30 Advanced Heart Disease

Melissa K. Cousino, Chelsea Heneghan, and Elizabeth D. Blume

Introduction

In contrast to adults, advanced heart disease (AHD) is a rare condition in children, with approximately 11,000–14,000 heart failure-related pediatric hospitalizations occurring annually in the United States.¹ There are two primary etiologies of pediatric AHD: cardiomyopathy and congenital heart disease (CHD). The overall incidence of CHD is approximately 8–10 per 1,000 live births,² and the incidence of cardiomyopathy is estimated at 1 per 100,000 children.^3,4 Only a fraction of children diagnosed with either form of pediatric heart disease eventually progress to AHD⁵ (Box 30.1), necessitating long-term intensive medical management and/or recurrent hospitalization. Those who do are at high risk for developing end-stage heart disease,^5,6 a clinical syndrome characterized by marked reduction in quality of life, functional status, nutritional deficiency, and respiratory distress. Similar to adults, the high mortality associated with pediatric AHD stems from its sequelae, including low cardiac output, respiratory failure, malignant arrhythmias, stroke, thromboembolism, multiorgan dysfunction, and infection.⁵ As such, children with AHD represent a diverse group ranging from those cared for at home by their parents while still participating in childhood activities to those in hospice care at the end of life. For some, advanced therapies, such as transplantation, ventricular assist device, or other high-risk interventions may be indicated. For others, therapies may be limited. Across this diverse patient population, there is increasing evidence that pediatric AHD impacts the physical and psychosocial health of affected children as well as their families. Palliative care provides an opportunity to proactively intervene across physical, psychosocial, and spiritual domains in order to maximize the quality of life and symptom management of children living with AHD and their families, as well as prepare families for complex treatment decision-making and end-of-life care.

The reported prevalence of CHD varies widely depending on the method of diagnosis and whether the data reflect prenatally detected CHD or live births of children with CHD. In general, the prevalence of CHD is approximately 3 per 1,000 for clinically severe defects (those requiring an intervention in the first year of life), 6 per 1,000 when including the more moderate defects, and 9–20 per 1,000 when including smaller septal defects and mild valve stenosis. Between 1940 and 2002, approximately 2 million infants were born with CHD in the United States.⁷ Over this same period, tremendous achievements in surgical and medical care led to improvements in long-term survival of children with CHD. Surgical procedures have been developed to treat most congenital heart defects, including those that were historically uniformly fatal, such as hypoplastic left heart syndrome. Major advances have also been achieved in intensive care management, ventilatory support, mechanical support, intraoperative management, long-term medical management, and diagnostic imaging.^8,9 Prenatal detection of CHD has led to more controlled perinatal transitions and earlier surgical repairs. As a result, the number of young people living with severe CHD has increased. In 1985, the median age of all patients living with severe CHD was 11 years. In 2000, the median age was 17 years, with 49% of the CHD population being adults.¹⁰ There are currently more than 2.4 million people (1 million children and 1.4 million adults) living with CHD.¹¹

Despite dramatically improved short- and long-term outcomes, CHD is the leading cause of infant deaths owing to congenital anomalies worldwide, and palliated heart disease remains one of the leading causes of nonaccidental death in childhood in the United States.^2,7 Although only a small number of children with CHD will progress to AHD, morbidity and mortality among this group is high. Approximately 14,000 children experience heart failure related hospitalizations annually in the United States, and 7% die during their hospitalization. This mortality rate is nearly 20 times greater than hospitalized children without heart failure and approximately double that seen in adult heart failure.¹ For those with end-stage disease, ventricular assist device therapies and heart transplantation offer the potential for extended survival. However, almost 20% of children listed for transplant will die waiting for an organ, and an additional 1 in 10 heart transplant recipients will die within the first year post-transplant.¹²

