Basics
Description
Heart failure (HF) is the pathophysiologic state in which the heart is unable to pump sufficient blood to meet the metabolic demands of the body.
Risk Factors
- In utero
- Arrhythmias: supraventricular or ventricular tachycardia, complete heart block (CHB)
- Volume overload: atrioventricular (AV) valve regurgitation or arteriovenous malformation (AVM)
- Primary myocardial disease: cardiomyopathy (dilated, hypertrophic), myocarditis
- Anemia: Rh isoimmune disease, thalassemia, and twin-twin transfusion
- Premature closure of ductus arteriosus with isolated right ventricular failure
- In neonates
- Myocardial dysfunction: asphyxia, acidosis, myocarditis, hypoglycemia, cardiomyopathy (dilated, hypertrophic, ventricular noncompaction), ischemia (anomalous left coronary artery from the pulmonary artery), metabolic defects, or pressure overload imposed by aortic stenosis, pulmonary hypertension, or coarctation of the aorta
- Volume overload: atrial septal defect (ASD) (large), ventricular septal defect (VSD) (large), patent ductus arteriosus (PDA) (moderate to large), truncus arteriosus, aortopulmonary window, anomalous pulmonary venous return, AVM in any location
- Arrhythmias: supraventricular or ventricular tachycardia, CHB
- Left heart inlet obstruction: mitral stenosis, cor triatriatum, pulmonary venous obstruction
- Note: Certain cyanotic heart diseases such as hypoplastic left heart syndrome may present with elevated pulmonary blood flow or depressed systemic blood flow and minimal desaturation. These patients may have HF in the 1st day of life due to increased pulmonary circulation or due to shock from ductal closure.
- In infants
- Myocardial dysfunction: cardiomyopathy (dilated, hypertrophic, restrictive, ventricular noncompaction), endocardial fibroelastosis, metabolic/mitochondrial diseases, myocarditis, Kawasaki disease, anomalous left coronary artery from pulmonary artery, or chronic pressure overload due to coarctation of the aorta or aortic stenosis
- Volume overload: ASD (large), VSD, PDA, common AV canal defect, partial anomalous pulmonary venous connections.
- Secondary causes: renal disease (volume overload, electrolyte disturbance), hypertension, hypothyroidism, sepsis
- Arrhythmias: supraventricular or ventricular tachycardia, CHB
- Pericardial effusion due to juvenile rheumatoid arthritis (JRA), systemic lupus erythematosus (SLE), other inflammatory diseases, or following repair of congenital heart disease (CHD)
- In childhood and adolescence:
- Unrepaired CHD with volume and/or pressure overload
- Repaired CHD with residual defects that result in volume and/or pressure overload
- Acquired heart disease: pericarditis, myocarditis, endocarditis, acute rheumatic fever
- Cor pulmonale (pulmonary hypertension, Eisenmenger syndrome, chronic lung disease)
- Cardiomyopathy due to primary myocardial disease (dilated, hypertrophic, restrictive, ventricular noncompaction), chemotherapy (anthracyclines), radiation therapy, sickle cell anemia, thalassemia, neuromuscular disease (e.g., Duchenne or Becker muscular dystrophy)
General Prevention
- Limited use of anthracycline drugs in cancer therapy
- Prompt treatment (within 10 days) of streptococcal pharyngitis to prevent rheumatic fever
- SBE prophylaxis to prevent infective endocarditis
Alert
- In patients with HF due to large left-to-right shunts, long-term spontaneous clinical improvement of HF with decreased murmur may indicate the development of pulmonary vascular disease (Eisenmenger syndrome), eventually leading to cyanosis.
- Care must be used in the administration of oxygen to the undiagnosed infant with heart disease. A patient with single-ventricle physiology (e.g., hypoplastic left heart syndrome) can have manifestations of HF and mild desaturation (92-98%). Providing oxygen in this situation can result in shock due to excessive pulmonary blood flow and inadequate systemic blood flow.
Etiology
- Low cardiac output HF (e.g., all cardiomyopathy, severe AV valve regurgitation)
- High cardiac output HF
- Left-to-right shunts (e.g., ASD, VSD, PDA)
- AVM
- Severe anemia, beriberi, and hyperthyroidism
Diagnosis
History
- Infants and neonates
- Prolonged feedings associated with tachypnea, retractions, or diaphoresis
- Emesis, inadequate caloric intake, irritability with feeding, and failure to thrive
- Frequent respiratory infections. Orthopnea: "Spoiled baby" becomes distressed when supine.
