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Heart Failure-2

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HEART FAILURE

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HEART FAILURE DEFINITION

It is a clinical syndrome that reflects the inability of the heart to meet the metabolic requirement of the body.

This definition includes both types of HF in children:

  • High cardiac output failure (Over-circulation failure).
  • Pump failure.

HF FUNCTIONAL CLASSIFICATION

Excessive Work Load

  • Volume Overload: VSD, A.R.
  • Pressure Overload: A.S, P.S, M.S.

Disorders of Myocardium

  • Systolic dysfunction (reduced ventricular contractility): impaired ejection of blood from ventricle.
  • Diastolic dysfunction (impaired ventricular filling and noncompliance): resulting in high ventricular filling pressures.

Disorders of Rhythm

  • Tachyarrhythmia / Bradyarrhythmia.

CAUSES OF HF IN INFANTS AND CHILDREN

Ventricular Dysfunction

Systolic Dysfunction / Diastolic Dysfunction

  • Structurally normal heart:
    • Cardiomyopathy / Myocarditis.
    • Myocardial ischemia / MI (e.g., ALCAPA, KD, familial hypercholesterolemia).
    • Arrhythmogenic (e.g., SVT, VT, CHB).
    • Drug/toxin exposure (e.g., Adriamycin for ALL).
  • Noncardiac causes: Sepsis, Renal failure, Respiratory disorders (e.g., bronchopulmonary dysplasia, cystic fibrosis, interstitial lung disease, SLE).
  • Complex congenital heart disease: e.g., children born with complex CHD who undergo surgical repair or palliation in early childhood.

Preserved Ventricular Contractility Z

Volume Overload

  • Left-to-right shunting: VSD, ASD, PDA.
  • Valvular insufficiency: AR, MR, PR.
  • Noncardiac causes: AV Malformation, fluid overload (KF).

Pressure Overload

  • Left-sided: AS, COA, Systemic hypertension.
  • Right-sided: PS, Pulmonary hypertension.

Note: Depending on severity and chronicity, pressure overload may result in either systolic or diastolic dysfunction.

Common Causes Detailed (By Etiology)

CHD (Congenital Heart Disease)

  • Pressure overload:
    • Aortic stenosis
    • Coarctation of aorta
    • Interrupted aortic arch
    • Pulmonary stenosis
    • Pulmonary vein stenosis
  • Volume overload:
    • Left-to-right shunts
    • Aortic regurgitation
    • Mitral regurgitation
    • Ebstein anomaly with severe tricuspid regurgitation
  • Both volume and pressure overload:
    • Hypoplastic left heart
    • CHD with single ventricle physiology
  • Ischemia:
    • Coronary anomaly
    • Perioperative injury/ischemia

Cardiomyopathy

  • Primary Cardiomyopathies:
    • Dilated cardiomyopathy
    • Hypertrophic cardiomyopathy
    • Restrictive cardiomyopathy
    • Arrhythmogenic RV dysplasia
    • LV noncompaction cardiomyopathy
  • Secondary Cardiomyopathies:
    • Inflammatory: Myocarditis
    • Infections: HIV, rheumatic fever, Chagas disease
    • Drug-or-toxin related: Anthracycline, iron overload
    • Arrhythmias: Tachyarrhythmia, congenital heart block
    • Ion channel disorder
    • Neuromuscular disorders
    • Nutritional deficiency: Carnitine deficiency
    • Sepsis
    • Stress
    • Endocrine: Thyrotoxicosis
    • Severe anemia
    • Systemic arteriovenous fistula
    • Genetic syndromes (Noonan) even without CHD
    • Infant of diabetic mother
    • Storage disorders: Pompe disease
    • Mitochondrial myopathies
    • Chronic renal failure

SYMPTOMS OF HF

Symptoms in Infants

  • Tachypnea.
  • Diaphoresis during feeds.
  • Easy fatigability.
  • Irritability.
  • Decreased volume of feeds, and poor weight gain.
  • Undernutrition may result in delayed motor milestones.
  • Growth failure.
  • Hepatomegaly.
  • Respiratory distress.

Symptoms in Toddlers / Young Children

  • GIT symptoms (abdominal pain, nausea, vomiting, and poor appetite).
  • Poor weight gain.
  • Easy fatigability.
  • Recurrent or chronic cough with wheezing.
  • Respiratory distress.
  • Decreased activity.
  • Hepatomegaly.

