Neonatal Shock - Etiology, Clinical Manifestations, and Evaluation - UpToDate

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Neonatal shock: Etiology, clinical manifestations, and evaluation

Author: Beau Batton, MD
Section Editor: Richard Martin, MD
Deputy Editors: Melanie S Kim, MD, Carrie Armsby, MD, MPH
All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Sep 2020. | This topic last updated: Jun 09, 2020.
INTRODUCTION
Shock is a dynamic and unstable pathophysiologic state characterized by inadequate tissue perfusion. Although the
effects of inadequate perfusion are reversible initially, prolonged hypoperfusion and tissue hypoxia can disrupt critical
biochemical processes, which if not addressed result in cell death, end-organ failure, and, possibly, death.
The classification and underlying mechanisms of neonatal shock are the same as those seen in pediatric and adult
shock. However, the etiology, clinical manifestations, and initial management of neonatal shock differ somewhat from
shock in other populations. (See "Initial evaluation of shock in children" and "Definition, classification, etiology, and
pathophysiology of shock in adults".)
The etiology, clinical presentation, and evaluation of neonatal shock will be reviewed here. Management of neonatal
shock is discussed separately. (See "Neonatal shock: Management".)
DEFINITION
● Shock – Shock, or circulatory failure, is defined as a physiologic state characterized by tissue hypoxia due to
reduced oxygen delivery and/or increased oxygen consumption or inadequate oxygen utilization. It is manifested
by physical findings of tissue hypoperfusion (eg, cold extremities, acrocyanosis, and poor capillary refill),
hypotension, and metabolic acidosis. Shock is often initially reversible, but must be recognized and treated
immediately to prevent progression to irreversible organ dysfunction.
It is important to recognize that hypotension, which is commonly used to define shock states in adults, is generally
a late finding of shock in neonates.
● Hypotension – In older infants and children, hypotension is commonly defined by a numeric threshold such as
blood pressure (BP) below the 5th percentile for age. However, characterizing a neonate's BP as "normal" or
"abnormally low" using a numerical definition is challenging because BP values vary considerably depending on
birth weight, gestational age, and postnatal age (figure 1 and figure 2). For these reasons, low BP should not be
the sole criteria for therapeutic intervention (see 'Vital signs abnormalities' below). In addition, extremely preterm
(EPT) infants may maintain adequate perfusion despite numerically low BP values. The definition of hypotension
(low blood pressure) in EPT infants is discussed in greater detail separately. (See "Assessment and management of
low blood pressure in extremely preterm infants", section on 'Definitions'.)
PATHOPHYSIOLOGY
Shock, a state of cellular and tissue hypoxia, is due to reduced oxygen delivery, increased oxygen consumption, and/or
inadequate oxygen utilization [1-3]. Cellular hypoxia results in a switch to anaerobic metabolism and accumulation of
lactic acid. Increasing levels of lactic acid causes metabolic acidosis, which interferes with cell and organ function and,
if not addressed, cell death. Tissue hypoperfusion also leads to endothelial dysfunction, stimulation of inflammatory
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and anti-inflammatory cascades, and activation of local humoral processes that disrupt the microcirculation, resulting
in further tissue injury. If untreated, these processes result in circulatory collapse, major organ failure, and death.
Shock is classified based on the underlying pathophysiologic mechanism:
● Hypovolemic (see 'Hypovolemic shock' below)

● Distributive (see 'Distributive shock' below)
● Cardiogenic (see 'Cardiogenic shock' below)
● Obstructive (see 'Obstructive shock' below)
● Multifactorial (see 'Multifactorial shock' below)
When the underlying mechanism is unclear or unknown, the term "undifferentiated shock" is used.
The pathophysiology of shock is discussed in greater detail separately. (See "Pathophysiology and classification of
shock in children".)