Palliative Care in Pediatric Advanced Heart Disease

To improve quality of life, reduce suffering, and support patient and family medical decision-making, there is growing attention being paid to the role of palliative care within pediatric AHD. However, the evolution of palliative care in children with AHD has occurred slowly. The slow progress is likely due to the growing perception of heart disease as highly treatable via surgical and mechanical interventions and the variable disease course with periods of both stability and acute-on-chronic decompensation (Figure 30.1).^13,14,15

Involvement of palliative care services for pediatric patients with AHD is considerably less when compared to other critical pediatric illnesses, although trends suggest this is changing.¹⁶ In a large, multisite study, only 8% of pediatric palliative care consults were for patients with underlying cardiac disease.¹⁷ Among pediatric patients who died of AHD at a single institution, 16% received a palliative care consult.¹³ In a recent study of circumstances surrounding death in pediatric heart transplant recipients, 30% had received a palliative care consult.¹⁸ There is emerging evidence highlighting the benefits of palliative care involvement for pediatric patients with critical heart disease and their families. In a randomized controlled trial of early palliative care referral for infants prenatally diagnosed with single-ventricle heart disease, maternal anxiety of surviving infants was less with improved family communication and relationships reported among those receiving early consultation.¹⁹

Despite high morbidity and mortality, along with increasing evidence for the important role of palliative care within pediatric^19,20 and adult AHD,²¹ pediatric cardiologists endorse barriers to requesting palliative care consultations. The most notable barriers include undermining parental hope and worry that parents would perceive the referral as their cardiology team giving up.²² In a recent study of communication about difficult topics in pediatric end-stage organ disease (e.g., advance care planning), multidisciplinary pediatric healthcare clinicians endorsed a number of communication challenges, including parents not wanting difficult conversations to occur with their child.²³ Despite many perceived barriers, it is encouraging to note that the majority (85%) of cardiologists find palliative care involvement to be helpful,²⁴ and many cardiologists feel comfortable providing care at end of life.²² The role of the specialty palliative care team in continuing to collaborate with and educate pediatric cardiologists and cardiothoracic surgeons to overcome barriers to move forward palliative care in pediatric cardiology cannot be underestimated.

Although there are no clinical practice guidelines specific to palliative care involvement within pediatric cardiology or AHD, the small but growing literature highlights diagnoses and patient populations that may especially benefit from specialty palliative care, including single ventricle anatomy, CHD associated with genetic disorders, pulmonary vein stenosis, and/or heart transplant/mechanical circulatory support (Figure 30.2).^25,26 A national survey of pediatric cardiologists found that palliative care referrals most commonly occurred for patients with pulmonary vein stenosis or those needing heart transplantation or mechanical circulatory support.²² Palliative care involvement specific to psychosocial support and goals of care discussions has been most common.²⁷ Symptom management of thirst may also be an area where palliative care can be particularly helpful.²² Looking forward, the establishment of “automatic” consults for specific triggers may help augment and systematize specialty palliative care consults.

While there has been considerable uptake of palliative care clinical services and research within pediatric cardiology, there remains additional opportunity for improvements. Figure 30.3 depicts the collaborative contributions of disciplines across palliative care and cardiology teams to the care of pediatric patients with AHD and their families. The interdisciplinary nature of palliative care, in partnership with cardiology teams, is helpful for promoting quality of life through reducing physical and psychosocial symptom burden and facilitating communication and decision-making.

Although the past decade has shown an increase in studies evaluating the quality of life in children with AHD, this continues to be an underresearched field.^{28,29,30,31,32} Early research described abnormalities in both the physical and psychosocial domains of quality of life, with 20% of children with mild to moderate CHD reporting significantly impaired quality of life. Parents and healthcare providers of children and adolescents with CHD more frequently identified physical dimensions, most often physical limitations, as quality-of-life concerns.³³ Physical limitations are a critically important element to quality of life in the subset of children with AHD who lack the physical stamina to participate in many developmentally appropriate activities. Scarring, medication regimens, receiving special treatment, school issues, and social issues were identified by school aged children and/or their parents as negatively affecting the children’s quality of life. Adolescents also noted the negative impact of feeling different from peers and recognition of their own mortality as contributing to lower quality of life.³³

