Congenital heart disease

Background

Congenital Heart Disease Types

Clinical Features

Clinical Presentation Causative Conditions in Neonates Causative Conditions in Infants and Children
CyanosisTransposition of the great arteries, TOF, tricuspid atresia, truncus arteriosus, total anomalous pulmonary venous returnTOF, Eisenmenger complex
Cardiovascular shockCritical aortic stenosis, coarctation of the aorta, HLHSCoarctation of the aorta (infants)
Congestive heart failureRare: PDA, HLHSPDA, VSD, ASD, atrioventricular canal
MurmurPDA, valvular defects (AS, PS)VSD, ASD, PDA, outflow obstructions, valvular defects (AS, PS)
SyncopeAS, PS, Eisenmenger complex
HypertensionCoarctation of the aorta
ArrhythmiasASD, Ebstein anomaly, postsurgical complication after repair of congenital heart defect

Differential Diagnosis

Sick Neonate

THE MISFITS [2]

Evaluation

Cyanotic

Cardiac Lesion Chest Radiograph ECG
Tetralogy of FallotBoot-shaped heart, normal-sized heart, decreased pulmonary vascular markingsRight axis deviation, right ventricular hypertrophy
Transposition of the great arteriesEgg-shaped heart, narrow mediastinum, increased pulmonary vascular markingRight axis deviation, right ventricular hypertrophy
Total anomalous pulmonary venous returnSnowman sign, significant cardiomegaly, increased pulmonary vascular markingsRight axis deviation, right ventricular hypertrophy, right atrial enlargement
Tricuspid atresiaHeart of normal to slightly increased size, decreased pulmonary vascular markingsSuperior QRS axis with right atrial hypertrophy, left atrial hypertrophy, left ventricular hypertrophy
Truncus arteriosusCardiomegaly, increased pulmonary vascular markingsBiventricular 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 syndromeCardiomegalyRight atrial enlargement, RVH, peaked P waves
Aortic stenosisCardiomegalyLVH in severe cases

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

Atrial septal defect Cardiomegaly with increased vascular markings Right axis deviation, RVH, RBBB
VSDCardiomegaly with increased vascular markingsLAH, LVH, (RVH with larger VSDs)
PDACardiomegaly with increased vascular markingsLVH, RVH with larger PDAs
Endocardial cushion defectCardiomegaly with increased vascular markingsSuperior QRS axis with RVH, RBBB, LVH, prolonged PR interval
Anomalous origin of the left coronary arteryCardiomegalyAbnormally deep and wide Q waves with precordial ST segment changes

Management

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

CHF

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

References

  1. Knipe K et al. Cyanotic congenital heart diseases. Radiopaedia. http://radiopaedia.org/articles/cyanotic-congenital-heart-disease
  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.
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