Advanced Heart Disease

Published on 09/04/2015 by admin

Filed under Hematology, Oncology and Palliative Medicine

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40 Advanced Heart Disease

Elizabeth D. Blume, Angela Green

My daughter has had heart surgery five times. Now the only option is a transplant. She was at home for a while, but now she is in the hospital with a ventricular assist device. She feels better, but being in the hospital is hard. She misses her friends. For us, it is really scary waiting and hoping for a transplant to give her a chance to live. Knowing that even with the transplant, she may not live.

In contrast to adults, severe advanced heart disease is a rare condition in children.1,2 There are two primary etiologies of pediatric heart failure: cardiomyopathy and congenital heart disease (CHD). The overall incidence of CHD is approximately 8 per 1000 live births,1 and the incidence of cardiomyopathy is estimated at 0.58 per 100,000 children.3,4 Yet only a fraction of children diagnosed with either form of pediatric heart disease eventually progresses to advanced heart disease5 necessitating long-term intensive medical management and/or recurrent hospitalization. Those that 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, however, the high mortality associated with pediatric advanced heart disease stems from its sequelae, including low cardiac output, respiratory failure, malignant arrhythmias, stroke, thromboembolism, multi-organ dysfunction, and infection.5 As such, children with advanced heart disease represent a diverse group ranging from those cared for at home by their parents and still participating in childhood activities to those in hospice care at the end of life. Regardless of the scenario, there is increasing evidence that advanced heart disease impacts not only the physical health of affected children, but also the psychosocial health and quality of life of the child and his or her family across the illness trajectory. Palliative care provides an opportunity to proactively address physical, psychosocial, and spiritual issues in order to maximize the quality of life children with advanced heart disease and their families, as well as prepare families for the complex end-of-life decision making.

Incidence and Epidemiology of Congenital Heart Disease and Pediatric Advanced Heart Disease

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 1000 for clinically severe defects, 6 per 1000 when including the more moderate defects, and 9 to 20 per 1000 when including smaller septal defects and mild valve stenosis.1,2 CHD is the leading cause of infant deaths owing to congenital anomalies worldwide.1,2 Between 1940 and 2002, approximately 2 million infants were born with CHD in the United States.1 In those same six decades, enormous achievements in medical and surgical care of these infants and children have resulted in improved long-term survival. Despite dramatically improved short- and long-term outcomes, palliated advanced heart disease remains one of the leading causes of non-accidental death in childhood in the United States. During the past 60 years, surgical procedures have been developed to treat most congenital heart defects, including those that were historically uniformly fatal, such as hypoplastic left heart syndrome.7 During the same time period, major advances have been achieved in intensive care management, ventilatory support, mechanical support, intra-operative management, long-term medical management, and diagnostic imaging.7,8 These improvements are forcing reassessment of the outcomes and impact of CHD. For example, prenatal diagnosis has led to earlier detection, more controlled perinatal transition, and earlier surgical repair as well as more educated and prepared parents. The traditional statistics of prevalence and outcomes are, therefore of historic value, but may be less helpful in determining the longer term needs of children with advanced heart disease.

Improved survival rates of children with CHD is reported over the past two decades. A review9 of the multiple-cause mortality files compiled by the National Center for Health Statistics of the Centers for Disease Control and Prevention from all death certificates filed in the United States found that from 1979 through 1997, mortality from heart defects for all ages declined 39 percent, from 2.5 to 1.5 per 100,000. In the last two years of the study, heart defects contributed to 5822 deaths per year. Of these deaths, 51% were infants and 7% were children 1 to 4 years old. In this series, age at death increased over the decade for every heart defect, as more palliated infants are surviving into adolescence and adulthood.9 Only a small number of children born with CHD will progress to advanced heart disease (Box 40-1). There are no published data on the numbers of children with CHD who meet criteria for advanced heart disease and prognostic indicators to assist with predicting which patients are likely to progress are limited.