- Family history of HF or sudden unexpected deaths
- Sweating, especially when supine/sleeping
- Childhood and adolescence
- Exercise intolerance with exertional dyspnea
- Palpitations or chest pain, especially during exercise
- Chronic cough, wheezing, orthopnea, fatigue, weakness, anorexia, nausea, and edema
- Gradual weight loss (anorexia, nausea, and increased metabolic demands)
- Sudden weight gain (fluid retention)
- Family history of HF or unexpected deaths
- Sweating, especially when supine/sleeping
Physical Exam
- Infants and neonates
- Tachycardia
- Gallop rhythm
- Murmur of outflow obstruction, increased flow, AV valve regurgitation, VSD, or semilunar valve incompetence
- Systolic click (semilunar valve abnormalities)
- Abnormal second heart sound (fixed split, loud P2 component)
- Tachypnea, wheezing, crackles, rales
- Nasal flaring, grunting, retractions
- Abdominal or cranial bruit
- Hepatomegaly, splenomegaly
- Edema (periorbital)
- Cool and/or mottled extremities
- Poor capillary refill or pulses
- Children and adolescents
- Tachycardia
- Gallop rhythm
- Murmur of outflow obstruction, increased flow, AV valve regurgitation, VSD, or semilunar valve incompetence
- Loud second heart sound (P2 component)
- Hyperactive precordium, displaced PMI
- Tachypnea, retractions
- Wheezing ("cardiac asthma") or rales
- Jugular venous distension
- Hepatomegaly, splenomegaly
- Edema (periorbital, peripheral)
- Pulsus alternans, pulsus paradoxus
- Cool extremities, poor pulses, poor capillary refill
- Manifestations of Kawasaki disease, rheumatic fever, or endocarditis on mucous membranes, skin, and extremities
Diagnostic Tests & Interpretation
Diagnostic Procedures/Other
- Chest x-ray
- Cardiomegaly, increased pulmonary vascular markings, hyperinflation, pleural effusion, Kerley B lines
- Electrocardiography
- Abnormal P-waves
- ST-T wave changes (ischemia, strain, inflammation/myocarditis)
- Heart block (1st-, 2nd-, 3rd-degree) or tachyarrhythmia
- Disease-specific findings: anomalous left coronary artery from the pulmonary artery (Q waves and inverted T waves in leads I and aVL, left ventricular hypertrophy, right ventricular hypertrophy, ischemic changes), restrictive cardiomyopathy, pericarditis pattern (diffuse ST segment changes, low voltages), hypertrophy (cardiomyopathy, CHD, storage disease)
- Echocardiography
- Rule out CHD, evaluate coronary origins
- Assessment of cardiac systolic and diastolic function
- Cardiac catheterization (in select cases)
- Assessment of cardiac hemodynamics and anatomy
- Endomyocardial biopsy may be helpful in the diagnosis of myocarditis, storage disease, or cardiomyopathy.
- Electrophysiologic study to evaluate for the induction of arrhythmia
- Cardiac MRI or CT (in select cases)
- Delineation of complex anatomic relationships
- Right ventricular performance
- Other laboratory abnormalities
- Blood gas: metabolic acidosis with elevated lactate
- Chemistry: hyponatremia (dilutional), prerenal state
- Blood counts: anemia, leukocytosis, or leukopenia (e.g., viral myocarditis)
- Elevated erythrocyte sedimentation rate (e.g., rheumatic fever, Kawasaki disease)
- B-type natriuretic peptide (BNP or NT-BNP) elevation
- Urine: proteinuria, high urine specific gravity, microscopic hematuria
- Evaluation for metabolic causes of cardiomyopathy may include pyruvate, amino acid quantification, urine organic acids, carnitine, selenium, acylcarnitine profile, and liver function tests.
- Viral evaluation (adenovirus, coxsackievirus, Epstein-Barr virus, cytomegalovirus, parvovirus, echovirus)
Differential Diagnosis
- Tachycardia
- Fever
- Dehydration
- Anemia
- Supraventricular tachycardia or ventricular tachycardia without HF
- Hyperthyroidism
- Pericardial effusion
- Tachypnea
- Respiratory disease or infection
- Pulmonary venous obstruction
- Acidosis (metabolic disease, poisoning)
- Pneumothorax, pleural effusion
- Carbon monoxide poisoning
- Edema
- Hypoalbuminemia
- Systemic inflammatory conditions/allergies
- Hypothyroidism
- Sepsis
- Hepatomegaly
- Liver disease
- Storage disease
- Extramedullary hematopoiesis
Treatment
General Measures
- Treatment of underlying cause
- Surgical palliation or correction of CHD
- Interventional cardiac catheterization (e.g., balloon dilation of aortic or pulmonary stenosis, coil embolization of PDA, device closure of ASD, dilation, or stenting of coarctation of the aorta)
- Carnitine, coenzyme Q10, riboflavin, antioxidant replacement for select cardiomyopathies
- Targeted medical treatment of endocarditis, myocarditis, anemia, rheumatic fever, Kawasaki disease, or hypertension
- Radiofrequency ablation of tachyarrhythmia
- Medical therapy for patients or mothers of fetuses with tachyarrhythmia
- Pacing for bradyarrhythmias (e.g., heart block)
- Control of chronic inflammatory conditions, such as SLE or JRA
- Management
- Assessment of degree of illness:
- If perfusion is compromised or acidosis is present, ICU care is indicated.
