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U N I V E R S I T Y O F M I N N E S O T A A m p l at z C H I L D R E N ’ S H O S P I T A L
in the adverse remodeling of the myocardium and vasculature that to evaluate atrial and ventricular size, pulmonary artery pressure
occurs in chronic heart failure. The overall net effect is an increased and cardiac function.
systemic vascular resistance and increased myocardial fiber length
Older infants and children with heart failure have signs and
classically described by Starling.
symptoms similar to those of adults. Children might have
shortness of breath with dyspnea that is exaggerated by exercise.
Clinical Presentation
A chronic cough secondary to pulmonary congestion might be
Four cardinal signs of heart failure in children present. Symptoms of congestive heart failure in children might
• Tachypnea be subtle but often an intercurrent illness will be enough to
• Tachycardia exacerbate the underlying hemodynamic abnormalities and allow
• Cardiomegaly congestive heart failure to become apparent.
• Hepatomegaly
Fatigue and weakness are late findings. On physical examination,
The signs and symptoms of heart failure vary depending on the age
children with mild to moderate heart failure might not appear
at presentation and the underlying etiology. The clinical findings
in distress; however, children with more severe heart failure
are different in infants versus older children and adolescents.
might demonstrate dyspnea and tachypnea at rest. A child or
Infants with heart failure typically present with feeding difficulties adolescent, who cannot speak in full sentences, is on the verge of
as this is one of their most demanding physical activities. They cardiorespiratory failure.
might take more time to feed and have associated tachypnea,
Children with chronic heart failure sometimes appear
tachycardia and diaphoresis. Growth failure is a classic feature in
malnourished and pale. Distention of the neck veins can be
infants with congestive heart failure. Alternatively, infants who
difficult to appreciate but reveals increased systemic venous
have chronic cough that are unresponsive to typical respiratory
pressure. Hepatomegaly is typically present and if the heart
therapies might be exhibiting signs of heart failure.
failure is acute in nature, associated flank pain might occur as
Physical examination of infants with heart failure will reveal resting a result of stretching of the liver capsule. Once an increase in
tachycardia and tachypnea. As HF symptoms progress, they might body weight oocurs - approximately 10 percent - the child might
develop signs of respiratory insufficiency including nasal flaring, develop peripheral edema in dependent parts of the body. In
retractions, and grunting. The cardiac exam is variable, depending more severe cases, children might develop ascites, pleural and
on the etiology of heart failure. Murmurs might be present. Infants pericardial effusions. Occasionally, presacral edema is seen in
with cardiomyopathy might have a mitral regurgitation murmur young as well as elderly recumbent persons in heart failure.
and a third heart sound. The third heart sound, however, may be
Cardiac exam is variable depending on the etiology and presence
difficult to appreciate with the rapid heart rate. Infants with HF
of congenital heart disease. A murmur might be audible from a
will have hepatomegaly associated with increased systemic venous
large systemic to pulmonary shunt or from atrioventricular valve
pressure and/or volume. Peripheral edema is rare in infants.
regurgitation. A gallop heart sound with a third and possibly a
With severe heart failure and low cardiac output, infants might fourth heart sound might be heard. Palpation of the chest can
have cool extremities, weak pulses, low blood pressure, mottling reveal a laterally displaced or diffuse apical impulse, and a right
of the skin and delayed capillary refill. A chest X-ray will typically ventricular heave.
show cardiomegaly. Most infants with HF have some degree of
The diagnosis of heart failure is sometimes made serendipitously
pulmonary venous congestion, which appears as a diffuse haziness
by ordering a chest X-ray for other reasons. The chest X-ray
on the chest X-ray. Infants with large systemic to pulmonary
in congestive heart failure typically shows cardiomegaly and
shunts show increased pulmonary vascular markings. Although
interstitial pulmonary edema, which, in more severe cases, causes
an electrocardiogram might be abnormal and provide clues to
diffusely hazy lung fields. Cardiac ultrasound is the primary
the diagnosis, such as the presence of Wolff-Parkinson-White
modality for defining the cardiac anatomy and for assessing
syndrome, it does not usually help in defining heart failure severity.
ventricular function in children with heart failure. Although
Cardiac ultrasound provides the most useful information in the
initial diagnosis of congenital heart disease in an older child
evaluation of infants with heart failure. The imaging defines the
is rare, some children who have undergone previous palliative
underlying cardiac anatomy. The imaging also allows practitioners
surgeries for congenital heart disease may later develop heart
failure several years after the palliation.
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H eart F ai l ure in C hi l dren
Etiologies
There is a wide spectrum of etiologies that can lead to heart
failure in children. Tables 1 and 2 summarize the more common
etiologies of heart failure in pediatrics, organized by the presence
of a structurally normal heart (Table 1) or the presence of
congenital heart disease (Table 2).
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U N I V E R S I T Y O F M I N N E S O T A A m p l at z C H I L D R E N ’ S H O S P I T A L
echocardiogram from a patient with dilated cardiomyopathy, in Cardiac ultrasound, 12-lead electrocardiogram, and chest X-ray
comparison to a normal echocardiogram. Most patients will not are standard for the initial workup. Cardiac ultrasound is the
develop symptoms until their function is severely diminished, most useful tool for evaluation of congenital heart disease and
with ejection fractions of 25 to 30 percent (with normal being ventricular function. Standard laboratory evaluation includes
>55 percent). In the acutely decompensated state, such patients thyroid-function testing, hemoglobin and carnitine levels.
might require inotropic or mechanical support. Many of them, Additionally, patients typically undergo laboratory assessment of
however, will remain stable for a prolonged period with oral the severity of heart failure by measurement of brain-anatriuretic
medication treatments, before later progressing to require cardiac peptide (BNP), troponin, and by evaluation for markers of renal
transplantation. and hepatic end-organ dysfunction.