BOX 40-1 Pediatric Heart Diseases That May Progress to Advanced Heart Disease

• Single ventricle palliation, such as hypoplastic left heart syndrome
• Pulmonary vein stenosis
• Systemic RV lesions, such as L-TGA
• Post-transplant coronary artery disease
• Cardiomyopathy
• Protein losing enteropathy and/or plastic bronchitis
• Pulmonary hypertension/Eisenmenger’s

Evolution of Palliative Care in Advanced Heart Disease in Children

Because of the previously described advances and improved longevity, pediatric cardiac teams now provide long-term care for children for whom previously the only treatment decision was the location of their death. Early efforts necessarily focused on survival. Over time, the focus expanded to include managing medical morbidity and maximizing the quality of life of children with CHD, including those with advanced heart disease. The evolution of palliative care in children with advanced heart disease has occurred slowly in part due to the perception of heart disease as treatable with the focus on surgical cures. This is similar to the evolution of care in adult advanced heart disease as well.10,11 It is important to recognize that many interactions between families and the healthcare team revolve around the surgical or interventional procedure to fix the child’s heart. Even in complex situations where palliative surgery is planned, death is rarely an immediate outcome. Aggressive, highly technological options and treatments are available and offered even in the final stages of advanced disease. Ongoing successes encourage caretakers and families to pursue continued therapies. Also, the progression of heart failure in children is largely variable, and the point at which there is no possibility for long-term survival is often unclear. Advanced heart disease is often marked by acute decompensations followed by periods of stability. This unpredictable nature often discourages end of life discussions for children and their families.

Because of the trajectory of acute decompensation alternating with periods of stability, many children with advanced heart disease die in the hospital, most often in an intensive care setting as advanced therapies are used even at the end of life. Little data are available for children with advanced heart disease. Fig. 40-1 shows the data from one large Cardiac Intensive Care Unit with approximately 1000 admissions per year. Overall, the ICU mortality is between 2% and 4% each year, which is approximately 30 to 40 deaths yearly. This percentage may vary between institutions and internationally. Although end of life discussions are happening with these families, there are limited data as to when and where these discussions are occurring.

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Fig. 40-1 Overall mortality in a large pediatric cardiac intensive care unit.

(Data provided by the authors.)

Despite ongoing challenges, providing palliative care for children with CHD is a critical need. Key aspects include symptom management, promoting psychosocial health and quality of life, and decision making. Each of these areas will be discussed in the sections that follow.

Symptom management for children with advanced heart disease

Children with advanced heart disease represent a diverse group with complex cardiac issues that result from either palliated complex CHD or from severe forms of cardiomyopathy or post-transplant care. Symptom management includes a variety of complex medical and psychosocial interventions in order to optimize quality of life. As cardiac function deteriorates, traditional symptoms of heart failure can ensue (Fig. 40-2). Symptom management can be complex and side effects of drugs can worsen symptoms. Some guidelines are offered in Table 40-1.

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Fig. 40-2 Pathophysiology of cardiac symptoms in children.

TABLE 40-1 Medication Guidelines

image image

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 advanced heart disease, 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 maintaining muscle tone. In addition, modified exercise programs have been shown to be beneficial to outlook and endurance in patients with CHD.12 For the more advanced heart disease patients, 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 milronone infusion. There is some anecdotal evidence that a short infusion holiday of 3 to 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.

Congestion and/or Fluid Overload

Fluid overload is a common symptom for children, adolescents, and young adults with advanced heart disease. Rarely, they will present with the traditional adult heart failure congestion with rales, respiratory distress, and shortness of breath. More commonly in children however, congestion and heart failure result in gastrointestinal symptoms such as nausea, vomiting, and anorexia. Diuretics are the key therapy for congestive symptoms in heart failure in children, and can often improve symptoms rapidly. Often GI symptoms will respond dramatically to diuretic therapy. 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. 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 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. In addition, timing of diuretics is critical in maintaining good sleep hygiene. Diuretics before bedtime will obstruct sleep. Diuretics should not be given within 4 hours of bedtime.

Dizziness and/or Syncope

Syncope can be a result of primary ventricular arrhythmia, secondary to myocyte fibrosis. This 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 be very disturbing for patients, as it may remind them of the pre-syncopal 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 anithypertensives 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. However, vasodilating medication could be given at bedtime, as dizziness is less likely once the patient is lying down.

Pain

Pain is unusual in the ambulatory advanced heart disease population. However, pain associated with medical procedures, including minor ones, is not unusual. Needle-stick pain is a major source of trauma for children in healthcare settings, including children who require frequent needle sticks. For outpatient procedures, it may be helpful to provide a written prescription for topical anesthetic cream and instructions for application for the parents. Managing inpatient needle sticks with pain prevention protocols, which include distraction techniques and topical anesthetic creams, may reduce the trauma. Pain can also result from excessive fluid overload and edema of neck, abdomen, and extremities.

Although chest pain is rare, it is very frightening and carries multiple meaning for the families. Opioids can improve coronary perfusion, and may have a primary cardiac role in patients with ischemic disease. Discussions about pain control should occur early on. Many families and caretakers worry about the cardiac effects of opioids. Most cardiac ICUs are comfortable with opioids in the post-operative period. They are critical for comfort at end of life, even while life-extending measures are still in play.