ETIOLOGIC CLASSIFICATION
Shock can be classified based on the underlying pathogenesis: hypovolemic, distributive, cardiogenic, and obstructive
shock (table 1). However, these processes are not mutually exclusive, and neonates with circulatory failure may have a
combination of more than one form of shock (multifactorial shock). When the underlying mechanism is unclear or
unknown, the term "undifferentiated shock" is used.
Hypovolemic shock — Hypovolemic shock occurs due to insufficient circulating blood volume, resulting in a reduction
in cardiac output and reduced oxygen delivery. Neonatal shock due to hypovolemia is most commonly due to
hemorrhage [4].
Causes of hemorrhagic shock in the neonate include:
● Fetomaternal hemorrhage (see "Spontaneous massive fetomaternal hemorrhage")
● Acute hemorrhage from umbilical cord prolapse or rupture associated with abnormal presentation (vasa previa) or
insertion (velamentous cord) (see "Umbilical cord prolapse" and "Velamentous umbilical cord insertion and vasa
previa")
● Acute bleeding into the subgaleal space (subgaleal hemorrhage) following a vacuum-assisted delivery (see
"Neonatal birth injuries", section on 'Subgaleal hemorrhage')
● Massive internal bleeding in the gastrointestinal (GI) tract, brain, lungs, or other major organ (see "Germinal
matrix hemorrhage and intraventricular hemorrhage (GMH-IVH) in the newborn: Pathogenesis, clinical
presentation, and diagnosis" and "Neonatal necrotizing enterocolitis: Clinical features and diagnosis")
● Tumor-associated acute hemorrhage (eg, sacral coccygeal teratoma) (see "Sacrococcygeal germ cell tumors")
In addition, acute blood transfusion between monochorionic twins may result in hypovolemic shock. (See "Twin-twin
transfusion syndrome and twin anemia polycythemia sequence: Screening, prevalence, pathophysiology, and
diagnosis".)
Other causes of hypovolemic shock include third spacing (ie, movement of intracellular fluid into the extravascular
space), as can occur with perinatal distress, and acute intestinal injury (eg, volvulus, necrotizing enterocolitis, intestinal
perforation). Less common causes include excess GI fluid loss from congenital chloridorrhea, polyuria due to
congenital diabetes insipidus, and neonatal nephrotic syndrome.
Distributive shock — Distributive shock is characterized by severely reduced systemic vascular resistance (SVR) and
abnormal vascular tone, which results in maldistribution of blood flow within the microcirculation and regional and
global hypoperfusion. The following are causes of neonatal distributive shock:
● Sepsis – In neonates, sepsis is the most common cause of distributive shock. Sepsis releases vasoactive mediators
that depress autonomic nervous system regulation of the systemic circulations resulting in diffuse vasodilation
and impaired perfusion. However, the mechanisms by which this occurs and the impact of altered autonomic
nervous system function are different in septic newborn infants compared with older patients. Sepsis may also
cause myocardial dysfunction (cardiogenic shock). (See "Pathophysiology of sepsis", section on 'Circulation' and
"Clinical features, evaluation, and diagnosis of sepsis in term and late preterm infants".)
● Disseminated herpes simplex virus (HSV) infection – The mechanisms underlying shock in neonates with
disseminated HSV infection are similar to those seen in bacterial sepsis. Disseminated HSV infection should be
suspected in infants with a sepsis-like illness with a maternal history of genital herpes lesions. However, most
neonates with HSV infection are born to asymptomatic women with no known history of HSV infection. Evaluation
and treatment of neonatal HSV is discussed in greater detail separately. (See "Neonatal herpes simplex virus
infection: Clinical features and diagnosis", section on 'Disseminated disease' and "Neonatal herpes simplex virus
infection: Management and prevention".)
● Adrenal insufficiency – Shock due to adrenal insufficiency is due to impaired synthesis or release of adrenocortical
hormones. This is most commonly associated with congenital adrenal hyperplasia. (See "Causes and clinical
manifestations of primary adrenal insufficiency in children", section on 'Adrenal crisis'.)
