Hypoplastic Left Heart Syndrome (HLHS)

A customer came to me with a special request. Her friend had just had a son born with hypoplastic left heart syndrome (HLHS). He was scheduled to have three surgeries, starting immediately and continuing through his first few years of life. She wanted me to paint a representation of what his heart would look like after completing these surgeries, as a gift to her friend: "I can just imagine [her son] telling his friends one day this is what my heart looks like!" I was honored to paint the piece, and was fascinated (and inevitably saddened) to learn more about HLHS throughout the process. And now, lucky you, I get to share all that info.

What is HLHS?

HLHS is a congenital heart defect where the left side of the heart is severely underdeveloped. Typically the left side of the heart accepts oxygen-happy blood from the lungs and pumps it out to the rest of the body. With HLHS, the left side of the heart is unable to carry out its role and blood flow to the body is heavily restricted. Babies with HLHS are born cyanotic (have bluish skin) and will require complicated surgical intervention shortly after birth in order to survive.

What is the prognosis?

Outlook for HLHS babies is much better than it used to be, thanks to advancements in care.  Immediate (sometimes even in-utero!) and ongoing surgery work to make the heart much more effective in providing the body with blood. If the child survives the three required surgeries, he or she will have a decent chance of making it to adulthood. However, these interventions should not be mistaken for a cure; lifelong complications will occur. And if the HLHS is very complicated (it varies from person to person), a heart transplant may be necessary.

Tell me more about these surgeries.

  1. The Norwood Procedure
    This first procedure is done within two weeks of the baby's birth. Surgeons actually create a new aorta and attach it to the right ventricle, and also provide a tube from the right ventricle that goes straight to the lungs. This allows the right side of the heart to do all the work, pumping blood to both the lungs and to the rest of the body. It is an incredibly difficult and lengthy surgery. Afterwards, the infant still may be cyanotic due to oxygen-rich and oxygen-poor blood still mixing in the heart.
  2. The Bi-directional Glenn Shunt Procedure
    This operation is performed four to six months later. It serves to provide a connection between the pulmonary artery (artery going to the lungs) and the superior vena cava (vein returning oxygen-poor blood from the upper body). This surgery gives the over-worked right ventricle a break by allowing blood returning from the body to flow directly to the lungs.
  3. The Fontan Procedure
    This final surgery is typically done between 18 months and three years of age. Doctors connect the pulmonary artery and the inferior vena cava (returning oxygen-poor blood from the lower body). This allows the rest of the blood coming back from the body to go directly to the lungs. Once this procedure is complete, oxygen-rich and oxygen-poor blood are no longer mixing in the heart and the child's skin will no longer be cyanotic.

The tl;dr...  basically, these surgeries serve to completely re-plumb the heart by disconnecting the vena cava from the heart and re-route it to go directly to the lungs to get oxygenated. This oxygenated blood can then go to the heart and get pumped out to the body by the right ventricle.

Anything I can do to help?

My heart just aches for the families while I research and write this post. Can you imagine? Watching your newborn child go through the most complicated of surgeries, knowing that if he or she survives, the outlook is only okay and more surgeries are in the future. That's why my biggest advice is simply sympathy and empathy. I recently saw this little note on things to say to preemie moms, and I think it still works for any parent dealing with a child with medical complications:

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But if you would like to donate to research and awareness of congenital heart defects, please consider http://www.childrensheartfoundation.org/donate-now

 

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