Psychosocial health promotion for children with advanced heart disease

Because the trajectory of advanced heart disease is marked by episodes of acute decompensation alternating with periods of stability, children with advanced heart disease may be community dwelling and attending school, community dwelling, and unable to attend school, hospitalized, or in hospice care. Health promotion is necessarily individualized to each child. However, acknowledging this, awareness of the pervasive impact of CHD on the child and family is critical. Children with CHD may have long-term difficulties with physical growth, gross motor development, exercise capacity, behavioral abnormalities, psychiatric abnormalities, school performance, and quality of life.13 Parents may be dealing with both the uncertainty of their child’s illness and the stress of managing complicated feeding regimens, multiple medications, outpatient visits, and diagnostic procedures. Though evidence is limited, this may result in physical and mental health problems in parents or siblings, attenuated education for mothers, and a negative impact on parent employment.14,15

Very little is known about the subset of children with advanced heart disease and their families in this regard. However, it is likely that their difficulties are even more accentuated.

The evidence that CHD has a pervasive impact on the child and family has resulted in a paradigm shift from survival and managing medical morbidity to proactively maximizing not only the physical health, but also the psychosocial health of both affected children and their families for all children with CHD. Therefore, palliative care provides an ideal framework for the long-term management of children with CHD beginning with diagnosis, recognizing that for some, this means beginning palliative care prenatally.

Psychosocial care for children with advanced heart disease and their families

As with any life-threatening chronic illness, CHD impacts the psychosocial health of affected children and families. The exact nature of the risk is difficult to pinpoint given the complexity of adaptation to chronic illness and inconsistencies in both research methods and findings. However, a body of research is emerging indicating that children with CHD may be at risk for a range of psychosocial issues, including behavioral problems, neurodevelopmental abnormalities, difficulty with social functioning, and psychiatric disorders.13,16 As healthcare team members evaluate children with CHD, each of these areas should be considered in order to identify areas for intervention to maximize the child’s quality of life.

Behavioral Problems

A systematic review of psychological adjustment of children with CHD described parental reports of behavioral difficulties ranging from 5% to 41% and at rates in excess of those occurring in normative samples or control groups.17 Both internalizing and externalizing problems are described in the literature.16–19 Further, school-age children who required surgery for CHD in the newborn period were 3 to 4 times more likely to achieve clinically significant scores for inattention and hyperactivity than normative samples.20 Disease severity was not significantly related to behavior problems in a recent meta-analysis.17 However, there is evidence that older age,17,19 deep hypothermic circulatory arrest, cyanosis, older age at repair, and multiple surgical procedures21 may be risk factors for long-term behavior problems. Despite the technological advances, behavioral outcomes of children repaired recently are not statistically different than those of children repaired in previous eras.22 Family characteristics have been associated with behavioral problems in children with CHD, including maternal worry and distress, maternal mental health, parenting style, and single parent household.17,23 Importantly, in a 2007 study,23 59% of the variance in behavioral outcomes was explained by parenting style, maternal worry, marital status, maternal mental health, and cyanotic status. Behavioral problems may affect many areas of quality of life, including family dynamics, school success, and social interactions, and are a key area for assessment and intervention when identified.

Neurodevelopmental Abnormalities

Children with CHD have, in general, higher rates of neurodevelopmental abnormalities than healthy peers13 with a reported incidence as high as 46% of children who require intervention for CHD.24 These abnormalities are multifactorial, including genetic abnormalities, brain abnormalities, and peri-operative factors such as deep hypothermic circulator arrest.13,25 Specific areas of concern include developmental and cognitive delays, sensorimotor abnormalities, and social skills delays.23,24,26–28 Much like behavioral problems, careful evaluation of the neurodevelopment status of children with CHD is very important along with early referral for special services when abnormalities are identified.

Social Functioning

The social milieu of children with CHD is much like that of other children, with an initial focus on the home and family and broadening to an ever-increasing focus on school and peers. Children with CHD and behavioral and/or neurodevelopmental abnormalities may be at particular risk socially because those abnormalities may accentuate differences from their peers. Further, limited exercise capacity may preclude participation in activities with their peers. These factors are critically important because adolescents and young adults with CHD often describe feeling different from their peers29,30 and report low self-esteem.31 School-age children who had undergone cardiac transplantation, most of whom had CHD, described the impact of scars, the need for medication, activity restrictions, and decreased physical endurance on their self-perception.32 Further, they described problems with being bullied and teased by their peers.32 Children with CHD may be more at risk for social difficulties as they mature, given the increasing importance of peer relationships with age. This may be particularly challenging for children with advanced heart failure because fatigue will decrease their ability to participate in social and recreational activities with peers and accentuate differences from peers.

