🫀 Chapter 491: Heart Failure
Nelson's Textbook of Pediatrics 22e | HFpEF · HFrEF · Compensatory mechanisms · Diuretics · ACE inhibitors · Beta-blockers · Aldosterone antagonists · Milrinone · VAD · Transplantation · Transition
📚 Reference: www.paeds.online — Paediatric Resource Hub
📌 Heart Failure – Definition (ISHLT)Clinical and pathological syndrome from ventricular dysfunction, volume/pressure overload. Signs: poor feeding, tachypnea, fatigue, hepatomegaly, pulmonary edema. Compensatory mechanisms: sympathetic activation, RAAS activation, Frank-Starling, hypertrophy.
📌 Classification – HFrEF vs HFpEFHFrEF (reduced EF, ≤40%): dilated cardiomyopathy, myocarditis. HFpEF (preserved EF, ≥50%): hypertrophic cardiomyopathy, restrictive cardiomyopathy, diastolic dysfunction.
📌 Etiology – Age-dependentNeonates: left-sided obstructive lesions (coarctation, HLHS), myocarditis, arrhythmias. Infants: VSD, PDA, anomalous coronary artery. Children/adolescents: myocarditis, DCM, HCM, rheumatic, anthracycline.
📌 Clinical Presentation – InfantsPoor feeding, tachypnea, diaphoresis with feeds, failure to thrive, irritability, hepatomegaly, gallop rhythm (S3). Tachycardia out of proportion.
📌 Clinical Presentation – Older ChildrenDyspnea on exertion, orthopnea, fatigue, exercise intolerance, abdominal pain (hepatic congestion), peripheral edema, JVD, pulmonary rales.
📌 Diagnosis – BiomarkersBNP/NT-proBNP elevated (rule out non-cardiac dyspnea). Troponin (myocarditis). Echocardiogram: EF, LV size, wall motion, valvular function.
📌 Medical Therapy – GDMT (Guideline-Directed Medical Therapy)Diuretics (furosemide, thiazides, spironolactone). ACE inhibitors (captopril, enalapril, lisinopril) – afterload reduction, reverse remodeling. Beta-blockers (carvedilol, metoprolol) – improve survival. Digoxin (adjunct).
📌 Acute Decompensated HF – InotropesMilrinone (preferred – inotrope + vasodilator). Dobutamine, dopamine. ECMO for refractory cardiogenic shock.
📌 Advanced TherapiesVentricular assist device (VAD) – bridge to transplant. Cardiac transplantation – 5-year survival ~80% in children. Indications: refractory HF, life-threatening arrhythmias, failure to thrive.
📌 Transition & PrognosisDCM: 30-50% recovery. HCM: risk of SCD, ICD. RCM: poor, early transplant. Transition to adult HF/cardiologist. Pregnancy high-risk if EF <40%.
🔍 Step 1: Recognize age-specific symptoms
Infants: poor feeding, tachypnea, diaphoresis during feeds, poor weight gain, irritability. Children: fatigue, exercise intolerance, dyspnea, orthopnea, abdominal pain (hepatic congestion).
📈 Step 2: Physical examination
Tachycardia, tachypnea, gallop rhythm (S3), hepatomegaly (most consistent), pulmonary rales/wheezing (left HF), JVD, peripheral edema (older children).
🩺 Step 3: Diagnostic tests
BNP/NT-proBNP elevated (rule out non-cardiac). Echocardiogram: LVEF, chamber size, valvular function, pericardial effusion. Chest X-ray: cardiomegaly, pulmonary congestion.
🔄 Step 4: Determine etiology (HFrEF vs HFpEF)
HFrEF: DCM, myocarditis, anthracycline. HFpEF: HCM, RCM, hypertension. Echocardiogram and cardiac MRI for tissue characterization.
🚨 Step 5: Assess severity (NYHA/Ross classification)
Ross I: asymptomatic. Ross II: mild tachypnea. Ross III: marked tachypnea, feeding difficulty. Ross IV: symptoms at rest (pre-terminal).
1️⃣ Acute decompensated HF – Inpatient
▪ ICU admission. Diuretics (furosemide IV) for pulmonary edema.
▪ Inotropes: milrinone (preferred), dobutamine, dopamine.
▪ Vasodilators: nitroprusside (if hypertension).
▪ ECMO for refractory cardiogenic shock.
2️⃣ Chronic HFrEF – GDMT (Guideline-Directed Medical Therapy)
▪ Diuretics (furosemide, spironolactone) – volume control.
▪ ACE inhibitors (captopril, enalapril, lisinopril) – afterload reduction, reverse remodeling.
▪ Beta-blockers (carvedilol, metoprolol) – start after stabilization, improve survival.
▪ Aldosterone antagonist (spironolactone) – advanced HF.
▪ Digoxin (adjunct, symptom control).
3️⃣ HFpEF (Diastolic dysfunction)
▪ Diuretics (judiciously). Avoid digoxin, beta-blockers may worsen? (use cautiously).