Note: These symptoms are often mistaken for gastroenteritis, reflux, asthma, and behavioral issues.

Symptoms in School Age / Older Children / Adolescents

  • Exercise intolerance.
  • Fatigue.
  • Anorexia, poor appetite, abdominal pain, nausea/vomiting.
  • Wheezing, dyspnea.
  • Edema.
  • Palpitations, chest pain.
  • Syncope.
  • Orthopnea.
  • Hepatomegaly.

Note: A high index of suspicion is needed to distinguish HF from other far more common pediatric illnesses (e.g., pneumonia, sinusitis, otitis media, viral illnesses, gastroenteritis, and asthma [50%]).

SIGNS OF HEART FAILURE

Signs of Impaired Myocardial Function

  • Cardiac enlargement.
  • Tachycardia.
  • Gallop rhythm.
  • Cold extremities, pallor, weak pulses.
  • Low BP, skin mottling, capillary refill.
  • Sweating.
  • Growth failure.
  • Pulses paradoxis-alternans.

Signs of Pulmonary Congestion

  • Tachypnea (most common).
  • Hyperpnea.
  • Wheezing / Rales (Older children).
  • Cyanosis.
  • Dyspnea and signs of respiratory distress.
  • Cough.

Signs of Systemic Venous Congestion

  • Hepatomegaly (the most common finding).
  • Neck vein distension.
  • Facial edema.
  • Peripheral edema.
  • Ascites and splenomegaly (may be present in severe right HF).
  • Jugular venous distension (not generally observed in infants and young children).

Other Findings for Underlying Etiology

  • High BP limited to arms and weak pulses in legs are suggestive of COA.
  • A systolic murmur may be seen in HOCM, AS, VSD, MR.
  • Precordial examination may reveal a “thrill” in patients with VSD.
  • A longstanding cardiomyopathy may have a “heave” with a laterally displaced point of maximal impulse.

ADAPTIVE MECHANISMS

  • Ventricular Dilatation/Hypertrophy.
  • Adrenergic Mechanisms.
  • Erythrocyte Oxygen Transport.
  • Regional Circulations (Pulmonary, Renal, GIT, Skin, Liver).
  • Natriuretic Peptide (ANP/BNP): Vasodilation, natriuresis, and diuresis.
  • Vasopressin Hormone.

img-1.jpeg Pathophysiology of chronic heart failure

CLASSIFICATION OF SEVERITY

Modified Ross Classification (Children < 6 years)

  • Class I: Asymptomatic.
  • Class II: Mild tachypnea or diaphoresis with feeding in infants; dyspnea on exertion in older children.
  • Class III: Marked tachypnea or diaphoresis with feeding in infants. Prolonged feeding times with growth failure; Marked dyspnea on exertion in older children.
  • Class IV: Symptoms such as tachypnea, retractions, grunting, or diaphoresis at rest.

NYHA Classification (Children > 6 years)

  • Class I: Asymptomatic.
  • Class II: Slight or moderate limitations of physical activity.
  • Class III: Marked limitation of physical activity.
  • Class IV: Symptoms at rest.

INVESTIGATIONS

Chest Radiography

  • Cardiomegaly: Left-to-right shunting defects, Dilated cardiomyopathy, Myocarditis, and Pericardial effusion.
  • Pulmonary congestion/pulmonary edema.
  • Pleural effusion.
  • Pericardial effusion.

Chest radiograph of a 13-year-old boy with dilated cardiomyopathy img-2.jpeg

Chest radiograph in a 6-year-old patient with a large secundum atrial septal defect img-3.jpeg Demonstrates moderate cardiomegaly with increased convexity of the right side of the heart (arrowheads). There is a large pulmonary outflow tract (arrow) and increased pulmonary vascularity. Findings consistent with large left-to-right shunt.

Electrocardiogram (ECG)

  • Sinus tachycardia.
  • ST segment and T wave abnormalities: Cardiomyopathy and myocarditis.
  • Increased QRS voltage: Hypertrophic or dilated cardiomyopathy (VH).
  • Decreased QRS voltage: Pericardial effusion and myocarditis.
  • Bi-atrial enlargement: Restrictive cardiomyopathy.
  • Deep Q wave in inferior and lateral leads (I, aVL, and V5-V6) with ST segment and T wave changes: Suggestive of myocardial infarct (ALCAPA).
  • Varying degrees of heart block: Rheumatic fever or neonatal lupus.
  • Arrhythmias (Atrial, junctional, or ventricular tachycardia or PAC or PVC): As an underlying cause of ventricular dysfunction or a complication.

