Author Question: Transplanting an Infant with a Congenital Heart Defect

Erin Asks:

I stumbled across your blog earlier this week and you have no idea how incredibly excited I was to have made the discovery. I’ve been devouring your recent blog posts and I am so impressed with the details of your answers. You are amazing!

I recently finished the first draft of a novel, tentatively titled The Blood Farm. It is a story about what happens to society when we begin to view one another as commodities.

I have tried to do my due-diligence in researching medical practices, specifically organ transplants, but I’m not confident in all the details.

Here is my question:

In the scene, an unborn baby is diagnosed with multiple genetic defects (including hypoplastic left heart syndrome) that have caused her organs not to form correctly. She is scheduled to undergo a heart and double-lung transplant following birth. The mother goes into pre-term labor and the baby’s outlook is dire.

What would the procedure be for prepping this baby for transplant surgery?

Jordyn Says:

Hi Erin! Thanks for all the compliments regarding my blog. I’m so glad you have found it to be a useful resource.

First of all, for the blog readers, a simple explanation of hypoplastic left heart syndrome (HLHS) is that the baby is born with an underdeveloped left ventricle. The left ventricle is the primary part of the heart that propels blood out to the body and is larger in size than the right ventricle. If the heart’s strongest chamber is weak, it’s easy to see how this can be problematic for life. Also, this is generally not the only thing wrong with the heart when there is this congenital heart defect.

There are two approaches to the management of Hypoplastic Left Heart Syndrome. One is a three-staged operation over the child’s first few years of life and the other is transplant, but the first surgery will still be required to save the baby’s life even if going for transplant.

First thing to know about HLHS is that it is a ductal dependent lesion which means that the way the baby’s heart formed in utero is required post birth. When a baby is born— two holes in the heart close— one (the PDA) pretty early. We need to keep the PDA open for these infants so a drug called Prostaglandin E is given as a continuous IV infusion to keep this hole from closing.

Most often, babies with this condition are known prior to birth so once they are born they could be intubated depending on their work of breathing and oxygen levels. These infants have lower than normal oxygen levels and don’t have normal oxygen levels until after their third surgery, but they are always started on Prostaglandin.

The plot you propose regarding a heart transplant for a newborn infant is very tricky. It will take months to find a heart for this baby. The earliest a doctor friend of mine had heard about transplanting infant’s like this was at three months of age. As I mentioned, there is a three-stage operation that can also be done with survival rates at about 70%. From what I can tell from this article this matches the transplantation survival rate. However, the ethical argument, due to the limited availability of donated infant hearts, is that the three staged operation should be used in these infants to save those limited infant hearts for children with other conditions for which there is no other treatment.

Babies born with HLHS will not leave the hospital until their first surgery is done.

So, all this to say, I’m not sure HLHS would be the best condition to give this baby.

You might want to consider some of the hypertrophic cardiomyopathies. This pamphlet would be a good place to start.

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Author Beware: Proof’s Problem with HIPAA

Proof (not to be confused with my debut medical thriller with the same title) is a medical drama starring Jennifer Beals as renowned cardiothoracic surgeon Dr. Carolyn Tyler.

Dr. Tyler is recruited by billionaire Ivan Turing to investigate near death experiences (NDEs) as he is soon to face the other side due to a terminal cancer diagnosis.

Tyler is a skeptical atheist and believes death is the end— even though she’s had a NDE herself and longs to reconnect with her teenage son who died in a car accident.

Of course, Turing uses his wealth and a big donation to the hospital to obtain Tyler’s cooperation.

Through the course of her investigations, nearly every religious permutation of the after life is explored— past lives, reincarnation, and soul jumping among them.

The issue becomes when families become aware of Tyler’s investigations and want information that in real life she should never disclose. They’re clearly HIPAA violations. I’ve blogged extensively on HIPAA here, here, and here.

Why is HIPAA so important? It is the law. It’s what healthcare workers are instructed (pounded into the head) to protect every single day. It’s not taken lightly. Medical people have been fired for violating a patient’s privacy by disclosing healthcare related information.

However, the television show Proof seems to not understand what HIPAA entails.

In one instance, a mother who lost her son begins to believe his soul has inhabited another child’s body because he has the same rare blood type, same rare heart condition, and was a piano playing genius. The mother latches onto him and offers to pay for his medical care.

It becomes a sticky situation because the mother who lost her child begins to overstep her bounds and Dr. Tyler begins to believe she’s at risk for kidnapping this other boy over the loss of her son.

To prevent her from taking that step, she begins to list a litany of medical reasons why this patient isn’t her son. The problem is, this mother has no right to any of this information. It is a HIPAA violation.

In another instance, Dr. Tyler convinces a wife to donate her brain dead husband’s heart. Now, she has a vested interest in this happening because one of her patient’s with a rare blood type (evidently everyone in this show has a rare blood type) has been waiting for a heart for years and is running out of time.

The wife agrees and the heart is transplanted but the patient nearly rejects the heart. When the wife of the heart donor catches wind that this has happened (she seems to be hanging around the hospital after the donation has occurred) Dr. Tyler gives her detailed medical information on how the patient who received her husband’s heart is doing.

Again, this wife, even though she donated her husband’s heart, has no right to this information. In fact, donor and recipient identities are highly protected. It’s not that these families never meet, but it usually happens months after and is coordinated by the organ bank and not doctors on site.

In fiction, you can break the rules. Healthcare workers can disclose medical information but they should also face a consequence for it just like we do in real life. The plus, it dramatically increases the tension which is always the goal of any work of fiction.