Congenital heart disease

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Congenital Heart Disease Types

Clinical Features

Clinical Presentation Causative Conditions in Neonates Causative Conditions in Infants and Children
Cyanosis Transposition of the great arteries, TOF, tricuspid atresia, truncus arteriosus, total anomalous pulmonary venous return TOF, Eisenmenger complex
Cardiovascular shock Critical aortic stenosis, coarctation of the aorta, HLHS Coarctation of the aorta (infants)
Congestive heart failure Rare: PDA, HLHS PDA, VSD, ASD, atrioventricular canal
Murmur PDA, valvular defects (AS, PS) VSD, ASD, PDA, outflow obstructions, valvular defects (AS, PS)
Syncope AS, PS, Eisenmenger complex
Hypertension Coarctation of the aorta
Arrhythmias ASD, Ebstein anomaly, postsurgical complication after repair of congenital heart defect

Differential Diagnosis

Sick Neonate



Algorithm for the Evaluation and Management of Suspected Congenital Heart Disease in Neonates


Cardiac Lesion Chest Radiograph ECG
Tetralogy of Fallot Boot-shaped heart, normal-sized heart, decreased pulmonary vascular markings Right axis deviation, right ventricular hypertrophy
Transposition of the great arteries Egg-shaped heart, narrow mediastinum, increased pulmonary vascular marking Right axis deviation, right ventricular hypertrophy
Total anomalous pulmonary venous return Snowman sign, significant cardiomegaly, increased pulmonary vascular markings Right axis deviation, right ventricular hypertrophy, right atrial enlargement
Tricuspid atresia Heart of normal to slightly increased size, decreased pulmonary vascular markings Superior QRS axis with right atrial hypertrophy, left atrial hypertrophy, left ventricular hypertrophy
Truncus arteriosus Cardiomegaly, increased pulmonary vascular markings Biventricular hypertrophy
  • Can use the "5 T's' mnemonic:[3]
    • 1 vessel: Truncus arteriosus
    • 2 vessels switched: Transposition of the great vessels
    • 3=TRIcuspid: Tricuspid atresia
    • 4 defects: Tetralogy of Fallot
    • 5 letters: Total anomalous pulmonary vascular return (TAPVR)

Acyanotic (duct-dependent)

Coarctation of the aorta Cardiomegaly with pulmonary edema (neonate) RVH, right bundle-branch block (neonate)
Rib notching and collateral vascularity (child) LVH (child)
Hypoplastic left heart syndrome Cardiomegaly Right atrial enlargement, RVH, peaked P waves
Aortic stenosis Cardiomegaly LVH in severe cases

Acyanotic non-duct dependent (i.e. CHF)

Atrial septal defect Cardiomegaly with increased vascular markings Right axis deviation, RVH, RBBB
VSD Cardiomegaly with increased vascular markings LAH, LVH, (RVH with larger VSDs)
PDA Cardiomegaly with increased vascular markings LVH, RVH with larger PDAs
Endocardial cushion defect Cardiomegaly with increased vascular markings Superior QRS axis with RVH, RBBB, LVH, prolonged PR interval
Anomalous origin of the left coronary artery Cardiomegaly Abnormally deep and wide Q waves with precordial ST segment changes


Shock (duct-dependent lesion)

Tet Spell

  • Knee chest position
    • Increased venous return to heart, increased SVR (decreased R>L shunting)
  • O2
  • Morphine or NS to increase preload
  • Sodium bicarbonate 2mEq/kg IV bolus (promotes vasodilation)
  • Propranolol 0.2mg/kg IV (relieves infundibular spasm)
  • Phenylephrine 2-10mcg/kg/min to increased SVR


Thrombolysis for Surgical Shunt Obstruction

  • Blalock-Taussig shunt should maintain flow murmur
  • Loss of flow murmur alongside profound hypoxia relative to baseline saturations should prompt consideration for shunt obstruction
  • Definitive treatment is surgical, but systemic recombinant tPA may be considered as salvage intervention when other options are not readily available[4]
    • Heparin bolus 50-100 u/kg
    • Notify cardiology, CT surgeon, ECMO
    • 0.01 mg/kg bolus r-tPA, then 0.03 - 0.06 mg/kg/hr


See Also


  1. Knipe K et al. Cyanotic congenital heart diseases. Radiopaedia.
  2. Brousseau T, Sharieff GQ. Newborn emergencies: the first 30 days of life. Pediatr Clin North Am. 2006 Feb;53(1):69-84, vi.
  3. Waldman JD, Wernly JA. Cyanotic congenital heart disease with decreased pulmonary blood flow in children. Pediatr Clin North Am. 1999;46(2):385-404. doi:10.1016/s0031-3955(05)70125-5
  4. Diaz F et al. Systemic thrombolysis with recombinant tissue plasminogen activator for acute life-threatening Blalock-Taussig shunt obstruction. Indian J Crit Care Med. 2016 Jul; 20(7): 425–427.