Congenital heart disease

Background

Congenital Heart Disease Types

Cyanotic
Acyanotic
- AV canal defect
- Atrial septal defect (ASD)
- Ventricular septal defect (VSD)
- Cor triatriatum
- Patent ductus arteriosus (PDA)
- Pulmonary/aortic stenosis
- Coarctation of the aorta
Differentiation by pulmonary vascularity on CXR^[1]
- Increased pulmonary vascularity
  - Large ASD
  - Large VSD
  - Total anomalous pulmonary venous return
  - Transposition of the great arteries
  - Truncus arteriosus
- Decreased pulmonary vascularity
  - Tetralogy of fallot
  - Rare heart diseases with pulmonic stenosis

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

THE MISFITS ^[2]

Trauma
Heart
- Congenital heart disease
- Hypovolemia
Endocrine
Metabolic
- Sodium
- Calcium
- Glucose
Inborn errors of metabolism
Seizure
Formula / feeding problems
Intestinal Disasters
Toxin
Sepsis

Evaluation

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

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

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

Management

Shock (duct-dependent lesion)

PGE1 0.1mcg/kg/min IV/IO
- Side Effects:
  - Apnea (intubate)
    - Hypotension
    - Bradycardia
    - Flushing
NS 10cc/kg
Dobutamine

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

O2 (give only if SpO2 <95%)
Furosemide 1-2mg/kg IV
Dopamine 5-10mcg/kg/min
Dobutamine 5-10mcg/kg/min

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

Disposition

Medication Dosing

Prostaglandin E1 0.1mcg/kg/min IV/IO IV/IO Propranolol 0.2mg/kg IV IV Phenylephrine 2-10mcg/kg/min IV infusion IV

References

↑ Knipe K et al. Cyanotic congenital heart diseases. Radiopaedia. http://radiopaedia.org/articles/cyanotic-congenital-heart-disease
↑ Brousseau T, Sharieff GQ. Newborn emergencies: the first 30 days of life. Pediatr Clin North Am. 2006 Feb;53(1):69-84, vi.
↑ 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
↑ 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.

Authors:

[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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