Heart Syndrome?

Hypoplastic Left Heart Syndrome is a severe congenital heart defect in which the left side of the heart is critically underdeveloped. This includes the left ventricle, mitral, aorta, and aortic valves. The condition prevents the left side of the heart from effectively pumping blood to the body, forcing the right side to compensate by pumping blood to both the lungs and the rest of the body.

Hypoplastic Left Heart Syndrome Symptoms

Symptoms of HLHS are noticeable soon after birth and may include:

  • Blue or gray tint to the skin (cyanosis)
  • Rapid, difficult breathing
  • Cold hands and feet
  • Poor feeding
  • Tiredness/Lethargy

Hypoplastic Left Heart Syndrome Causes & Risk Factors

The exact cause of HLHS is unknown, though it likely involves a combination of genetics and environmental factors. Risk factors may include a family history of heart defects or genetic conditions.

Complications of Hypoplastic Left Heart Syndrome

Complications of HLHS can be life-threatening and require immediate intervention. These include heart failure, difficulty breathing, and developmental delays due to reduced blood flow and oxygen delivery to the body.

Diagnosing Hypoplastic Left Heart Syndrome

Diagnosing HLHS typically occurs either prenatally through an ultrasound or shortly after birth based on the newborn’s symptoms and an echocardiogram, which can detail the structure and function of the heart.

Treatment Options for Hypoplastic Left Heart Syndrome

Treatment for HLHS requires a series of surgeries to reroute the blood flow, typically conducted in these three stages:

  1. Norwood Procedure: Performed shortly after birth to create a new pathway for blood flow.
  2. Glenn Operation: Conducted when the child is a few months old to connect half of the venous blood flow directly to the lungs.
  3. Fontan Procedure: Completed around 2-3 years of age to direct the remaining venous blood to the lungs.
  4. Home Health Care: At Care Options for Kids, we offer home healthcare that is like family. We have seasoned nurses who offer support and understand the importance of HLHS. 

In some cases, a heart transplant may be considered as an alternative to the three-stage surgery.

Support Available Through Care Options for Kids

At Care Options for Kids, our specialized pediatric home health care includes:

  • Individualized Care Plans: Customized to meet each child's specific medical and developmental needs.
  • Skilled Nursing Care: Our pediatric nurses provide daily care, monitor heart function, and support post-operative recovery at home.
  • Family Education: Teach families how to care for a child with a heart condition, recognize signs of potential complications, and manage everyday care needs.

Emotional and Practical Support for Families

Facing HLHS can be an emotional rollercoaster for families. Some additional support may include: 

  • Healthcare Navigation Assistance: Guidance through the complexities of healthcare management, from understanding medical jargon to handling insurance issues.
  • Community Resources: Connecting families to local and national support networks and resources tailored to children with severe health conditions.

Understanding and managing Hypoplastic Left Heart Syndrome is a demanding journey, but you are not alone. Care Options for Kids provides the necessary support, care, and expertise to help your family navigate this challenging path. Our dedicated team ensures that your child receives the best possible care in the comfort and familiarity of your home.

Schedule Your Child’s Care Assessment Today

At Care Options for Kids, we understand the unique challenges of caring for a child with basic to medically-complex health conditions. Our dedicated team of pediatric home health nurses is here to support your family with compassionate, expert care tailored to your child’s needs. Contact us today to schedule an assessment and learn how we can help you navigate this journey with confidence and care.

Click here to start your journey to better care.