Family Psychosocial Impact

Life-threatening CHD affects the child’s family as well. Parents of children with CHD have higher rates of psychosocial morbidity than parents of either healthy children or those with other chronic medical conditions.33 Research has identified areas of risk for parents, including high care-giving demand, psychological distress, and psychiatric disorders.13 Parents describe high care-giving demands, particularly related to feeding and medication regimens.34 However, they identify psychological stress as their most significant problem15 and at rates in excess of normative samples.35 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.34–38 Parents of children with hypoplastic left heart syndrome may be at particular risk18 as are parents of children with advanced heart failure. Parents also experience significant rates of psychiatric comorbidities, including depression, anxiety, and somatization, and there is evidence that these are chronic in nature.39 Further, parents of children with CHD may also be at risk for post-traumatic stress disorder given the known incidence in parents of children with life-threatening illness.40,41 Risk factors for psychiatric comorbidities include the burden of care giving, social isolation, financial issues, and dissatisfaction with medical care.39 Further, mothers of children with CHD may be at risk for attenuated education and employment.15

Less is known about the psychosocial impact of CHD on siblings. However, there is evidence that healthy siblings of children with CHD have an increased incidence of behavior, school and emotional problems, and depression.16,42 In a 2005 study,43 30% of parents indicated that CHD affected their healthy children in a variety of ways including anxiety, depression, anger, jealousy, recreational opportunities, feeling left out, and perceiving that the child with CHD lived under different rules. Parents of transplanted children and those with cyanotic CHD were more likely to report negative sibling effects.43 Given the described impact of CHD on the psychosocial well-being of the affected child and his or her parents and siblings, palliative care for children with CHD involves attention to the psychosocial health of the entire family.

Promoting Quality of Life for Children and Families

Much of the research describing the quality of life of children with CHD indicates that it is good and/or equivalent to that of healthy children, yet describe abnormalities on both the physical and psychosocial domains of quality of life.13,17,44,45 Little is known about the quality of life of children with advanced heart failure. However, in a 2008 study, 20% of children with CHD reported significantly impaired quality of life, with similar rates for both mild and severe CHD.44 In children and adolescents with CHD, parents and healthcare providers were asked to identify quality of life concerns, all groups more frequently identified physical dimensions, most often physical limitations.46 This is critically important in the subset of children with advanced heart failure 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-age children and/or their parents as negatively affecting the children’s quality of life.46 While medication regimens were also identified by adolescents and their parents, adolescents also noted the negative impact of feeling different from peers and recognition of their own mortality.46 Adolescents also noted positive aspects of CHD, including increased strength as a person and not taking life for granted.46 Research with children with cancer and after heart transplant has demonstrated the utility of asking children a few simple questions to assess quality of life: what makes a good day for you, what makes a bad day for you, and are there some things you would like to do that you cannot?32,47 Although less is known about the quality of life of parents and siblings of children with CHD, in a 2008 study, parents of children with CHD reported significantly worse quality of life than control parents on all dimensions.48

Decision making in children with advanced heart disease

As summarized previously, many children and families with advanced heart disease have long-standing and complex behavioral, psychosocial, and neurocognitive issues that must be clearly understood and evaluated before or in parallel with the decision-making process. There are, however, limited data surrounding the end-of-life care in pediatric cardiology. There are several issues that are specific to children with advanced heart disease that need to be understood in order to help families with decision-making around end of life. The overall level of palliative care needs is most likely similar between cardiac and oncology patients; that is need for symptom relief, and communication and decision-making issues with intense family support. This is complicated by the highly variable trajectory of advanced heart disease with many patients experiencing acute exacerbations followed by periods of stability. The trigger point to discuss palliative decision making versus curative therapy is often hazy, and caretakers do not always agree on that point in time. Care of children with CHD is interdisciplinary from the beginning. However, the shift from a curative approach to palliative care is a critical time for the interdisciplinary team to work together to address symptom relief and end of life options. While the roles of many team members overlap, such as providing support to the child and family, and communicating with the child, family, and other team members, each team member makes a valued contribution to the palliative care of children with advanced heart disease (Table 40-2).