▪ Treat underlying cause (HCM: beta-blockers, RCM: diuretics).
4️⃣ Advanced therapies
▪ Ventricular assist device (VAD) – bridge to transplant.
▪ Cardiac transplantation – for refractory HF.
▪ ICD for high-risk (HCM, DCM with VT, LMNA mutations).
5️⃣ Manage complications
▪ Anticoagulation if LVEF <25% or mural thrombus.
▪ Treat arrhythmias (AF, VT).
▪ Nutrition support (caloric supplementation, NG feeds).
6️⃣ Family screening & genetic counseling
▪ First-degree relatives of DCM/HCM proband: ECG, echocardiogram, genetic testing if pathogenic variant identified.
7️⃣ Transition & pregnancy
▪ Lifelong HF management. Pregnancy high-risk if EF <40% (contraindicated).
▪ Transition to adult HF/cardiologist with cardiomyopathy expertise.
❓ Reflex prompt 1: A 6-month-old with poor feeding, tachypnea, hepatomegaly. Echo shows LVEF 25%. Next step?
✅ Answer: Admit to PICU, start diuretics, ACE inhibitor. Consider inotropes if decompensated. Evaluate for myocarditis/DCM.
❓ Reflex prompt 2: A 12-year-old with DCM, LVEF 20%, on optimal GDMT. Still NYHA III. Next option?
✅ Answer: VAD as bridge to transplant. Evaluate for heart transplantation.
❓ Reflex prompt 3: A 14-year-old with HCM and syncope during exercise. Next step?
✅ Answer: ICD for secondary prevention. Beta-blocker therapy. No competitive sports.
❓ Reflex prompt 4: A 9-year-old with DCM, LVEF 25%, and NSVT on Holter. Medication?
✅ Answer: Beta-blocker (carvedilol) – antiarrhythmic + reverse remodeling. ICD if sustained VT.
❓ Reflex prompt 5: A 15-year-old with DCM, LVEF 30%, on carvedilol, lisinopril, furosemide. Develops acute worsening, BP 80/50. Next step?
✅ Answer: IV milrinone, reduce beta-blocker temporarily. Diuretics for congestion.
❓ Reflex prompt 6: A 5-year-old with DCM, LVEF 35%, mother has DCM. Most likely inheritance?
✅ Answer: Autosomal dominant (familial DCM). Screen first-degree relatives.
📌 Heart Failure – Core Concepts (Nelson 22e, Chapter 491)

1. Definition: Clinical syndrome of ventricular dysfunction, volume/pressure overload. Compensatory mechanisms: sympathetic activation, RAAS activation, Frank-Starling, hypertrophy (initially beneficial, then maladaptive).
2. Classification:
- HFrEF (EF ≤40%): dilated cardiomyopathy, myocarditis, anthracycline.
- HFpEF (EF ≥50%): hypertrophic cardiomyopathy, restrictive cardiomyopathy, hypertension, aortic stenosis.
3. Etiology – Age-dependent:
- Neonates: left-sided obstructive lesions (coarctation, HLHS), myocarditis, arrhythmias.
- Infants: VSD, PDA, anomalous coronary artery, myocarditis.
- Children/adolescents: myocarditis, DCM, HCM, rheumatic heart disease, anthracycline cardiotoxicity.
4. Clinical presentation: Age-specific (infants: poor feeding, tachypnea, diaphoresis; older: dyspnea, fatigue, edema). Physical exam: tachycardia, gallop (S3), hepatomegaly, rales, JVD.
5. Diagnosis: BNP/NT-proBNP elevated. Echocardiogram (EF, LV size, valvular function, pericardial effusion). Cardiac MRI (scar, edema, fibrosis).
6. Medical therapy (GDMT):
- Diuretics (furosemide, spironolactone).
- ACE inhibitors (enalapril, lisinopril) – afterload reduction, reverse remodeling.
- Beta-blockers (carvedilol, metoprolol) – improve survival, start after stabilization.
- Digoxin (adjunct).
7. Acute decompensated HF: ICU, inotropes (milrinone preferred), diuretics, ECMO for refractory shock.
8. Advanced therapies: VAD (bridge to transplant), cardiac transplantation (5-year survival ~80%).
9. Prognosis: DCM: 30-50% recovery. HCM: risk of SCD (ICD). RCM: poor, early transplant.
10. Transition & pregnancy: Lifelong follow-up. Pregnancy high-risk (contraindicated if LVEF <40%).
💡 Clinical pearls:
➤ In infants, hepatomegaly is the most consistent sign of HF. Pulmonary rales often absent.
➤ Beta-blockers should be started after stabilization (not in acute decompensated HF).
➤ BNP is useful to differentiate cardiac from pulmonary causes of dyspnea.
➤ LMNA mutations: high risk of sudden death, ICD indicated even with preserved EF.
➤ Anthracycline cardiotoxicity: lifelong surveillance required.