Ventricular Septal Defect Case Study

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This module uses a real case study about a baby named Caleb who was born with a serious heart defect. The case is medically accurate and will help us understand how heart anatomyThe study of the structure of the human body., tissue structure, blood flow, and electrical conductionThe transmission of nerve impulses along neurons. all work together. However, I want you to know that this case has a tragic outcome. If you feel you need to step away at any point during our discussions, please feel free to do so. We’ll also have opportunities to discuss the emotional aspects of being healthcare providers.

Tiffaney is worried about her newborn son. Ever since she brought Caleb home from the hospital, it has been so hard to get him to eat. He seems to be breathing too hard all the time.  Although Tiffaney and her family have very lightly-colored skinThe body’s largest organ, providing protection and regulation. tones, Caleb seems abnormally pale.  She stopped breast-feeding and tried every bottle and formula on the market, but nothing has worked. So, at his one month check-up, her stomach is in knots as they place Caleb on the scale. The nurse says, “9 pounds, 7 ounces.” Tiffaney realizes Caleb has only gained one pound since he was born and she breaks into tears.

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Dr. Baker checks over Caleb in the exam room, taking extra time feeling and listening to his chest. During the exam, Tiffaney explains her struggle with trying to get her son to eat. It seems as though he can’t feed and breathe at the same time.  He cries almost the entire day.

Murmur?

After the exam Dr. Baker says, “When I listen to Caleb’s heart I hear an extra sound called a murmur. Murmurs are usually due to faulty valves of the heart. Sometimes they are due to other abnormalities or obstructions in the way of blood flow.  Caleb’s murmur is a systolic murmur. He might have a faulty mitral valve. This is quite common.  I want to use an echocardiogram to get a good picture of all the parts of his heart.”

Dr. Baker reviewed the test results for Caleb, with specific interest into the echocardiogram (see below). It was as Dr. Baker had feared: Caleb had a ventricular septal defect. This defect is a hole in the interventricular septum. The septum of myocardium separates the left and right ventricles. It prevents the mixing of blood between the sides of the heart (remember, paint fence, not paint house). Dr. Baker looked through more results. These included a CBC, a metabolic panel, and an EKG. He came back to the interpretation of the echocardiogram. Little Caleb’s ventricular septal defect was 7 mm and considered to be in a range that requires surgery to fix. If it were smaller, there’d be a 90% chance it would close on its own. If not repaired soon, damage to the lungs could occur. This could reduce Caleb’s chances of making it to the age of 25 by about 60%. Dr. Baker thought carefully how to communicate with Tiffaney with empathy.

Tiffaney and her mother have a follow up Dr. Baker in a virtual visit. As they wait for Dr. Baker to join the meeting, Tiffaney’s mom grabs her hand. She reassures her by saying, “Honey, it’s going to be OK whatever it is.”

Doctor Baker joins the meeting and asks Tiffaney and her mom how they are and how things have been with Caleb since their in-office visit. Dr. Baker recognizes the care-giver fatigue in Tiffaney’s reluctant answer. Dr. Baker says, “After a careful review of all the information, I have discovered that Caleb’s murmur is not from a faulty valve, but from another inconsistency in blood flow through his heart.  Caleb has a hole in the heart muscle wall between his right and left ventricles which are these large chambers in the heart that produce the force of the pump. We call it a ventricular septal defect (VSD).” As Tiffaney’s eyes welled up with tears, her mom reached for a tissue and pressed it into Tiffaney’s hand.

His oxygenated and deoxygenated blood is mixing because of this hole. This makes him feel as though he’s not getting enough air.  Tiffaney looked up and through tears said, “Is that why he doesn’t want to feed? He’s trying to get air instead of food?” Dr. Baker agreed and took the opportunity to continue.