In one of the first studies to explore parental perspectives on suffering and quality of life at the end of life in children with AHD, almost half of bereaved parents perceived their child experienced suffering at end of life. Furthermore, more than two-thirds of parents perceived their child’s quality of life in the last month of life as poor or fair. These parents specifically noted that symptoms contributed to suffering. Parents of children younger than 2 years perceived breathing difficulties, feeding difficulties, and pain to be associated with a great deal of suffering. Parents with children older than 2 years reported fatigue, sleep disturbances, breathing difficulties, poor appetite, and chest pain as contributing to significant suffering.¹⁵ Further research supports pain, fatigue, breathing difficulties, and feeding difficulties as contributing to suffering in children with AHD.³⁴ These studies highlight an opportunity for improvement in quality of life by specifically addressing symptoms.

Understanding the medical context, including prognostic indicators of a child’s AHD, can be helpful to identify the need for consulting specialty palliative care. As described earlier, many children with AHD would accept a consult to specialty palliative care at any time within the disease course. Additionally, earlier conversations about prognosis may help families make decisions that fit within their personal values. Continued conversations with the child and family may shed light and offer opportunities to optimize quality of life through symptom management according to patient and family priorities. Interventions and symptom management recommendations must be tailored to the needs of the patient and family. If the family’s stated goals are to have every opportunity to pursue disease-directed therapies and interventions, medications with side-effect profiles that may risk those goals would likely not be appropriate interventions. On the other hand, many patients and families make the loving decision to focus on comfort and symptom management, foregoing future interventions. Specialty palliative care may be particularly beneficial in clarifying and delineating some of these hopes to further align the teams’ approach with the goals of the child and family. We will note particular branch points in symptom management recommendations later based on stated goals while also acknowledging that, for many pediatric patients and their families, goals may continue to remain blended and become clearer throughout the illness course.

Symptom management includes a variety of complex medical and psychosocial interventions to optimize quality of life. As cardiac function deteriorates, traditional symptoms of heart failure can ensue. Symptom management can be complex, and side effects of drugs can worsen these symptoms. The first strategy to good symptom management in children with AHD is good heart failure management. Improved cardiac output will improve symptoms. Some guidelines are offered in Table 30.1.

Pain

To best understand pain in children with AHD, it is important to first review and understand the pain etiology (see Chapter 23). From recent research we know that parents of children with AHD report almost 80% of children hospitalized with AHD suffered considerably due to pain.³⁴ The most commonly experienced types of pain in patients with AHD are abdominal pain from fluid overload and/or ischemia as well as procedural pain. Furthermore, as with all symptoms, it is important to obtain a full understanding of the pain a child is experiencing, how they are experiencing it, and what interventions (both pharmacologic and nonpharmacologic) have been most helpful.

Much of the pain experienced in both the ambulatory and inpatient setting in children with AHD is procedural pain, particularly needle-stick pain. For outpatient procedures, it may be helpful to provide a written prescription for topical anesthetic cream and instructions for application for the parents. Additionally, managing inpatient needle sticks with pain prevention protocols, including distraction techniques and topical anesthetic creams, may reduce trauma. Optimizing nonpharmacologic strategies, such as a cold compress, applying pressure or vibration, and repositioning can also help to lower levels of anxiety, distress, and pain.³⁵ In a focus group of parents of children on ventricular assist devices, dressing changes were the most distressing reason for perceived pain (Cousino, May, and Blume, 2019, direct communication). Collaborating with child life specialists to develop patient unique coping strategies (e.g., iPad distraction, diaphragmatic breathing) around procedural pain can be incredibly beneficial.