● Other rare conditions resulting in distributive shock include toxic shock syndrome and hydrops fetalis (the latter
more often is multifactorial) [5]. (See "Staphylococcal toxic shock syndrome" and 'Multifactorial shock' below.)
Cardiogenic shock — Cardiogenic shock is characterized by reduced cardiac output due to ventricular dysfunction or
rhythm disturbance. Cyanosis and hypoxemia are common manifestations when there is also inadequate pulmonary
blood flow. Cardiogenic shock in the neonate can be caused by a variety of conditions, including the following [4]:
● Myocardial ischemia/hypoxemia can lead to myocardial damage resulting in poor contractility. Myocardial injury
and dysfunction may be complications associated with intrapartum asphyxia, systemic bacterial infection, and
chronic fetal hypoxemia.
● Congenital heart disease (CHD) – Some infants with critical CHD may present in the early neonatal period in
cardiogenic shock as the ductus arterious closes and systemic perfusion decreases. Most commonly, this is seen in
infants with unsuspected critically obstructive left heart lesions (table 2), including:
• Hypoplastic left heart syndrome (HLHS) (figure 3) (see "Hypoplastic left heart syndrome: Anatomy, clinical
features, and diagnosis").
• Critical aortic valve stenosis (see "Valvar aortic stenosis in children", section on 'Critical aortic stenosis').
• Critical coarctation of the aorta (figure 4) (see "Clinical manifestations and diagnosis of coarctation of the
aorta").
• Interrupted aortic arch (figure 5).
Infants with total anomalous pulmonary venous connection (TAPVC) (figure 6) most commonly present with
cyanosis and tachypnea. However, if there is significant obstruction at the atrial communication, systemic
perfusion will be impaired. (See "Identifying newborns with critical congenital heart disease", section on 'Shock'.)
● Cardiac arrhythmias
• Complete congenital heart block due to maternal antibodies in neonates born to mothers with systemic lupus
erythematosus or Sjögren syndrome. (See "Neonatal lupus: Epidemiology, pathogenesis, clinical
manifestations, and diagnosis", section on 'Heart block'.)
• Supraventricular tachycardia. (See "Clinical features and diagnosis of supraventricular tachycardia in children".)
• Ventricular tachycardia. (See "Causes of wide QRS complex tachycardia in children".)
● Myocarditis may occur as an isolated illness or as a component of a systemic illness. In the newborn, myocarditis
is usually due to viral infection, most commonly coxsackievirus. (See "Clinical manifestations and diagnosis of
myocarditis in children".)
● Congenital cardiomyopathy is a rare cause of cardiac dysfunction that typically presents with hydrops fetalis.
Obstructive shock — Obstructive shock occurs when extracardiac diseases lead to impaired cardiac output.
Obstructive shock is categorized as either pulmonary vascular (eg, pulmonary embolism, severe pulmonary
hypertension) or mechanical (eg, tension pneumothorax, pericardial tamponade, constrictive pericarditis).
Pneumothorax and pulmonary hypertension are potential complications of severe neonatal respiratory disease (eg,
respiratory distress syndrome, meconium aspiration). Pulmonary embolism and cardiac tamponade rarely cause
neonatal shock. (See "Pulmonary air leak in the newborn", section on 'Pneumothorax' and "Persistent pulmonary
hypertension of the newborn".)
Multifactorial shock — Neonatal multifactorial shock may be observed in the following clinical settings. It is important
to identify the multiple etiologies in order to guide treatment decisions.
● Sepsis – As noted above, sepsis is typically classified as distributive shock. However, patients may exhibit
cardiogenic shock due to myocardial depression and hypovolemic shock due to capillary leak and third spacing
(movement of intracellular fluid into the extravascular space). (See "Clinical features and diagnosis of bacterial
sepsis in preterm infants <34 weeks gestation", section on 'Severe sepsis and septic shock'.)