TABLE 40-2 Interdisciplinary Team Approach to Children with Advanced Heart Disease

Team member Contribution to care
Child and family
• Head of the team
• In-depth knowledge of child, family, and illness experience
• Awareness of needs, beliefs, values, and culture
• Make decisions in collaboration with team
• Communicate with one another and team members
• Provide care for child

Cardiology medical team:

Primary cardiologist
Pediatric intensivist
Cardiac surgeon
Advanced practice
nurse

• Assess child and family
• Manage the child’s advanced heart disease and multi-system effects
• Manage symptoms
• Presentation of possible appropriate treatment choices
• Refer to consultants as appropriate
• Assist with decision making
• Provide support to child and family
• Communicate with child, family, and other team members
• Communicate with primary care team

Primary care team:

Pediatrician
Outpatient therapists
Teachers

• Assess child and family
• Manage primary care issues
• Provide support to child and family
• Assist with decision making
• Communicate with child, family, and other team members
Cardiology nursing team
• Assess child and family
• Respond to physical and psychosocial responses to advanced heart disease
• Provide bedside nursing care
• Coordinate care among disciplines
• Advocate for child and family
• Provide support and education to child and family
• Communicate with child, family, and other team members
Palliative care team
• Assess child and family
• Identify goals for care
• Assist with decision making
• Assist with symptom management
• Provide a proactive approach to end-of-life discussions
• Identify resources
• Provide support to child and family
• Communicate with child, family, and other team members
Social worker
• Assess child and family
• Respond to child and family psychosocial needs
• Advocate for child and family
• Assist with coping with illness and hospitalization
• Provide support to child and family
• Identify resources, including financial, and refer as appropriate
• Communicate with child, family, and other team members
Psychologist
• Assess child and family
• Respond to psychological needs
• Provide support to child and family
• Communicate with child, family, and other team members
Respiratory therapist
• Assess child and family
• Manage respiratory care
• Provide support to child and family
• Communicate with child, family, and other team members
Child life specialist
• Assess child and family
• Prepare child and family for procedures and provide support during
• Provide opportunity for medical play, distraction, and therapeutic play
• Assist with coping with illness and hospitalization
• Provide support to child and family
• Communicate with child, family, and other team members
Physical and occupational therapist
• Assess child and family
• Help child maintain function
• Coordinate rehabilitation
• Provide support to child and family
• Communicate with child, family, and other team members
Nutritionist
• Assess child and family
• Manage nutritional and growth needs
• Provide support to child and family
• Communicate with child, family, and other team members
Pharmacist
• Assess child and family
• Collaborate with team for safe management of complex medication regimens
• Educate child and family regarding medications
• Communicate with child, family, and other team members
Chaplain
• Assess child and family
• Provide spiritual care and respond to spiritual needs
• Provide support to child and family
• Provide support for decision making within the context of religious and spiritual beliefs
• Communicate with child, family, and other team members

High Technological Interventions and Advanced Planning

Decisions regarding technological devices are another important component to decision making. The wider acceptance of mechanical support in the form of ventricular assist devices and extracorporeal membrane oxygenation (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 advanced heart disease. Families and caretakers can hold on to the hope of recovery or transplant while on a device, while also making preparations and plans for symptom relief and minimizing suffering. This balance is delicate. Reliance on mechanical support strategies will provide an important new area for understanding palliative care needs in advanced heart disease patients, and in children in particular.

Many children who progress to advanced heart disease already have an implantable defibrillator. 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.

Because of the advanced nature of the heart disease of children who require ECMO or ventricular assist device support, there may be instances when withdrawing mechanical support is a consideration. Because both means of support require anticoagulation, there is a risk of hemorrhagic stroke. Children may develop multi-system organ failure that precludes transplantation. In those settings, many families elect to withdraw support. The difficulties for families with a ventricular assist device (VAD) often revolve around the patient, who may be cognitively aware and neurologically intact, with the heart beating, with severe end-organ dysfunction. This type of withdrawal by terminating the pump function feels different to the medical caretakers and families than withdrawal of an endotracheal tube. In a review of caretakers following the withdrawal of intervention support in two patients, medical caretakers identified significant psychological distress regarding discontinuation of VAD support.49 Formal institutional policy for VAD patients at end of life may help improve medical caretakers and family stress around this issue. These issues will need to be addressed with the intensive care unit teams and families together, in a sensitive and proactive manner.