Abdominal pain can be challenging to diagnose and treat, particularly in children with AHD. The later section on gastrointestinal (GI) disturbances offers differentials for nausea/vomiting that may contribute to abdominal discomfort. Visceral pain, which is diffuse in character, can be a symptom in children with CHD. Pain is initiated when visceral receptors, located on smooth tissue membranes, within intestinal muscle, and in the mucosa of hollow organs, are stimulated by excessive contraction, stretching, tension, or ischemia. Visceral pain in children with AHD may result from vasoconstriction and/or ventricular injury causing decreased skeletal perfusion and ischemia to the organs and gut. Some studies and anecdotal evidence suggest gabanoids (gabapentin/pregabalin) can be successful in treating this type of pain.³⁶ Consultation to specialty palliative care may offer further expertise in titration of gabanoids and use of amitriptyline/nortriptyline for visceral hyperalgesia, as well as possible recommendations for methadone (see Chapter 23).

Specific considerations for methadone initiation in patients with AHD include potential risk of QTC prolongation, and increased risk of torsades de pointes. Research of the side effect profile of methadone in children with cardiac disease is limited. Most research comes from the adult literature and pediatric oncology patients and excludes patients with cardiac disease. Adult literature has identified individuals with cardiac disease are at significant risk for QTC prolongation on methadone.^37,38 The APS guidelines, in adult patients, recommend reconsideration of methadone use with a QTC interval between 450 and 500 ms and avoidance of methadone with a QTC above 500 ms.³⁹ There are no recommendations on guidelines for ECG monitoring for methadone addition in pediatric patients with AHD. Thus, when adding any potential QTC prolonging medication, careful and thoughtful evaluation of the child’s and family’s goals for treating the symptom, as well as weighing the child’s potential risk for that symptom is necessary. Close collaboration with specialty palliative care and cardiology teams can be particularly helpful in this instance.

Pain may also result from excessive fluid overload and edema of neck, abdomen, back, and extremities. Diuretics can provide symptom relief caused by peripheral edema, and postural changes can help with back pain related to edema in the sacral area. Although chest pain is rare, it is very frightening for children and families. Opioids can improve coronary perfusion and may have a primary cardiac role in patients with ischemic disease. Discussions about pain management should occur early on. Many families and caretakers worry about the cardiac and respiratory effects of opioids. Understanding a family’s specific worries about opioids will strengthen the therapeutic relationship between patient/family and provider and allow the provider to address those concerns. Most cardiac ICUs are comfortable with opioids in the postoperative period. Providing education about opioid use to cardiology staff outside of the postoperative period may improve provider comfort. Recognizing symptoms which might benefit from opioids and creating a trial period with opioids for these symptoms can be a helpful approach. Pain management is critical to improve suffering throughout the disease course while continuing life-extending measures in children with AHD.

Fatigue

The activation of both the renin-angiotensin system and the sympathetic nervous system results in vasoconstriction and poor skeletal muscle perfusion. This often leads to overwhelming fatigue, sometimes out of proportion to the cardiac dysfunction. For patients in the early phases of AHD, fatigue can be particularly difficult to sort out, as it can often be confused with laziness or depression. Modified physical therapy and cardiac rehabilitation programs can be useful to avoid deconditioning and maintain muscle tone. In addition, modified exercise programs have been shown to be beneficial to outlook and endurance in patients with CHD.^40,41 Fatigue may be a marker of overall symptom burden (Molloy, personal communication). If fatigue is the most prominent symptom, it is useful to do a comprehensive symptom history. For those AHD patients whose fatigue is secondary to poor muscle perfusion, systemic vasodilators, such as milrinone, can help lower systemic vascular resistance and result in temporary improvement of fatigue. Many children and young adults describe “something lifting off my chest” after a few hours of a milrinone infusion. There is some anecdotal evidence that a short infusion holiday of 3–5 days can have an improved effect lasting for several weeks. Some patients may benefit from this therapy either intermittently or as a continuous infusion.