● Severe intestinal injury – Patients with severe intestinal injury (eg, Stage III necrotizing enterocolitis [NEC] or
malrotation with midgut volvulus) may develop circulatory collapse due to hypovolemic shock from third spacing
and distributive shock caused by sepsis. (See "Neonatal necrotizing enterocolitis: Clinical features and diagnosis"
and "Intestinal malrotation in children", section on 'Clinical presentation'.)
● Pulmonary hypertension – Pulmonary hypertension is typically classified as obstructive shock. However, patients
with severe disease commonly have biventricular dysfunction leading to cardiogenic shock. (See "Persistent
pulmonary hypertension of the newborn".)
● Hydrops fetalis ‒ Hydrops fetalis is defined as abnormal fetal fluid collection in a minimum of two anatomic
locations caused by dysregulation of the net fluid movement between the vascular and interstitial spaces. The
etiology of hydrops fetalis is broad, and neonatal shock is a common manifestation of an affected infant at birth.
Distributive shock occurs in all patients with hydrops fetalis due to capillary leak, but neonates with hydrops fetalis
and shock frequently may exhibit hypovolemic shock (eg, third spacing), cardiogenic shock (eg, congenital cardiac
disease), and obstructive shock (eg, congenital obstructive lymphatic abnormality). (See "Postnatal care of hydrops
fetalis".)
CLINICAL MANIFESTATIONS
Vital signs abnormalities — Vital sign abnormalities are a hallmark of shock and may include:
● Abnormal heart rate (HR) – Tachycardia (HR >180 beats per minute) is a common but nonspecific finding in
neonatal shock. Increasing the HR is the neonate's chief compensatory mechanism to maintain cardiac output
when there is a limited capacity to increase stroke volume (SV) [6]. Variability of HR may be another early sign of
septic shock [7]. In term infants, bradycardia (HR <90 beats per minute) is often a terminal finding, whereas in
preterm infants, bradycardia can occur at any time.
● Hypotension – Low blood pressure (BP) (hypotension) is generally a late finding of shock, and ideally shock is
diagnosed prior to the onset of hypotension [6]. BP below the 5th percentile for GA and postnatal age is cause for
concern and warrants additional investigation. If low BP is accompanied by clinical signs of poor perfusion,
treatment should be initiated. (See "Neonatal shock: Management".)
Definitions of hypotension are based on normative BP values, which vary somewhat depending on gestational and
postnatal age:
• Full-term infants (figure 7)

• Preterm infants – Gestational age (GA) 28 to 36 weeks (figure 2)
• Extremely preterm (EPT) infants – GA <28 weeks (figure 1)
Of note, BP in very preterm (VPT) infants correlates poorly with systemic blood flow such that BP alone is an
imperfect measure to detect early shock, especially in EPT infants [8-10]. EPT infants may have low BP and still have
adequate perfusion and may not need further interventions. This is discussed in greater detail separately. (See
"Assessment and management of low blood pressure in extremely preterm infants".)
By contrast, the finding of hypotension in a neonate with hypovolemic shock may signal a need for massive
volume resuscitation. This is because hypotension does not occur as a manifestation of hypovolemic shock until
the neonate has lost 30 to 40 percent of its blood volume. (See "Neonatal shock: Management", section on 'Acute
blood loss'.)
● Abnormal body temperature – Fever is often present with neonatal sepsis. However, infants with shock due to
any etiology (including sepsis) may have impaired autonomic nervous system function, which can result in
hypothermia.
Decreased peripheral perfusion — Signs of decreased peripheral perfusion include:
● Cool extremities, acrocyanosis, and pallor are initial signs of decreasing cardiac output, as vasoconstrictive
mechanisms compensate for decreased tissue perfusion by redirecting blood from the periphery to the vital
organs (eg, coronary, cerebral, and adrenal perfusion). These signs may be difficult to differentiate from findings in
the normal neonate whose hands and feet are often cool to the touch with varying degrees of acrocyanosis. Pallor
may also be an indication of anemia.
● Delayed capillary refill >4 seconds is suggestive of neonatal shock especially in combination with other findings
indicative of poor peripheral perfusion (low BP, weak pulses, cool extremities, and abnormal neurologic signs) [8,9].