Left ventricular hypertrophy with strain pattern img-4.jpeg ST-T wave abnormalities secondary to LVH (“strain”) in anterolateral leads (I, aVL, V4-V6). Typical abnormalities include a horizontal or downsloping ST segment and T wave inversions. In some cases, there is concavity to the ST segment, which has a final downward turn that blends into an inverted T wave.

Normal ECG img-5.jpeg Normal sinus rhythm at 75 beats/minute, PR 0.14s, QRS 0.10s, QRS axis ~75°.

Electrocardiogram of 13-year-old boy with dilated cardiomyopathy img-6.jpeg Demonstrating increased QRS voltage with ST segment and T wave abnormalities.

Electrocardiogram in a 10-week-old infant with ALCAPA img-7.jpeg Demonstrating deep Q waves in anterior and lateral leads (I, aVL, and V5-V6) with ST segment and T wave abnormalities.

First do x-ray then echocardiography espicially for pneumonia case suspected Z

Echocardiography

  • Underlying structural congenital heart disease (CHD).
  • Atrial and ventricular sizes (chamber size, wall thickness).
  • LV and RV global and regional systolic function.
  • LV diastolic function.
  • Pericardial effusion.
  • Estimation of RV and pulmonary artery pressures.
  • Atrial or ventricular thrombi.
  • Presence and amount of valvular stenosis and regurgitation.

Other Imaging and Testing

  • Cardiac MRI: Accurate and detailed information regarding cardiac anatomy, ventricular function, myocardial inflammation, and infiltration by fat and fibrous tissues.
  • Cardiac Catheterization: Coronary anatomy, endomyocardial biopsy, evaluate pulmonary vascular resistance (PVR) and vasodilator responsiveness.
  • Holter Monitoring: If symptoms suggest arrhythmia.
  • Exercise Testing: For children with cardiomyopathy to determine functional class and risk stratification.

Laboratory Observations

  • Complete Blood Count and Iron Studies: Anemia may contribute to HF.
  • Serum Electrolytes: Hyponatremia. Baseline electrolytes needed prior to initiating therapy with diuretics or ACE inhibitors.
  • Urea and Creatinine: Kidney function impairment may be a contributing factor.
  • Liver Function Tests: May be elevated due to hepatic congestion with right-sided HF.
  • Blood Gases and pH: Acidosis.
  • Glucose: Hypoglycemia.
  • ESR: High ESR is a poor prognosis sign.
  • Urinary Findings: Albuminuria.

Cardiac Biomarkers

  • B-type Natriuretic Peptide (BNP) / NT-proBNP:
    • Used to assess severity of HF and monitor response to therapy.
    • Discriminates between cardiac and noncardiac causes (e.g., lung disease).
    • Correlates with degree of shunting in ASD/VSD/PDA.
    • In ventricular dysfunction, correlates negatively with ejection fraction.
    • Correlates with functional class of HF and outcome.
  • Troponin:
    • Elevated in myocarditis and myocardial ischemia.
    • In children with LV dysfunction, elevated troponin may suggest acute myocarditis rather than dilated cardiomyopathy.

HF DIFFERENTIAL DIAGNOSIS

  • Respiratory Distress (Pulmonary Disorders):
    • Neonates: TTN, RDS, meconium aspiration, pneumonia.
    • Older infants/children: Pneumonia, bronchiolitis, asthma.
  • Shock: Overwhelming sepsis, hypovolemia, IEM.
  • Poor Weight Gain: Protein-milk allergy, cystic fibrosis, celiac disease.
  • Peripheral Edema: Kidney failure, venous thrombosis.

MANAGEMENT

General Measures

  • Reversible Contributors: Anemia, Hypertension, Renal failure, Acidosis, Malnutrition, Respiratory disorders (e.g., asthma, obstructive sleep apnea, interstitial lung disease), Thyroid disorders.
  • Nutritional Support:
    • Children with HF often have increased caloric needs due to an increased metabolic demand, often tire with feeding and their intake may be limited.
    • Intermittent or continuous nasogastric or gastrostomy tube feeds may be required.
  • Exercise and Physical Activity:
    • Promoting safe physical activity is important.
    • Recommendations tailored based on diagnosis and exercise capacity.

