Advanced Heart Disease - Clinical Tree

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. 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, and the incidence of cardiomyopathy is estimated at 0.58 per 100,000 children. Yet only a fraction of children diagnosed with either form of pediatric heart disease eventually progresses to advanced heart disease necessitating long-term intensive medical management and/or recurrent hospitalization. Those that do are at high risk for developing end-stage heart disease, 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. 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. CHD is the leading cause of infant deaths owing to congenital anomalies worldwide. Between 1940 and 2002, approximately 2 million infants were born with CHD in the United States. 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. 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. 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 review 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. 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. 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.

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

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 .

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

TABLE 40-1

Medication Guidelines

Adapted from Miller-Hoover, S.R. Pediatric and Neonatal Cardiovascular Pharmacology. Pediatric Nursing 2003; 29(2): 105–115. Reprinted with permission of Janetti Publications, Inc. www.pediatricnursing.net

Medication	Indication for use	Dosing	Onset	Side effects	Half-life	Metabolism	Contraindications
Dopamine	To increase cardiac output, blood pressure, and urine output. Volume depletion should be corrected before starting dopamine.	Renal and mesenteric vasodilation: 2–5 mcg/kg/min (beta) effect: 5–10 mcg/kg/minIV infusion	Within 5 min	Tachydysrhythmias, vasoconstriction, at higher doses. Anginal pain, and palpitations	2 min	Metabolized in the plasma, kidneys, and liver	Hypersensitivity to sulfites. Care must be given to ensure that extravasation does not occur. Extravasation causes severe tissue necrosis
Dobutamine	To increase cardiac output and to manage short-term cardiac decompensation. Volume depletion should be corrected before starting dobutamine.	2–20 mcg/kg/min IV infusionTransient hypotension	1–10 min	Ectopic heart beats, increased heart rate, chest pain, headache, nausea and vomiting, dyspnea	2 min	Metabolized in the tissues and liver	Hypersensitivity to sulfites and idiopathic hypertrophic subaortic stenosis
Milrinone	To treat low cardiac output syndrome and congestive heart failure. Increase cardiac output and stroke volume, decrease intracardiac filling pressures, and decrease systemic vascular resistance without changing heart rate or myocardial oxygen consumption. Milrinone has fewer side effects.	Maintenance infusion: 0.2 to 1 mcg/kg	5–15 min	Headache, ventricular dysrhythmias, hypotension, and chest pain	1–5 hrs	Excretion by the kidneys	Severe pulmonary or aortic obstructive disease and hypersensitivity to milrinone
Furosemide	A loop diuretic used to reduce preload. Furosemide as either a continuous drip or intermittent boluses can be given.	IV infusion: 0.05 mg/kg/hr titrated to clinical effect. Intermittent IV: 1–2 mg/kg/dose every 6–12 hrs2 mg/kg orally 3 times a day	IV: 5 minPO: 30–60 min	Hypotension, dizziness, uticaria, electrolyte imbalances, potential ototoxicity with high doses, jaundice	Normal renal function 30 min; with renal failure—9 hrs	Metabolized by the liver in 80% excreted in the urine	Indomethacin decreases effect
Bumetanide	A loop diuretic is indicated to reduce preload	0.015–0.1mg/kg/dose, given every 6–24 hrs IV/PO	Within a few minutes when given IV	Hypotension, chest pain, dizziness, rash, hyperglycemia, electrolyte imbalance, elevated liver enzymes, ototoxicity, elevated serum creatinine	Ranges from 1 hr to 2½ hrs	Partially metabolized in the liver and excreted in the urine	Hypersensitivity to bumetanide and anuria or increasing azotemia
Spironolactone	A potassium-sparing diuretic acting on the distal collecting duct of the nephron. This drug is indicated for treatment of CHF. May be used in conjunction with Lasix and digoxin	1–2 mg/kg/day orally given once a day or divided into 2 doses given every 12 hrs	1 to 3 hrs	Dysrhythmias, lethargy, confusion, ataxia, rash, electrolyte imbalance, dehydration, decreased renal function		Metabolized in the liver and excreted in the urine and biliary tracts	If given with potassium supplements, may increase potassium serum levels. Spironolactone, when given with potassium supplements, may potentiate inotropic affect of digoxin while decreasing digoxin clearance
Prostaglandin E ₁	An endogenous fatty acid indicated to maintain the patency of the ductus arteriosus. This is vitally important with ductul dependent lesions such as severe coarctation of the aorta, critical pulmonary stenosis, and transposition of the great arteries	0.05 mcg/kg/min may be doubled every 15–30 min up to a maximum dose of 0.2 mcg/kg/min until desired effect is obtained. The infusion may be decreased to the lowest effective dose once ductal patency is achieved	Relatively short, with immediate effects	Apnea, hypotension, seizures, flushing, elevated temperature		Metabolized rapidly in the pulmonary circulation and excreted by the kidneys

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. 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.

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