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HLHS Diagnosis: The Choices You Face

Updated on October 8, 2013

Hypoplastic Left Heart Syndrome

Diagram Comparing Normal Heart to a Heart with Hypoplastic Left Heart Syndrome
Diagram Comparing Normal Heart to a Heart with Hypoplastic Left Heart Syndrome | Source

What is HLHS?

HLHS, or Hypoplastic Left Heart Syndrome, is a congenital heart defect, wherein the aorta and left ventricle are underdeveloped at birth. Without surgery, this defect is life threatening, because it prevents blood from being properly distributed to the body.

Life-Changing Choices

If you learn your child has hypoplastic left heart syndrome prenatally, there is now the opportunity to undergo surgery in utero to attempt to correct or improve the defect. Albeit hearing the news that your child has Hypoplastic Left Heart Syndrome postnatally may already be much to digest, now you are faced with arduous decisions to make. Physicians will most likely provide you with three choices. One, you can place your child on a heart transplant waiting list. Two, you can opt for a three-stage palliative open-heart surgery, or third, you provide your infant with compassionate care. With the first option, you are gambling that your child is either bumped up to priority on the transplant waiting list and wait for someone else's child to die, or you risk losing your child during a surgery. For the second option, your child undergo three surgeries with the first taking place only days after birth. If you choose compassionate care, you are child will be delivered to you, to take home, and allow nature to take its course.

Your Child, YOUR CHOICE

As the parent of the child pictured in the photograph, the path I chose was a very spiritual and yet a very scientific experience. All of the choices were closely measured; however, we never considered compassionate care. In our personal experience, we chose to gamble on the three-staged palliative surgery, with the first one (Norwood) occurring when our child was only four days old, the second (Hemi-Fontan) occurring at 5 months and the last (Fenestrated Fontan) at age 3 (Pictured).

Our daughter, aside from HLHS is perfectly healthy. Although she did experience some complications during her surgeries, she fought hard and got through them. She is now 9 years old, and an Honor Student in 4th grade. She loves to ride her bike, run, jump, swim and just be a kid. We encourage her to live life to the fullest, but with some caution. She knows that her heart limits some of her activities, but it doesn't define her. Each experience with a congenital heart defect is different, and by no means am I a medical expert. I suggest doing your research as we did, but I encourage you to find strength in our own experience. Each day, EVERYONE in our family counts another day with Rhianna as a blessing. When I think about it, we are all on borrowed time. Nine years is so much more, than had we not thought about fighting for our child's life.

Child, Age 3,  Post-Fontan Surgery
Child, Age 3, Post-Fontan Surgery | Source

The Choices in Detail

The advancements in medicine with regard to treating infants with HLHS have all taken flight in the last thirty years or so. What seemed to have such a high mortality rate is now seen as something that can be manageable and have a higher life expectancy depending on the severity and long-term care. Year after year, medicine continues to evolve into new techniques, which now give heart patients hope.

The Transplant List

While waiting on the transplant provides some families with hope, it is at no lesser cost than death to another loved one's family. To ensure that transplant donations are justly provided, prospective recipients are placed on a waiting list that is prioritized based on urgency and availability of the heart. Keep in mind that until recently, most HLHS babies died within the first month of their lives; There are thousands of people in America, on the heart transplant waiting list.

Three-stage Palliative Surgical Procedures

Norwood Procedure

In this first surgery, the objective is to modify the single ventricle into providing blood flow into the lungs.

Hemi-Fontan Procedure (Bidirectional-Glenn)

The Hemi-Fontan is when surgery is completed to bring oxygen-deficient blood from the upper branches of the body to the lungs. At this surgical stage, more relief is brought to the single ventricle, and the patient's condition is not as delicate.

Fenestrated Fontan Procedure

Lastly, the Fenestrated Fontan Procedure allows oxygen deficient blood from the upper and lower extremities through the lungs solely by the pressure created by the veins.

Compassionate Care

Making the decision to provide compassionate care requires a lot of soul-searching and faith. You are essentially deciding whether you choose to fight the Congenital Heart Defect battle and commit to a life-long journey or allow your child to pass away naturally in the comfort of your home.

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