“Small to moderately sized VSDs close on their own but Caleb’s hole is 7 mm in diameter, which is similar to about a third of an inch.” Dr. Baker picks up a small yellow ruler from their desk and holds it up to show Tiffaney the approximate diameter of the hole. “Caleb will need surgery to fix the VSD. Let’s get you to see a pediatric surgeon in the next few days and schedule a surgery.” Tiffaney carefully said, “I feel guilty that I’m relieved to know that it’s this horrible thing my son needs surgery for.” Dr. Baker explained about care-giver fatigue, involving Tiffaney’s mom in the conversation and setting a few goals for Tiffaney to have some breaks from Caleb’s care. Tiffaney’s mom offered to hire a night nurse for Tiffaney to which Dr. Baker replied, “Great thinking! Caleb is our patient, but Tiffaney and mom, your well-being is my concern so that you can give Caleb the care he needs. You’ve been doing that so well, but it’s hard over a long period of time. There’s no need to feel guilty in finding a break or relief in any part of this situation.”

Caleb undergoes surgery for the VSD. Unfortunately, the VSD proved to be larger than expected by the time of the surgery. The diameter of the opening was approximately 10 mm. The myocardium of Caleb’s right ventricle had begun to increase in thickness in an effort to compensate for the blood that was pushed through the VSD from left to right ventricle with every beat. This overgrowth is called hypertrophy. Although a compensatory mechanism, it comes with consequences by causing congestion in the systemic circuitThe part of the circulatory system that carries oxygenated blood from the heart to the body and retu as fluids start to become unevenly distributed in the circuits.

Although the surgery attempted to stitch shut the VSD, damage to the AV bundle of the cardiac conduction system occurred. Severing the electrical connection between the atria and ventricles, the chambers were now contracting with disregard to each other. The sinoatrial node was conducting the atria to contract while the AV bundles were directing the ventricles to contract. Both electrical components were acting with disregard to each other.

At the 2 week follow up after surgery, Tiffaney bought Caleb to the surgeon’s office, knowing that the new was not good. Caleb had been declining back into the signsObjective clinical findings observable by a provider (e.g., edema, fever). and symptomsSubjective experiences reported by the patient (e.g., nausea, fatigue). that had led them to Dr. Baker in the first place. Plus, she knew that Caleb was not putting weight. They did an EKG in the office and it revealed almost twice as many QRS waves than P waves on the EKG. The surgeon reported the bad news that the VSD was not healing, but growing.

When Tiffaney got home, her mother knew from the look on her daughter’s face that their fears had been confirmed. In the coming weeks, a slew of family and friends took care of all Tiffaney’s household responsibilities such as laundry, shopping, and food preparation. The pediatric hospice nurse that her mother hired was an angel in human form. John-the-Nurse, as he later came to be known, was tender and caring with Caleb, understanding and supportive with Tiffaney, and cheeky with Tiffaney’s mom.

Late one night, with Tiffaney rocking a sleeping Caleb, John asked Tiffaney if she had considered how Caleb might be able to give life to other children. John asked if he could get her any information about the process of organ donation. After a long pause, Tiffaney said, “I was waiting for someone to ask because I just couldn’t bring it up on my own.” The next day, John arranged a virtual appointment with the Donation Coordinator at the local hospital. The Donation Coordinator answered all Tiffaney’s questions with candor and empathy, never pushing, never judging.

Caleb’s organs restored life to 4 children. Tiffaney decided that the donation of Caleb’s organs would be the last secret between mother and son. She felt solace in her decision that Caleb was still in this world with her, but she didn’t want to debate child-organ donation. Although she chose to donate, she saw both sides of such a difficult decision.

Caleb’s story is difficult, but it teaches us about both the science and art of medicine. Honor patients like Caleb by studying diligently, caring deeply, and committing to being an excellent healthcare provider.

Note 1 “Ventricular Septal Defect” figure above reproduced from Khatoon et al., 2019, “Transthoracic echocardiogram showing VSD flow (ventricular septal defect).” ResearchGate, https://www.researchgate.net/figure/Transthoracic-echocardiogram-TTE-showing-VSD-flow-VSD-ventricular-septal-defect_fig1_330029434. Licensed under Creative Commons Attribution 3.0 Unported (CC BY 3.0).