As an isolated finding, the predictive value of capillary refill is poor, and it is not a reliable physical finding alone to
either confirm the diagnosis of shock or to assess response to therapy in newborns [8,11,12].
Neurologic findings — In the initial stages of shock, nonspecific neurologic findings may include lethargy, irritability,
poor feeding, and poor tone. In the later stages, there is progression to stupor or coma. Other neurologic findings
include decreased spontaneous movements of the extremities, diminished deep tendon reflexes, and absence of
primitive reflexes (eg, palmar grasp reflex, Moro reflex, and rooting reflex). However, the differential diagnosis of
abnormal neurological findings in a neonate is broad, and shock should not be presumed to be the underlying cause.
(See "Neurologic examination of the newborn".)
Respiratory findings — The following nonspecific respiratory findings may be seen in neonatal shock:
● Tachypnea is generally seen in infants with septic or cardiogenic shock as a compensatory response to metabolic
acidosis due to lactate production from poorly perfused tissue.
● Other signs of respiratory distress, such as grunting, retractions, nasal flaring, and gasping, often accompany
tachypnea. These are observed especially in patients with primary pulmonary disease (eg, pneumonia) or
cardiopulmonary compromise with pulmonary edema.
● Periodic breathing and apnea are usually centrally mediated and are more likely to be associated with decreased
cerebral perfusion or significant metabolic acidosis that impairs cerebral function. As a result, these findings can
present in all forms of neonatal shock.
● Hypoxemia may be present in infants with shock due to cardiac dysfunction or obstructed blood flow, but isolated
hypoxemia is rare. Hypoxemia associated with neonatal shock may be due to persistent pulmonary hypertension
that may accompany bacterial infections, especially those caused by group B streptococcus (GBS), cyanotic
congenital heart disease (CHD), and severe anemia. (See "Persistent pulmonary hypertension of the newborn",
section on 'Maladaptation' and "Cardiac causes of cyanosis in the newborn".)
Other findings
● Renal – There is a strong correlation between low urinary output (oliguria) and low systemic blood flow. However,
there is often a time delay between the development of circulatory compromise and the recognition of oliguria
[13].
● Gastrointestinal (GI) – The following nonspecific GI findings may be seen in patients with neonatal shock:
• Poor feeding due to lethargy and/or respiratory distress.
• Vomiting as a manifestation of decreased motility that results from non-mechanical disruption of the normal
coordinated propulsive motor activity of the GI tract, which may progress to paralytic ileus. Bilious emesis is
uncommon, and when present it should prompt an investigation into intra-abdominal causes of shock (eg,
malrotation with midgut volvulus or necrotizing enterocolitis). (See "Intestinal malrotation in children" and
"Neonatal necrotizing enterocolitis: Clinical features and diagnosis".)
• Abdominal distension as a manifestation of ileus.
Laboratory findings — The most common laboratory feature characteristic of neonatal shock is metabolic acidosis
with decrease in serum/plasma bicarbonate and an increase in lactate due to increased anaerobic metabolism
(increased lactate production) and decreased clearance of lactate.
Other less common laboratory features include:
● Anemia due to blood loss observed in hemorrhagic hypovolemic shock [14,15] or hemolysis in septic shock.
● Prolonged prothrombin time (PT)/international normalized ratio (INR) and partial thromboplastin time (PTT) tests
can occur with consumptive coagulopathy, which may be present in infants with septic shock or complications
from pregnancy, including fetal anoxia/birth asphyxia (distributive shock) and placental abruption (hypovolemic
shock). Coagulopathy can also occur as a consequence of hepatic dysfunction. (See "Disseminated intravascular
coagulation in infants and children", section on 'Other etiologies in neonates'.)
● Glucose levels may be elevated or decreased during neonatal shock. The glucose level varies depending upon
catecholamine release during the body's stress response (which affects insulin release and peripheral utilization of
glucose) and the body stores of glucose and glycogen. (See "Neonatal hyperglycemia" and "Pathogenesis,
screening, and diagnosis of neonatal hypoglycemia".)
