Congenital Heart Disease, Acyanotic, Emergency Medicine

Basics

Description

Abnormality in the cardiocirculatory system that is present at birth but does not cause mixing of deoxygenated and oxygenated blood: �

L→R shunting lesions:
- Ventricular septal defect (VSD)
- Atrial septal defect (ASD)
- Patent ductus arteriosus (PDA)
- Endocardial cushion defects (AV canal)
Ventricular outflow obstructions:
- Coarctation of aorta (LV)
- Aortic stenosis (LV)
- Pulmonic stenosis (RV)
- Hypoplastic left-heart syndrome (HLHS)
Ductal dependent: Symptoms as DA closes:
- Coarctation of aorta
- Critical aortic stenosis
- Critical pulmonic stenosis
- HLHS

Etiology

For most forms, cause is unknown: �

Genetic: Down (AV canal), Turner (coarct)
Environmental: Congenital rubella (PDA, AS)

Diagnosis

Signs and Symptoms

History

Many asymptomatic
Lethargy, poor feeding, and failure to thrive
Dyspnea on exertion
Recurrent respiratory infections

Physical Exam

VSD and AV canal:
- Dusky color, hepatomegaly
- Holosystolic and diastolic murmurs + thrill
- Hyperdynamic precordium, displaced PMI
ASD:
- Fixed, split S2
- Systolic ejection and diastolic murmurs
PDA:
- "Machine-like"� murmur and bounding pulses
Coarctation:
- Differential cyanosis (pink only upper 1/2)
- BP upper extremities > BP lower extremities
- ↓ or absent lower-extremity pulses
AS:
- Harsh systolic murmur, thrill, aortic click
PS:
- Systolic ejection murmur, thrill, pulmonic click
- Widely split S2
- Jugular venous A-waves
HLHS:
- Dusky, listless, tachypneic, ↓ pulses
- Single heart sound, systolic ejection murmur

Essential Workup

Oxygen saturation (pre- and postductal)
ABG, CBC, basic chemistries, and glucose
Sepsis evaluation
CXR to assess pulmonary blood flow
EKG (axis, hypertrophy, conduction delays)
4-extremity BPs
Cardiology consult with ECG

Diagnosis Tests & Interpretation

Imaging

CXR:
- L→R shunting lesions all show cardiomegaly (specific chambers) and ↑ pulmonary markings
  - ASD (RA, RV), VSD (RV, LA), PDA (LA, LV)
  - AV canal (globular; all chambers enlarged)
Obstructive lesions: Normal to cardiomegaly

Diagnostic Procedures/Surgery
EKG: �

ASD: Right axis deviation:
- RVH or right bundle branch block (RBBB)
VSD-LAH, LVH (if large, also RVH):
- Notched or peaked P-waves (large VSD)
PDA: Biventricular hypertrophy (large PDA)
AV canal: Superior axis, LVH, RVH:
- RBBB and prolonged PR interval
AS: Normal to LVH (severe cases)
PS: Normal to RVH, RAE (severe cases):
- RBBB
Coarctation of aorta: RVH or RBBB
HLHS: RAE, RVH, peaked P-waves

Differential Diagnosis

CHF
Hypertrophic cardiomyopathy
Cardiogenic shock
Aortic dissection
Myocarditis
Bronchopulmonary dysplasia
Pulmonary HTN
Pneumonia/bronchiolitis
Hypoglycemia
Adrenal insufficiency, CAH
Glycogen storage diseases
Sepsis
Shock

Treatment

Initial Stabilization/Therapy

Maintain warmth and oxygenation.
Treat hypoglycemia and acidosis.
Establish IV access.
Prepare for endotracheal intubation.

High oxygen tensions promote ductal closure. �

Ed Treatment/Procedures

Administer prostaglandin E1 (PGE1) to dilate or reopen the ductus arteriosus:
- Continuous IV infusion 0.05-0.1 μg/kg/min
- Complications include apnea, bradycardia, hypotension, and seizures.
Evaluate and treat alternate causes:
- Septic workup and empiric antibiotics
- Maintain normoglycemia
Circulatory collapse from CHD:
- Fluid resuscitation (increments of 10 mL/kg)
- Inotropes
- Aggressive treatment of acidosis
CHF:
- Digoxin and diuretics

Medication

Ampicillin 50 mg/kg IV
Digoxin dosing requires extreme caution:
- Range 25-40 μg/kg IV
Dobutamine: 5-20 μg/kg/min IV
Dopamine: 2-20 μg/kg/min IV
Epinephrine: 0.1-2 μg/kg/min IV
Furosemide: 1 mg/kg IV
Gentamicin: 4 mg/kg/d IV or 2.5 mg/kg/dose
Milrinone 0.25-1 μg/kg/min
PGE1: 0.05-0.1 μg/kg/min
Sodium bicarbonate: 1-2 mEq/kg IV

Follow-Up

Disposition

Admission Criteria

All newborns with suspected CHD:
- Admit to pediatric ICU.
CHD with acute worsening of cyanosis or CHF
CHD with pneumonia or bronchiolitis

Discharge Criteria
Determine in consult with cardiologist �
Issues for Referral
Primary care physician to coordinate care with cardiologist and cardiothoracic surgery �

Followup Recommendations

Plan for follow-up should be made in consult with the pediatric cardiologist. �

Pearls and Pitfalls

Acyanotic lesions presenting at 2-12 wk:
- Coarctation as DA closes
- Septal defects as pulmonary vascular resistance drops
Classic ECG in AV canal: Superior QRS axis
Classic CXR in coarctation: Rib notching (late)

Additional Reading

Bonow �RO, Mann �DL, Zipes �DP, et al., eds. Congenital heart disease. Braunwalds Heart Disease. 98th ed. Philadelphia, PA: Saunders Elsevier; 2012:1411-1467.
Dolbec �K, Mick �N. Congenital heart disease. Emerg Med Clin North Am. 2011;29:811-827.
Yee �L. Cardiac emergencies in the first year of life. Emerg Med Clin North Am. 2007;25:981-1008.

Codes

ICD9

745.4 Ventricular septal defect
745.5 Ostium secundum type atrial septal defect
746.89 Other specified congenital anomalies of heart
747.0 Patent ductus arteriosus
745.60 Endocardial cushion defect, unspecified type
747.10 Coarctation of aorta (preductal) (postductal)

ICD10

Q21.0 Ventricular septal defect
Q21.1 Atrial septal defect
Q24.8 Other specified congenital malformations of heart
Q25.0 Patent ductus arteriosus
Q21.2 Atrioventricular septal defect
Q25.1 Coarctation of aorta

SNOMED

78485007 Acyanotic congenital heart disease (disorder)
30288003 Ventricular septal defect (disorder)
70142008 Atrial septal defect (disorder)
83330001 Patent ductus arteriosus (disorder)
15459006 Endocardial cushion defect (disorder)
7305005 Coarctation of aorta (disorder)

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