Sleep Disturbances

Poor sleep in children with AHD may be a variable that contributes to overall fatigue as well exacerbates other symptoms. Understanding a child’s sleep routine will provide valuable insight into guiding management. Parents of children with AHD reported that sleep disturbance contributed to their child’s suffering.¹⁵ Sleep disturbances may be caused from a number of different reasons including obstructive apnea, environmental factors (hospital setting, timing of medications), dyspnea, pain, depression, and anxiety. Trialing nonpharmacologic management (as described in Chapter 22) by optimizing the environment to support circadian rhythm throughout the day and nighttime is typically a good first step. In addition, timing of diuretics is critical in maintaining good sleep hygiene because diuretics before bedtime will interrupt sleep. Diuretics should not be given within 4 hours of bedtime. We recommend twice-daily diuretics be retimed to 8 am and 4 pm to allow for minimal sleep disturbances secondary to need to void. Pharmacologic therapy can start with melatonin. Though ramelteon, olanzapine, trazodone, and clonidine are all medications that have been used with good effect in children for insomnia, each of these medications has potential cardiac side effects. Special care in understanding insomnia as well as goals of care must be made prior to initiation of any of these medications. Olanzapine and trazodone both have QTC prolongation side effects. Clonidine reduces sympathetic outflow from the central nervous system, thus special attention to blood pressure must be noted prior to initiation. Furthermore, trials of methylphenidate may be trialed in patients experiencing extreme fatigue who have not benefited from methods discussed earlier (both pharmacologic and nonpharmacologic). However, methylphenidate may increase the risk of arrhythmia and should be thought about carefully with primary cardiology team.

Breathing Difficulties

Breathing difficulties in children may be a result of a variety of different reasons. In a study of parental perspectives, while almost all children hospitalized with AHD who reported breathing difficulties were treated for this symptom, only about 40% of parents felt their child was treated successfully.³⁴ One common reason for breathing difficulties in children, adolescents, and young adults with AHD is fluid overload. Although many children do not present with the traditional adult heart failure congestion with rales, respiratory distress, and shortness of breath, some children do present with these symptoms. Discussions around what interventions make the most sense for the patient physiologically should be considered.

Children with end-stage disease may experience dyspnea, the subjective feeling of breathlessness. Dyspnea may be managed through nonpharmacologic techniques as well as pharmacologic (see Chapter 24). A fan near the face is very effective for relief of the breathlessness of dyspnea in this population. Children as young as 3 or 4 years can ask for and control the use of a small handheld fan effectively. Oxygen can be added if the patient feels relief from it, though some children are bothered more by the cannula and the dryness versus the relief from oxygen itself. It is important to have specific conversations with families, as many families believe that oxygen will prolong life or improve symptoms, when the cannula themselves are causing more distress.

Gastrointestinal Disturbances, Feeding Difficulties, and Thirst

Children with congestion and heart failure often have GI symptoms such as nausea, vomiting, anorexia, and extreme thirst. Diuretics and fluid management are the key therapy for congestive symptoms in heart failure in children and can improve GI symptoms rapidly. Many loop diuretics, such as furosemide, have a threshold effect, and if patients with normal renal function are not responding, then doses can be doubled. Ascites can present as pain or feeding intolerance. This is often accompanied by edema of the bowel wall and poor GI absorption of diuretics, requiring intravenous diuretics.

Fluid restriction for these patients is critical in maintaining homeostasis and decreasing hospital admissions. However, fluid restriction can be very difficult as the body’s response to lower output is secretion of anti-diuretic hormone (ADH) and subsequent thirst. Discussion about the utility of fluid restriction and the understanding that the thirst response is counter-regulatory may help some patients and families. The maintenance of fluid restriction for many families is endless and futile, and increasing diuretics may be the simplest action. Some children find that sour candies and hot sauce in small amounts seem to satiate the thirst response. Frozen fruits such as grapes and watermelon can help with thirst as well. Working with child life to set up a chart for fluid allowance throughout the day is helpful. Additionally, collaborating with a team nutritionist can be beneficial in thinking about how to modify diet to best aid in fluid restriction. Many children with AHD have a nasogastric or gastrostomy tube to assist with feeding. Modifying feeding regimens to deliver feeds continuously rather than bolus feeds may also improve abdominal discomfort.