Medical Therapy

Goals & Strategy

  • Goals: Relieve symptoms, slow progression, improve survival and quality of life.
  • Unstable Patients: Presenting with shock require prompt treatment to restore perfusion.
  • Structural Heart Disease (Preserved Function): Causing volume overload (e.g., VSD, ASD) or pressure overload (e.g., PS, AS), management is surgical or catheter-based interventions. Medical therapy is needed for stabilization or symptom relief while awaiting a more definitive intervention.

Therapy by Stage y

HF StageDefinitionExamplesTherapy Approach
Stage A (At Risk)Patients at risk for HF who have normal cardiac function and chamber size.• Exposure to cardiac toxic drugs.
• Family history of heritable CM
• Repaired or palliated CHD with normal ventricular function (e.g., ccTGA)
• Duchenne muscular dystrophy.		HF-specific therapies generally not necessary. Predisposing conditions should be treated.
Stage B (Presymptomatic)Evidence of systemic ventricular dysfunction on echo or other imaging who are asymptomatic (No past or present HF symptoms).• History of anthracycline exposure with reduced LVEF
• Repaired or palliated CHD with reduced ventricular function
• Isolated LV noncompaction		ACE inhibitors (first choice). ARBs, beta blockers can be used.
Stage C (Symptomatic)Evidence of systemic ventricular dysfunction on echo or other imaging who are symptomatic (including past or present HF symptoms).• Symptomatic cardiomyopathy
• Repaired or palliated CHD with symptomatic ventricular dysfunction		See detailed regimen below.
Stage D (Advanced)Patients with end-stage HF requiring continuous infusion of inotropic agents, mechanical circulatory support, cardiac transplantation, or hospice care.• End-stage state of any of the above examples.
• There is marked impairment and symptoms at rest despite maximal medical therapy		Interventions: IV inotropes (milrinone), diuretics, Positive pressure ventilation, CRT, Heart transplantation.

Stage C Regimen (Symptomatic Systemic Ventricular Dysfunction)

  1. Initial: ACE inhibitor or ARB plus Spironolactone.
  2. Fluid Overload: Oral Furosemide / Metolazone as needed.
  3. Persistent Dysfunction: Add Beta Blocker (e.g., Carvedilol / Metoprolol) after stabilization.
  4. Symptom Relief: Digoxin can be added if needed.
  5. Reassessment: Consider Angiotensin Receptor-Neprilysin Inhibitor (ARNI; Sacubitril-Valsartan) if tolerating full regimen.
  6. Severe Cases: If severe limitation, growth failure, intractable arrhythmias, or restrictive CM, consider early referral for Heart Transplant.

Other Measures

  • Oxygen.
  • Prostaglandin.
  • Morphine.
  • Noninvasive Ventilation: High-flow nasal cannula (HFNC), CPAP.
  • Position.
  • Bed rest.

Nonpharmacologic Interventions for Advanced HF

  • Cardiac Resynchronization Therapy (CRT):
    • For advanced HF unresponsive to medical therapy, esp. reduced EF (<35%) and LBBB pattern.
    • LBBB/conduction delay worsens HF via dyssynchrony; CRT uses biventricular pacing to minimize this.
  • Extracorporeal Membrane Oxygenation (ECMO):
    • Total heart-lung bypass device.
    • Used for Postcardiotomy shock or Acute Myocarditis.

COMPLICATIONS

  • Thromboembolism: Risk of intracardiac thrombi, pulmonary embolus, cerebral embolic strokes, death (due to systemic ventricular dysfunction).
  • Arrhythmias: Sustained atrial/ventricular tachyarrhythmias can rapidly impair hemodynamics.
  • Sudden Cardiac Death (SCD):
    • ICD (Implantable Cardioverter Defibrillator) indicated for patients at risk (ventricular arrhythmia, history of unexplained syncope, or recurrent sustained ventricular dysrhythmias).

PROGNOSIS & COUNSELING

  • Mortality: Ranges from 6 - 15% among hospitalized children.
  • Risk Factors for Mortality: Comorbid renal/respiratory failure, symptoms at diagnosis (DCM), neuromuscular disease, lower LV shortening fraction.
    • Note: Myocarditis is associated with better survival compared to some other causes.
  • Medical Management Success: Improves outcomes in 60% of NYHA/Ross Class II/III patients.
  • Counseling Factors: Severity of underlying condition, control of arrhythmias, new therapy modalities, cardiac transplantation availability.
  • Transplantation Stats:
    • Mortality highest in first year post-transplant.
    • Median survival ranges from 13 to 22 years.

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