Another increasingly important category of patients who have In the absence of structural cardiac disease, evidence for
heart failure are adolescents or adults with congenital heart myocarditis is sought by viral culture and polymerase chain
disease. Almost any patient who had repair or palliation of reaction (PCR) analysis. Genomic testing of the more common
congenital heart disease can later develop cardiac dysfunction forms of familial dilated cardiomyopathy is indicated if a first-
and HF. This might relate to poor myocardial preservation degree relative has a cardiomyopathy. Urine organic acids and
during earlier surgeries, ventricular dysfunction from multiple serum amino acids are tested to rule out the possibility of
bypass runs, arrhythmias, residual defects or progressive valvular metabolic disorders. Skeletal muscle biopsy can be obtained if
dysfunction despite repair. Patients with two specific congenital there is suspicion of systemic mitochondrial or metabolic disease,
anomalies are particularly prone to the late development of or if cardiac muscle biopsy is considered to be high risk.. After
congestive heart failure. One is single ventricle after Fontan stabilization of the child, completion of cardiac catheterization
procedure, the other is d-transposition of the great vessels with endomyocardial biopsy often helps to determine
palliated with an atrial baffle procedure. Adolescents or adults hemodynamics and establish a diagnosis. Figure 2 provides a
with repaired or palliated congenital heart disease and heart paradigm for the stepwise approach to this diagnostic workup.
failure often benefit from a referral to a pediatric cardiologist who
is familiar with the natural history of these repaired anomalies. Management
These two groups of patients represent a large proportion Therapy for heart failure depends on the age of the patient,
of adults with congenital heart disease who are referred for specific cardiac diagnosis, time course of the symptoms (acute
consideration for cardiac transplantation. versus chronic), and existence of other underlying conditions.
Practitioners should customize treatment plans based on these
Diagnosis factors.
The diagnostic approach for patients with heart failure Just as heart failure is a continuum — with symptoms ranging
depends on: from mild to life-threatening, so is the management. Therapies
• Age of patient range from outpatient administration of oral medications
to listing for cardiac transplantation and implantation of
• Presence or absence of congenital heart disease
left ventricular-assist devices. The goal in managing acutely
• Presence of systemic disorders
decompensated heart failure is to restore pump function to
• Severity of heart failure improve hemodynamic instability, improve symptoms and
Patients who have signs and symptoms of congestive heart failure minimize end-organ damage.
should have an evaluation by a pediatric cardiologist. Depending
First-line therapy for mild to moderate congestive heart failure
on the child’s presentation, the evaluation might take place on an
includes diuretics such as furosemide (Lasix) or bumetanide
outpatient basis during the course of a few days. Patients also can
(Bumex) with angiotensin-converting enzyme inhibitor (ACE) or
have an inpatient evaluation — sometimes on an intensive care
angiotensin-recptor blocker (ARB) medications such as enalapril
unit — over the course of a few hours.
and losartan and beta-blocker therapy such as carvedilol. See table
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H eart F ai l ure in C hi l dren
The newest device available for pediatric use is the Berlin Heart • Progressive deterioration of ventricular function or functional
EXCOR. It is commercially available in Europe and is available status despite optimal medical care
on a compassionate use or FDA study use in the United States. • Malignant arrhythmia
The Berlin Heart is available in multiple pump sizes for various • Survival after cardiac arrest unresponsive to medical
patient sizes. To date, more than 500 patients worldwide have treatment, catheter ablation or AICD
received support on this device, and over 200 patients within • Progressive pulmonary hypertension that could preclude
North America. transplantation at a later date
Through 2008, the survival rates for the North American • Growth failure secondary to severe heart failure
experience are approaching 75 percent in the current era; the • Unacceptably poor quality of life secondary to heart failure
most recent outcomes are shown in figure 3. The University of • Progressive deterioration in functional status and/or presence
Minnesota is one of 15 centers in the country that is an FDA of certain high-risk conditions following the Fontan
study site for the Berlin Heart. Since 2008, our program has procedure
implanted devices in five children, and have outcomes similar to
the national data.
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U N I V E R S I T Y O F M I N N E S O T A A m p l at z C H I L D R E N ’ S H O S P I T A L
Once it is determined that a child needs to be listed for heart hemodynamic stability and minimizing end organ damage. In
transplantation, the child is entered into the United Network the patient with chronic heart failure, therapies are directed at
for Organ Sharing (UNOS) wait list. Each candidate awaiting improving long-term outcomes by minimizing inflammatory and
heart transplant is assigned a status code, which corresponds to fibrotic changes to the myocardium and systemic and pulmonary
how medically urgent it is that the child receive a transplant. vascular systems. Some patients might have progression of their
The criteria for the different status levels for pediatric heart heart failure despite optimal medical therapy. New ventricular-
transplant candidates are shown in table 4. Depending on the assist devices in development for pediatric patients offer another
severity of their illness, children awaiting transplantation might therapeutic option with the hopes of decreasing wait-list mortality
be hospitalized requiring mechanical or ventilatory support, for children who are awaiting heart transplantation. Heart
intravenous inotropic medications, intravenous inotropic transplantation remains a viable option for patients with end-stage
medications at home, or oral medications at home. heart failure, with improving survival outcomes in the most recent
A computerized match system, that UNOS operates, matches decade. However, wait-list mortality due to limited donor organ
donors and recipients based on blood type, age, size, listing status, availability and long-term morbidity and mortality associated with
wait-list time and location. transplantation continue to be an issue.