Dizziness and/or Syncope

Syncope can be a result of primary ventricular arrhythmia secondary to myocyte fibrosis and can be very frightening for families and patients. It may be worsened by electrolyte abnormalities such as hypomagnesemia and hypokalemia, which should be monitored closely. Dizziness can also be very disturbing for patients as it may remind them of the presyncopal state. It may be due to low cardiac output. More often, however, it is a symptom related to side effects of the cardiac medications and can limit quality of life. The use of multiple antihypertensives together in the morning can often lead to dizziness and headache. Awareness of the total medical picture can help improve timing of medications for patient comfort. Vasodilating medications given at bedtime may reduce dizziness during the day.

Psychosocial Functioning

Similar to other serious pediatric illnesses, children and young adults with AHD experience increased rates of emotional and behavioral challenges. In a sample of adolescents with single ventricle anatomy, lifetime prevalence of a psychiatric diagnosis was 65%, with nearly half of the participants meeting criteria for a diagnosis at time of study assessment.⁴² Moreover, the sample had an approximately five times increased rate of lifetime anxiety disorders (35%) and attention deficit hyperactivity disorder (ADHD) when compared to healthy controls. In a recent study of children and adolescents pre-heart transplantation, 14% presented with a psychiatric admission during their hospitalization.⁴³ Of those 8 years or older with a ventricular assist device (VAD) in place, 40% met criteria for a psychiatric diagnosis, with adjustment (20%) and anxiety (17%) disorders being most common. For those with AHD who go on to receive heart transplantation, psychosocial risks persist. Approximately 20–50% of pediatric heart or heart-lung transplant recipients have been found to have impaired psychological functioning many years post-transplant.^44,45,46

Notably, psychological symptoms and advancing heart disease symptoms often overlap. For example, fatigue and anhedonia may be due to worsening depression, heart failure, or a combination of both. Thus, thoughtful and frequent assessment of psychological health throughout the heart disease course is important.⁴⁴ Antidepressant therapy, specifically the use of selective serotonin reuptake inhibitors (SSRIs) for anxiety and/or depression, are used in a subset of patients with AHD requiring heart transplantation (7%), with more common use seen among those on VAD therapies (25%).⁴³ In addition, psychological support is recommended utilizing evidence-based interventions, including cognitive-behavioral therapy and relaxation skills training, to address psychological symptoms commonly experienced among this population. Both psychology intervention and child life support services can be utilized for patients with significant procedural distress (e.g., needle-based procedures, dressing changes) to enhance coping and improve quality of life.

Family Impact

It is important to recognize that AHD impacts the entire family system. Parents of children with CHD have higher rates of psychosocial morbidity than do parents of either healthy children or those with other chronic medical conditions.³³ Sources of stress include medical and surgical procedures, activity restrictions, uncertainty, the child’s prognosis, the child’s behavior, decisions about disclosing medical details to the child, transitioning older children to self-management, and perceptions that their child is different.^{34,35,36,37,38} Parents also experience significant rates of psychiatric comorbidities, including depression, anxiety, and somatization, and there is evidence that these are chronic in nature.³⁹ Approximately 9% of parents of children post-heart transplantation endorsed symptoms of posttraumatic stress,⁴⁰ further underscoring that the impact of pediatric heart disease on parents is long-lasting.

Although less is known about the psychosocial impact of AHD on siblings, one study found that 30% of parents reported adverse sequelae of CHD on healthy siblings, including anxiety, depression, anger, jealousy, feeling left out, and perceptions that the child with CHD lived under different rules.⁴³ Given the impact of pediatric AHD on the psychosocial well-being of the affected child and his or her parents and siblings, palliative care for this population involves attention to the psychosocial health of the entire family.