● Hyperkalemia is a result of release of intracellular potassium stores due to acidosis, tissue injury, and cell death. It
may be further exacerbated by renal impairment. Hyperkalemia may also be a manifestation of congenital adrenal
hyperplasia, an important though rare cause of neonatal shock. (See "Neonatal acute kidney injury: Pathogenesis,
etiology, clinical presentation, and diagnosis", section on 'Presentation due to other laboratory abnormalities' and
"Causes and clinical manifestations of primary adrenal insufficiency in children", section on 'Adrenal crisis'.)
● Serum bilirubin levels and liver enzymes may be elevated due to hepatic injury and dysfunction.
● Serum creatinine and blood urea nitrogen (BUN) may be elevated due to acute kidney injury.
DIAGNOSIS
The diagnosis of shock is clinically based on a constellation of clinical, biochemical, and hemodynamic features. These
include physical findings of tissue hypoperfusion (eg, cold extremities, acrocyanosis, and poor capillary refill), and
metabolic acidosis. Patients in the early stages of shock may present with tachycardia, peripheral vasoconstriction (eg,
cold extremities and acrocyanosis), and normal blood pressure (BP) (ie, compensated shock). Hypotension typically
occurs in the late stages of shock. Bradycardia is usually observed in the terminal stage in term infants, but may occur
at any time in preterm infants. (See 'Clinical manifestations' above.)
INITIAL STABILIZATION
Stabilization of the patient's hemodynamic status takes precedence over the diagnostic evaluation. Resuscitation
should not be delayed to obtain history, perform a physical examination, or obtain laboratory tests. However, during
the initial stabilization, a focused evaluation to determine the etiology should occur concomitantly in order to best
direct subsequent therapy. The goals of the initial stabilization and evaluation include rapid recognition and correction
of circulatory compromise and other life-threatening conditions.
The approach to initial stabilization is discussed in greater detail separately (algorithm 1). (See "Neonatal shock:
Management", section on 'Initial stabilization'.)
It generally includes the following interventions:
● Provide respiratory support as needed. (See "Mechanical ventilation in neonates".)
● Obtain vascular access. (See "Vascular (venous) access for pediatric resuscitation and other pediatric emergencies",
section on 'Umbilical vein access'.)
● Administer initial fluid bolus – An initial fluid bolus of 10 to 20 mL/kg isotonic crystalloid is appropriate for most
neonates presenting in shock. However, fluid boluses should be administered cautiously in extremely preterm
infants (gestational age <28 weeks) or if there is suspicion for a cardiac etiology of shock. The initial fluid
resuscitation in neonatal shock is discussed in greater detail separately. (See "Neonatal shock: Management",
section on 'Fluid resuscitation'.)
● Administer empiric antibiotics, and, if there is clinical suspicion for herpes simplex infection, acyclovir. (See
"Management and outcome of sepsis in term and late preterm infants", section on 'Initial empiric therapy' and
"Neonatal herpes simplex virus infection: Management and prevention", section on 'Acyclovir therapy'.)
● Address any correctable abnormalities noted on the initial evaluation. (See "Neonatal shock: Management", section
on 'Other interventions'.)
DIAGNOSTIC EVALUATION
For neonates presenting with clinical signs of shock, a focused diagnostic evaluation to determine the underlying cause
should occur in concert with initial stabilization. The type and cause of shock can generally be determined from the
history, physical examination, and basic diagnostic studies, which may guide management (algorithm 1) [6]. (See
"Neonatal shock: Management", section on 'Ongoing goal-directed therapy'.)
History — The newborn history, including review of maternal health issues, antenatal screening, and pregnancy and
delivery complications, often can identify the underlying cause of shock.
