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Higher Goals for Children With Congenital Heart Disease

By Jessica Olsen, BSN, RN, CCRN Aug 30, 2022

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I will never forget my first pediatric cardiac patient.

I will never forget my first pediatric cardiac patient. She seemed like a typical preschooler except for her clubbed fingers and toes, surgical scar and chest tubes. She was in the hospital recovering from the Fontan operation. As a nursing student, I had not heard of the Fontan operation or her original congenital heart defect - hypoplastic left heart syndrome (HLHS). The diagnoses and procedures discussed in report were foreign to me, so I rushed to write them down and planned to ask an experienced nurse or look them up later. Over the next two days, I was captivated by this little girl and the surgeries that had given her the opportunity to have a childhood. I was fascinated by how a child born with half a functioning heart could undergo a series of operations turning that heart into a pump for her body. I was amazed she was there playing with me.

After graduating from nursing school, I started my career in a pediatric cardiac ICU and learned more about this corner of the nursing profession I now call home. As I learned about children with congenital heart disease (CHD), I realized the unique opportunity we have as we continue to search for the best ways to care for them. Because that little girl was born during this time in history, she will go to preschool, and there are other children just like her living into early adulthood. What else will become possible for patients like these within our lifetime, and what role will we play in making that a reality?

The Changing Odds - This Moment in History

Caring for children with CHD is a young and rapidly growing area of medicine. The timelines for the development of treatments, palliation and surgical corrections for various heart defects are each unique. Using the HLHS diagnosis for my first cardiac patient shows just how quickly this area of medicine is growing. If she had been born in the 1970s, she would have died in the first days or weeks of life. At birth, her body would depend on blood flow through the patent ductus arteriosus (PDA) to allow mixed blood (oxygenated and deoxygenated) to flow from the right ventricle, through the pulmonary artery, across the PDA and to her body. Over time that PDA would close, and her heart would be left unable to supply oxygenated blood to her body. If she had been born in the 1980s, she may have been one of the first babies to undergo an early version of the first-stage palliation of HLHS – the Norwood procedure. Initially, most of these patients died in the days to months following the surgery. Today, babies born with HLHS still face critical illness, a series of risky surgeries and lifelong medical needs, but more and more of them are living into adulthood. The odds are changing, which presents a unique opportunity for us. While we continue to search for the best treatments for these children, it’s important to think about how to prepare them to thrive in adulthood.

Thriving Beyond Surviving

In the early years of caring for children with CHD, the most immediate goal was to find a way to help them survive. As more children with CHD survive into adulthood, another challenge has emerged. Many of them face neurodevelopmental delays ranging from relatively minor to quite severe. Estimates of the exact scope of this problem vary, but one estimate claims approximately 50% of children with CHD will experience some level of neurodevelopmental disability. Other estimates indicate a much larger impact. These neurodevelopmental impacts include physical delays such as feeding intolerance, speech delays, increased prevalence of anxiety and depression, learning disabilities and behavioral challenges. What causes these neurodevelopmental impacts? What can we, as nurses, change? We are entering a new era of caring for these patients where thriving after survival is the goal.

Developmental Care in the Cardiac ICU

As I have learned more about my patients’ risk for neurodevelopmental delays, I have realized that we are uniquely positioned as nurses to help lessen some of the challenges. Children with CHD already overcome so many obstacles from the moment they are born to have a chance at life. If we can stand between them and further complications, we should. While we don’t know the exact causes of neurodevelopmental delays in these children, it is most likely a multifactorial issue. We cannot change many things about their hospitalization that may contribute to developmental challenges. Many of them will have low levels of oxygen to their brain for a portion of their early lives. They will be exposed to medications and equipment with unknown impacts on their developing brains. They will be subject to the schedules, noises and increased stimulation of the ICU environment. While we cannot change all these aspects of their early lives, there are many that we can. This article by Peterson and the Cardiac Neurodevelopmental Outcome Collaborative are good resources. Following are a few interventions that my colleagues and I have applied when appropriate in our ICU to create a more supportive environment for infant development.

  • 1Holding

    Infants belong in their parents’ arms, and holding supports healthy bonding between parents and infants during a critical time. Holding can also be an effective form of nonpharmacologic pain control. While it may be challenging to move critically ill infants to their parents’ arms, ways to do it safely should be explored and encouraged as soon as possible. When infants are unable to be held safely, there are other ways to promote comforting touch between parents and their child. One way is for parents to gently place their hands on the infant’s head and lower extremities and apply gentle, firm pressure. This method can mimic the feeling of being held without moving the infant.