Communication and Advanced Care Planning

Although it is widely recommended that adults with AHD participate in advance care planning,^47,48 minimal prognostic, advance care planning, and end-of-life care focused communication occurs across adult heart failure populations. Among adults with CHD, only 1% recalled discussing advance care planning with their medical team even though 78% of the participants believed their healthcare providers should initiate end-of-life decision-making discussions early in the disease course.⁴⁹ Advance care planning and end-of-life care communication occurs even less frequently in pediatric AHD populations.

In a recent pilot study of adolescents and young adults with AHD undergoing heart transplant evaluation,²⁰ the majority of participants (83%) indicated that they wanted to discuss their prognosis, while 67% stated that they would want to discuss their end-of-life care preferences if very sick. Although few had engaged in formal advanced care planning discussions, study participants expressed preferences for how such conversations should occur. The majority expressed a preference for family involvement in end-of-life decision-making. There was wide-ranging variability in terms of when young people would want to start such a discussion, thus highlighting the importance of tailoring advance care planning discussions to the individual needs and preferences of patients and families. It is also important to recognize that although parents understood their child’s desire to be involved in medical treatment decision-making (e.g., decision to be listed for heart transplant), only 58% of parents perceived that their child would want to be involved in end-of-life decision-making.²⁰

Findings align with research in other pediatric illness groups suggesting that young people desire prognostic information and want to be made aware of their options and engage in shared decision-making with caregivers.^50,51 Randomized controlled intervention research in both pediatric cancer and HIV populations has demonstrated that family-centered advance care planning with young people results in increased awareness of adolescents’ and young adults’ end-of-life wishes, reductions in invasive interventions at end of life, reduced decisional burden, and increased likelihood of home deaths.^51,52,53,54 Advanced care planning should begin with the primary cardiology team. Research from pediatric oncology patients and their families endorsed preferring to have these discussions with trusted and established care providers.⁵⁵ Specialty palliative care can further support the primary team as well as the patient and family.

Taken together, advance care planning for children with AHD should be tailored to the individual needs and preferences of patients and families with specific attention paid to clear communication about possible interventions and their limitations, such as extracorporeal membrane oxygenation (ECMO), transplant, and VAD, as well as less invasive interventions, such blood draws and cardiac catherizations. Providers can establish consistent time points for when preferences for end-of-life decision-making are assessed and revisited. Honest and systematic conversations that occur throughout the disease trajectory decrease unexpected decision-making discussions during highly stressful times.⁵⁶

Decision-Making

Shared decision-making in pediatric cardiology is critical yet challenging amid the changing landscape of improved long-term outcomes, more children living into adolescence and adulthood, and the rapid emergence of highly technologic therapies. Decision-making specific to life-prolonging interventions is complicated by the highly variable trajectory of AHD, with many patients experiencing acute exacerbations followed by periods of stability. Furthermore, risk stratification depends on large numbers of randomized studies which are not possible in this field. Many parents have been told their child’s anatomy is unique and that options and outcomes for each child are uncertain and unknown. In addition, many families of infants with complex CHD have already made significant important decisions about goals of care by continuing the pregnancy. It is imperative that pediatric cardiologists review with new families their previous experience and their prior decisions. Understanding this will encourage this important connection between primary cardiac subspecialist and parents. Specialty palliative care support and consultation to assist with understanding decision-making can be especially helpful to support primary care teams, patients, and their families—who are likely to encounter a number of high-stakes decisions, such as decisions regarding surgical interventions, transplant listing, use of technology and/or mechanical support, and end-of-life care.