- Hospitalization may be necessary to initiate treatment or prepare for surgery (e.g., coronary abnormalities, aortic coarctation).
- Many patients diagnosed as outpatients with CHD or cardiomyopathy may not require inpatient treatment. Immediate consultation with a pediatric cardiologist should be arranged.
- Immediate management
- General measures: activity restriction as indicated, oxygen as needed (not for patients with pulmonary overcirculation)
- Tube feedings for severe failure to thrive or parenteral nutrition if there is concern for splanchnic circulation
- Drainage of pericardial effusion, if needed.
- Inotropic agents (digoxin, milrinone, dobutamine in refractory cases)
- Intravenous immunoglobulin (IVIG) for myocarditis or Kawasaki disease
- Loop diuretics (e.g., furosemide)
- Nesiritide (synthetic BNP) for refractory fluid overload
- Mechanical respiratory or circulatory support, if necessary (ventilator, extracorporeal membrane oxygenation, ventricular assist device)
- Chronic therapy
- Digoxin
- Loop diuretics (e.g., furosemide) for fluid overload/edema
- Afterload reduction (e.g., angiotensin-converting enzyme [ACE] inhibitors)
- Antagonism of activated neurohormonal systems: ACE inhibitor or angiotensin receptor blocker, spironolactone, beta-blocker
- Anticoagulation or antiplatelet therapy (especially in restrictive and severe dilated cardiomyopathy)
- Biventricular pacing/resynchronization in some cases
- Heart and heart/lung transplantation in select cases
Ongoing Care
Follow-up Recommendations
- Dependent on the etiology and degree of HF. Generally, initial follow-up of a patient with HF should be intensive, focused on assessing response to therapy. Initial follow-up generally weekly, spreading to monthly or quarterly over time, under the supervision of a pediatric cardiologist.
- Depending on the etiology and degree of HF, echocardiography, ECG, blood chemistry, BNP levels, INR, Holter monitoring, and chest radiography will be evaluated.
Additional Reading
- Aurbach SR, Richmond ME, Lamour JM, et al. BNP levels predict outcome in pediatric heart failure patients: post-hoc analysis of the Pediatric Carvedilol Trial. Circ Heart Fail. 2010;3(5):606-611. [View Abstract]
- Kay JD, Colan SD, Graham TP Jr. Congestive heart failure in pediatric patients. Am Heart J. 2001;142(5):923-928. [View Abstract]
- Kindel SJ, Miller EM, Gupta R, et al. Pediatric cardiomyopathy: importance of genetic and metabolic evaluation. J Card Fail. 2012;18(5): 396-403. [View Abstract]
- Rosenthal DN. Cardiomyopathy in infants: a brief overview. Neoreviews. 2000;1(8):e139-e145.
- Shaddy RE. Optimizing treatment for chronic congestive heart failure in children. Crit Care Med. 2001;29(10)(Suppl):S237-S240. [View Abstract]
- Towbin JA, Bowles JA. The failing heart. Nature. 2002;415(10):227-233. [View Abstract]
- Wilkinson JD, Landy DC, Colan SD, et al. The pediatric cardiomyopathy registry and heart failure: key results from the first 15 years. Heart Fail Clin. 2010; 6(4):401-413. [View Abstract]
Codes
ICD09
- 428.0 Congestive heart failure, unspecified
- 779.89 Other specified conditions originating in the perinatal period
- 746.7 Hypoplastic left heart syndrome
ICD10
- I50.9 Heart failure, unspecified
- P29.0 Neonatal cardiac failure
- Q23.4 Hypoplastic left heart syndrome
SNOMED
- 42343007 congestive heart failure (disorder)
- 276514007 neonatal cardiac failure (disorder)
- 62067003 Hypoplastic left heart syndrome (disorder)
FAQ
- Q: My child has a large VSD and is prescribed digoxin and furosemide. Should I take salt out of his diet?
- A: No. Excessive salt restriction is seldom enforceable and is not necessary. A no-added-salt diet is generally sufficient.
- Q: What is the importance of tachycardia and bradycardia in HF?
- A: Tachycardia limits diastolic filling time and may result in decreased cardiac output. However, bradycardia may be poorly tolerated in patients with HF and a relatively fixed stroke volume who are dependent on heart rate to maintain an appropriate output. Either may be problematic for a patient in chronic HF. Most HF patients do not have as much heart rate variation as healthy people.
- Q: What are the major causes of death to HF patients?
- A: Younger children tend to die of progressive HF. Ventricular arrhythmias are the most common cause of sudden death in older children and adults with HF. Other causes of mortality include infection and stroke.
- Q: My patient has a normal blood pressure, but the cardiologist says more ACE inhibition is necessary. Why?
- A: Blood pressure is the weight that the myocardial muscle must "lift" with every beat. By decreasing the blood pressure as much as possible (short of causing dizziness or syncope), the work done by the heart and myocardial oxygen consumption are reduced. Reduction of the systemic blood pressure also potentially reduces the amount of left-to-right shunting through a VSD, PDA, and AP window.