airway for acute obstruction is of utmost important. Surgical excision or
sclerotherapy is the treatment of choice depending on the type and impact of the
lesion.
FIGURE 96.32 Benign gingival cysts. Epstein pearls. (Reprinted with permission from
O’Doherty N. Atlas of the Newborn . Philadelphia, PA: JB Lippincott; 1979.)
FIGURE 96.33 Bohn nodule. Pearly nodule on the gum of a neonate. (Reprinted with
permission from Fletcher MA. Physical Diagnosis in Neonatology . Philadelphia, PA:
Lippincott–Raven Publishers; 1998:216.)
NEONATAL CARDIAC EMERGENCIES
This section focuses on ductal-related structural heart disease in the neonate
presenting in the first few weeks of life. Please see Chapter 86 Cardiac
Emergencies for emergency management of arrhythmias, shock, and acquired
heart disease.
KEY POINTS
Congenital heart lesions can present as late as 2 to 3 weeks of age as
the ductus arteriosus closes.
Tachypnea may be the only sign of congestive heart failure.
Femoral pulses may be palpable with aortic coarctation because of
collateral vessels. Instead palpate distal lower extremity pulses and
obtain 4-extremity blood pressures.
Prostaglandin infusion may be lifesaving even if the specific cardiac
diagnosis is not known.
Right-to-left shunts often are not associated with heart murmurs.
RELATED CHAPTERS
Signs and Symptoms
Shock: Chapter 10
Cyanosis: Chapter 21
Heart Murmurs: Chapter 35
Respiratory Distress: Chapter 71
Septic-Appearing Infant: Chapter 73
Tachycardia: Chapter 77
Medical, Surgical, and Trauma Emergencies
Cardiac Emergencies: Chapter 86
Current Evidence
Cardiac disease presenting in the neonatal period can be acquired but is often
congenital. Age at presentation of CHD varies with the underlying lesion, its
impact on cardiac performance, and its dependence upon the ductus arteriosus
(DA). The DA, the conduit between the aorta and main pulmonary artery, allows
for fetal circulatory viability in utero by allowing blood to shunt away from the
collapsed lungs. Postnatally, patency of the DA can preserve systemic perfusion
even under conditions of abnormal cardiac anatomy. In cases of impaired
systemic blood flow, the right-to-left shunt of the DA mitigates the effects of even
complete aortic and/or mitral atresia, allowing right ventricular output to reach
the systemic circulation. In cases of obstructed pulmonary blood flow, the ductus
allows systemic (left ventricular) output to reach the pulmonary circulation. This
left-to-right shunt becomes crucial once the infant with impaired pulmonary
blood flow becomes dependent on his/her own pulmonary circulation for
oxygenation after delivery. The DA closes functionally within the first day of life
and is generally structurally closed by 3 weeks. Symptoms in neonates with
ductal-dependent lesions therefore typically present as early as the first few days
of life and up to 2 to 3 weeks of age.
Ductal-dependent cardiac lesions are divided into two categories. Ductaldependent pulmonary blood flow lesions, or right-sided obstructive lesions,
include pulmonary atresia, tricuspid atresia, and critical pulmonary stenosis.
Ductal-dependent systemic blood flow lesions, or left-sided obstructive lesions,
include hypoplastic left heart syndrome (aortic atresia), aortic coarctation, and
aortic arch interruption.
Goals of Treatment
For the baby with CHD and obstruction of the systemic or pulmonary blood flow,
closure of the ductus can precipitate rapid clinical deterioration. Emergency
intervention with prostaglandins is lifesaving and therapy must be initiated even
before a precise diagnosis is confirmed. Once supportive care is initiated and the
neonate stabilized, immediate consultation with cardiology is warranted, with
timely transfer to appropriate pediatric cardiac care center.
Clinical Considerations
Clinical Recognition
Newborn screening in the nursery is aimed at detecting cyanotic CHD and may
not detect noncyanotic lesions. Because CHD may be missed during postnatal
hospitalization, ED physicians should consider CHD in the differential diagnosis
of neonates with symptoms as vague as difficulty feeding, poor weight gain, or
irritability. Any newborn with sudden onset or persistence of central cyanosis,
severe hypoxemia, unexplained acidosis, shock, or tachypnea is considered at risk
for a ductal-dependent cardiac lesion.
Triage Considerations
Neonates should be triaged urgently because they can deteriorate quickly and
physical findings are subtle. Poor feeding may be the only presenting symptom
on history. Vital signs, oxygen saturation measurement, and careful auscultation
for murmurs should be performed. Symmetric strong distal pedal pulses are
reassuring. Pulse oximetry should be measured from the preductal right hand and
either postductal foot. A confirmed difference in oxygen saturation between hand
and foot confirms a patent ductus. Blood pressure should be measured in the right
arm and either leg. A confirmed difference in blood pressure of more than 20 mm
Hg between the right arm and any leg suggests aortic coarctation or interrupted
arch.
Clinical Assessment
Physical examination should be efficient but thorough. Carefully assess for gallop
or murmur. A pathologic murmur is often audible in neonates with CHD.
Qualities of pathologic murmurs include harsh quality, murmur intensity grade 3
or higher, pansystolic duration, or abnormal S2. Always check for symmetric
peripheral arterial pulses. Weakened distal pulses are concerning for aortic
coarctation and critical aortic stenosis but may be found with any low cardiac
output state. Hepatomegaly can be found in patients with left-sided obstructive