0071643192.pdf

PEDIATRICS
SYMPTOMS

Infants and children can present to the ED with undiagnosed CHD at any time.
Both the age at presentation and type of presentation (presence/absence cyanosis,
presence/absence of increased pulmonary blood flow, presence/absence of CHF)
can give important clues to the underlying type of CHD. In infants, symptoms
are most often elicited during feedings. Poor feedings, weak cry, coughing,
wheezing, and poor growth or weight gain are also associated with CHD.

EXAM

Look for presence of typical murmurs and check pulse, blood pressure, and
differential O 2 saturation in all four extremities, comparing bilateral upper to
lower extremities.

DIFFERENTIAL

Sepsis, inborn errors of metabolism, endocrine disorders, and drug ingestion.
Patients with known CHD usually present for exacerbation of their underly-
ing condition, for which an evaluation of their underlying anatomy is often
needed (evaluation of shunt patency, etc.).

DIAGNOSIS

■ Thehyperoxia testis often helpful in testing for presence of a shunt as
cause of cyanosis (ability to increase PO 2 above 150 mm Hg on 100% O 2
suggests pulmonary cause of cyanosis rather than CHD).
■ Other useful tests include ECG, CXR, and blood gas (see Figure 5.2).
■ Further testing (eg, echocardiogram, MRI) depends on suspected diagnosis

TREATMENT

Depends on underlying disorder

TETRALOGY OFFALLOT(TOF)

The most common cyanotic CHD; the tetrad—R ventricular outflow tract
obstruction, overriding aorta, VSD, right ventricular hypertrophy (RVH). These
defects result in decreased pulmonary blood flow and varying degrees of R → L
shunting. Cyanosis can present at any time during infancy, depending on sever-
ity of R →L shunting.

CHD in infants often presents

with sweating during feeding

or poor feeding.

Pulmonary vessels

on CXR

Increased

L→ R shunt

Tachypnea Cyanosis

Admixture R→ L shunt

12 3

Decreased

VSD

ASD

PDA

CAVC

TAPVR

Truncus arteriosus

HLH

TGA

Tricuspid atresia

PA with IVS

HRH

TOF

CAVC: complete atrioventricular canal

TAPVR: total anomalous pulmonary venous return

HLH: hypoplastic left heart

TGA: transposition of the great arteries

PA with IVS: pulmonary atresia with intact ventricular septum

HRH: hypoplastic right heart

TOF: tetralogy of fallot

FIGURE 5.2. Classification system for congenital heart disease lesions based on CXR findings and associated
symptomatology.

(Reproduced, with permission, from Le T, Lam W, Rabizadeh S, Schroeder A, Vera K. First Aid for the Pediatric Boards,
New York: McGraw-Hill, 2006:63.)

TOF tetrad: RV outflow tract

obstruction, Overriding

aorta, VSD, RVH