Advance planning for children with advanced heart failure should include discussion of high-technology interventions such as ECMO and VAD. Many families choose to do everything possible. It should also include consideration of less technologically intensive interventions such as cardiac catheterizations, non-invasive cardiac imaging studies, and blood draws. Many families choose high-risk surgical or interventional procedures with uncertain outcomes because they do not know what may lie ahead otherwise. Families and medical caretakers need to understand the limitations of doing everything. Cardiologists and cardiac surgeons are not always good at validating the choice some families have to preserve independence and not push forward. We must recognize individual differences in preferences for quality of life vs. survival for these children. Key considerations include: impact of the interventions on the child’s survival, impact on the child and family’s quality of life, and impact on current management. In collaboration with the interdisciplinary team, families may elect to forego interventions based upon burden and/or ambiguous outcomes.

Clinical Vignette

AB was diagnosed with cardiomyopathy at 1 year of age and underwent cardiac transplantation without complication. His course was uncomplicated for the first 5 years post transplant and he was managed as an outpatient by the multi-disciplinary team. At 5 years of age, he presented to clinic because his mother observed that he “did not look right,” had poor appetite, and was uninterested in play. On echocardiogram, his cardiac function was very poor and he had evidence of myocarditis, rejection, and cardiac allograft vasculopathy. He was admitted to the intensive care unit for intravenous inotropes to maintain cardiac output. Because of progressive cardiac allograft vasculopathy, persistent poor cardiac function, and continued need for high-dose inotropes, he was re-listed for transplant. Work-up revealed exposure to parvovirus, a likely cause of his acute decompensation. His mother, who was pregnant, was also exposed to parvovirus and delivered at 33 weeks’ gestation. The infant died in the newborn period of viral sepsis and cardiomyopathy. AB’s physical condition stabilized on IV inotropes and symptoms were manageable medically. However, AB’s and his family’s psychosocial care was particularly challenging as the team worked to help the family cope with both AB’s need for re-transplant and the loss of the premature infant.

AB underwent his second cardiac transplantation 40 days after admission and had a complicated course, including the need for ECMO on post-operative day 1 for graft failure. Once more, the family required intensive psychosocial support from the multi-disciplinary team as they worked to managed the uncertainty of AB’s condition in the context of the recent loss of their newborn. His rejection was managed medically and he was weaned off ECMO after 6 days. His course thereafter was uncomplicated. However, he required intensive rehabilitative physical and occupational therapy to restore function after his lengthy critical illness. He was discharged to home just 3 weeks after transplant with outpatient medical management including physical and occupational therapy.

Two years later, AB presented with respiratory distress and was diagnosed with cytomegalovirus pneumonia and was successfully treated. His hospital stay was less than 2 weeks and he was discharged home for continued outpatient medical management. Within 6 months of this admission, he developed signs of heart failure and evaluation revealed cardiac allograft vasculopathy, which once again progressed rapidly. When he required intravenous inotropes for his decompensated heart failure, treatment options, including ECMO and VAD, were considered. Because he had developed cardiac allograft vasculopathy with both his first and second transplant, he was not a candidate for a third transplant. Because AB did not have a long-term option, the family and team elected to forego mechanical support and concentrate on supportive care. AB had a wish to see the Disney princesses, therefore the team and family focused on transitioning AB to oral medications to manage his heart failure. AB was discharged home on oral medications for heart failure and went to Disney World with his family. He rode all the rides and saw all the Disney princesses. On the last day of the trip, he was laughing on the water ride with his mom and sister, “fell asleep,” and died.

Future Issues

Although there are descriptive studies that help identify areas for palliative care intervention in children with advanced heart disease, few interventions are described in the literature. Therefore, the next steps in the palliative care of children with advanced heart disease includes the need for development and testing of interventions to improve the multidimensional health and quality of life for children with CHD. Further, very little is known about the spiritual health and end of life issues for children and families, even from a descriptive standpoint.

The role for palliative care for children with advanced heart disease is expanding. Although many issues are similar to other life-threatening chronic illnesses, it is critical to understand the differences in the cardiac population in order to maximize the impact of palliative care for children with advanced heart failure and their families. A critical first step in improving palliative care is gaining understanding of and identifying clear prognostic indicators for children with advanced heart disease. This will help the healthcare team better understand which patients will benefit from an early palliative approach. In addition, it is critical to educate physicians and families that using a palliative care model supports maintaining hope for long-term survival while also providing symptom support and decision-making structure. Lastly, research studies attempting to understand bereavement issues for the families around end of life will aid in developing future interventions for this growing population.

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49. Thiagarajan R: Personal communication, abstract in submission.

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