Fetal Counseling

For many families, challenging decision-making occurs at the time of prenatal diagnosis of severe CHD. Although staged reconstruction is often the most common path and provider recommendation for hypoplastic left heart syndrome,⁵⁷ a single-center retrospective study found that 11% of families chose to terminate the pregnancy and 7% opted to proceed with comfort care only at birth.⁵⁸ Specialty palliative care consultation in the prenatal period, especially for complicated CHD,⁵⁹ provides an opportunity for cardiology and palliative care teams to work together to guide families through their decision-making. Furthermore, in a randomized controlled trial, Hancock et al. showed a decrease in maternal stress in the postpartum period among patients born to families who had a specialty palliative care consult in the fetal period.¹⁹ For many families where palliative resources are not available, primary palliative care skills for the fetal cardiologist are critical. Starting the encounter with questions about what the parents understand about their child’s condition and whether there are any care plan decisions already made will help this first encounter move more successfully. For example, for parents who are not considering termination to hear about termination as an option for the first 10 minutes of the encounter can derail the relationship from the start.

Technology, Ventricular Assist Devices, and Mechanical Circulatory Support

Decisions regarding technological devices are another important component to decision-making in pediatric cardiology. The wider acceptance of mechanical support in the form of VAD and ECMO support bring another level of complexity to the decision-making process. The acceptance of technology must not be seen as working in contrast with palliative care. These technologies must be incorporated into the spectrum of therapy for AHD. Families and caregivers can hold on to the hope of recovery or transplant while supported by a device, while also making preparations and plans for symptom relief and minimizing suffering. This balance often feels more delicate to the provider than to the family. Most families can hold both if the presentation is clear and direct. Pediatric cardiologists should set aside time to discuss the “what ifs” with parents away from the bedside, in a conversation that does not include a medical update or plan for the week. Interdisciplinary team huddles to enhance coordination of roles during family meetings may increase family involvement in these important discussions.⁶⁰

Consultation with specialty palliative care prior to initiation of these therapies can be particularly helpful for enhancing the longitudinal relationship with the patient, family, and care teams. Reliance on mechanical support strategies will provide an important new area for understanding palliative care needs in all AHD patients and in children in particular. Because of the advanced nature of the heart disease of children who require ECMO or VAD support, there may be instances when discontinuing mechanical support is a consideration. Although complications such as stroke with VAD are decreasing, discussing end-of-life care process prior to implantation is critical. The difficulties for families of patients with a VAD often revolve around the patient who may be cognitively aware and neurologically healthy, with the heart beating, but with severe end-organ dysfunction, and thus no longer a transplant candidate. This type of discontinuation by terminating the pump function may feel different to medical providers and families than discontinuation of an endotracheal tube. Specialty palliative care involvement for VAD patients has been shown to be valuable, with early involvement leading to increased discussions about goals of care and advanced directives.⁶⁰

Moreover, the development of a deactivation checklist for providers and families has been offered as a starting point to be sure the goals are reviewed and that pertinent people are present.⁶¹ It is important to note that recommendations about premedications and symptom management for these situations vary between institutions. General guidance advises avoiding neuromuscular blocking agents and titrating sedatives and analgesia to treat objective signs of discomfort that evolve with the discontinuation of life-sustaining therapies. Providers continue to have diverse views about the process of deactivation of VAD in children, and knowledge gaps are notable.^62,63

In addition to ECMO and VAD, many children who progress to AHD already have an implantable defibrillator (ICD). Again, this technology provides some safety from sudden death but at the expense of pain and anxiety from defibrillation if the device fires, as well as the possibility of continued firing at end of life. Turning off the defibrillator is a difficult decision for families and patients, and plans should be set in place before end of life. Many families cannot bear to turn it off until the end stage, which can sometimes be disturbing at end of life. Formal institutional policies for end-of-life care for pediatric patients with ECMO, VAD, and/or ICD may help reduce medical provider and family decisional stress, burden, and regret.

Related posts:

Quality in Pediatric Palliative Care Program Development The Language of Pediatric Palliative Care Relationships with Children and Ensuring Their Voices in Decision-Making Oncological Illnesses Children’s Voices: The Experience of Patients and Their Siblings

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