Congenital heart disease

Background

• Cyanotic
  • Tetralogy
  • Tricuspid anomalies
  • Truncus arteriosus
  • Total anomalous pulmonary venous return
  • Transposition
• Acyanotic
  • VSD
  • ASD
  • PDA
  • AV canal
  • Pulmonary/aortic stenosis

Diagnosis

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 AS, 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

Work-Up

Cyanotic

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

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 (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

Treatment (by presentation)

1. Shock (duct-dependent lesion)
   1. PGE1
      1. 0.1mcg/kg/min IV/IO
   2. NS 10cc/kg
   3. Dobutamine
2. Tet spells
   1. Knee chest position
      1. Incr venous return to heart, incr SVR (decr R>L shunting)
   2. O2
   3. Morphine or NS to increase preload
   4. Nabicarb 2mEq/kg IV bolus (promotes vasodilation)
   5. Propranolol 0.2mg/kg IV (relieves infundibular spasm)
   6. Phenylephrine 2-10mcg/kg/min to incr SVR
3. CHF
   1. Diuresis, NTG, inotrops

PGE1 Side Effects

1. Apnea (intubate)
2. Hypotension
3. Bradycardia
4. Hyperexia
5. Flushing