  • 2Positioning

    Many infants who have prolonged admissions in the ICU are unable to participate in traditional developmental activities, such as tummy time, until after their sternal incision heals and they are clinically stable. Positioning them with their hips and shoulders supported can promote muscle development, postural alignment and comfort, and help them meet future developmental milestones such as crawling and walking. The Infant Positioning Assessment Tool is a helpful resource on proper infant positioning in the ICU setting. Of note, this type of positioning is only appropriate in the ICU setting where infants are under continuous monitoring, because it does not align with safe sleep guidelines.

  • 3Oral Skills

    Although methods to promote safe feeding in children with unrepaired cardiac defects are being studied, many children with cardiac defects have limited opportunities to develop oral feeding skills before and immediately after surgery. When babies are orally intubated, they have constant negative stimuli. Even when they are nasally intubated, they often do not have the same opportunities to explore their environment and learn oral skills as babies who are not critically ill. The effects of repeated negative oral stimulation and limited opportunities to develop and practice oral skills may lead to oral aversion and delays in feeding skills. One simple way to try to encourage oral skills in these infants is oral care with breast milk. When done gently, it can be performed with even the most critically ill infants and provides positive oral stimulation. More broadly, we should be careful with how we introduce any oral stimulation to these infants. This includes pacifiers, oral care and suctioning equipment. Care should be taken to introduce oral stimulation gently to promote positive experiences and prevent further negative associations for the infant.

  • 4Clustering Care

    This is perhaps the broadest intervention, but its implications are far reaching. The ICU is not designed for an infant’s developing brain. Even the quietest ICUs have bright lights, alarms and interruptions from members of the care team at all hours of the day and night. Clustering care to create extended periods of undisturbed rest and promote sleep is critical. Cycling lighting so infants have a break from bright hospital lights also gives their brains time to rest. As the members of the care team who are most often at the bedside, nurses are in a unique position to encourage clustering of care between multidisciplinary teams.

These are only a few of the developmental care principles that can be included in the care of these critically ill infants. Many of these interventions are borrowed from the neonatal ICU environment where they are more traditionally a part of infant care. More research should be done on how to safely incorporate and tailor these aspects of newborn care into the cardiac ICU setting. What other ways can we support these infants’ growing brains?

Fighting for the Best Adulthood

CHD is a monster of an opponent that these babies fight from the moment they are born. Most of them will continue this battle for the rest of their lives. If we can play a part in lessening this battle by protecting them from further neurodevelopmental challenges, we should. I may never know the full effect of these actions, but I have seen glimmers of impact. I took care of an infant who was born with dilated cardiomyopathy. She was in severe heart failure and listed for transplant. She was too small for a ventricular assist device and remained on our unit for months as we treated her heart failure and waited for her transplant. She was paralyzed, sedated and intubated. She was unable to participate in activities to meet developmental milestones. The goal was to help her survive to transplant. I took care of her frequently and tried to care for her in a way that supported her development. I positioned her body so her hips and shoulders stayed aligned. I performed oral care gently with her mom’s breast milk, hoping to encourage positive oral stimulation and not contribute to further oral aversion that she would later have to overcome.

Even though she could not be held safely, I encouraged her family to interact and comfort her in other ways. I also advocated for decreased stimulation, clustering cares and coordinating with her care team to promote periods of time for rest. So much of her hospitalization was hindering normal development. I did not want to add any more obstacles. Eventually this baby received a heart transplant and was ultimately discharged home with her family.

Several years later she came back to our unit after a procedure. She was stable, and I saw her playful toddler personality. She talked to staff as they came into her room, and her mom showed me videos of her child at home. Watching her meet milestones, such as walking, eating and talking, even if she was a little behind the progress of the average child her age, made me wonder about the impact of my actions when she was a tiny infant waiting for a heart.

The medical field has a long way to go in its search for new surgical procedures, medications and therapies to treat children with CHD. I am excited to see how care for these children improves over the course of my career, and I feel honored to be their nurse. As more and more of these infants live to become adults, we must continue to advocate for their little brains as well as their hearts. Survival is no longer the highest goal for them.

How else will we fight for these children to thrive as they grow to adulthood?