The following historical findings may help identify the underlying mechanism of shock (table 1) (see 'Etiologic
classification' above):
● Hypovolemic shock:
• Significant blood loss from placental anomalies, maternal bleeding, or umbilical cord abnormalities. (See
"Placenta previa: Epidemiology, clinical features, diagnosis, morbidity and mortality".)
• Internal bleeding due to traumatic vacuum-assisted delivery (eg, subgaleal bleed). (See "Neonatal birth
injuries".)
● Septic shock (see "Clinical features, evaluation, and diagnosis of sepsis in term and late preterm infants", section
on 'Maternal risk factors'):
• Prolonged rupture of membrane
• Maternal chorioamnionitis
• Maternal fever during labor
• Group B streptococcal (GBS) bacteriuria during the pregnancy
• Previous delivery of an infant affected by GBS disease
• Maternal history of herpes genital lesions may be indicative of shock due to disseminated herpes simplex virus
(HSV) infection. (See "Neonatal herpes simplex virus infection: Clinical features and diagnosis", section on
'Transmission'.)
● Cardiogenic shock:
• Maternal history of systemic lupus erythematosus or Sjögren syndrome resulting in neonatal heart block. (See
"Neonatal lupus: Epidemiology, pathogenesis, clinical manifestations, and diagnosis".)
• Antenatal asphyxia with abnormal heart rate (HR) pattern. (See "Perinatal asphyxia in term and late preterm
infants", section on 'Cardiovascular manifestations'.)
• Congenital heart disease (CHD) detected by prenatal ultrasound or newborn screening. (See "Fetal cardiac
abnormalities: Screening, evaluation, and pregnancy management" and "Newborn screening for critical
congenital heart disease using pulse oximetry".)
● Hydrops fetalis is generally characterized by distributive shock but frequently is associated with multifactorial
shock. (See "Postnatal care of hydrops fetalis".)
In some cases, fetuses with an increased risk of requiring resuscitation at birth can be identified prior to delivery. In
such cases, the neonatal team should be alerted so that preparations for resuscitation can be set up in the delivery
room. (See "Neonatal resuscitation in the delivery room", section on 'High-risk delivery'.)
Physical examination — The physical examination is important to assess the severity of shock and to monitor the
response to therapeutic interventions. (See "Neonatal shock: Management", section on 'Monitoring'.)
Physical findings in neonatal shock are generally nonspecific and include decreased peripheral perfusion (cold
extremities, acrocyanosis, delayed capillary refill) and poor vigor. (See 'Clinical manifestations' above.)
However, certain findings may point to a specific etiology. These include:
● Pathologic murmurs and/or gallop rhythm may raise suspicion of a cardiac cause. However, some forms of critical
CHD may not have an appreciable murmur (eg, hypoplastic left heart syndrome [HLHS]). Murmurs commonly
occur in some non-cardiac causes of neonatal shock (eg, tricuspid regurgitation murmur in birth asphyxia). (See
"Approach to the infant or child with a cardiac murmur" and "Common causes of cardiac murmurs in infants and
children".)
● Weak or absent lower extremity pulses (particularly in comparison to upper extremity pulses) might suggest
cardiogenic shock due to critical coarctation of the aorta. (See "Clinical manifestations and diagnosis of coarctation
of the aorta", section on 'Neonates'.)
● Chest asymmetry and absent breath sounds on one side might suggest tension pneumothorax. (See "Pulmonary
air leak in the newborn", section on 'Pneumothorax'.)
● Abdominal distension might suggest necrotizing enterocolitis and sepsis. (See "Neonatal necrotizing
enterocolitis: Clinical features and diagnosis", section on 'Clinical presentation'.)
● Ambiguous genitalia should raise suspicion for the possibility of adrenal insufficiency (table 3). (See "Causes and
clinical manifestations of primary adrenal insufficiency in children", section on 'Adrenal crisis' and "Causes and
clinical manifestations of primary adrenal insufficiency in children", section on 'Congenital adrenal hyperplasia'.)
● Skin findings might suggest an infectious etiology (cellulitis, vesicles [herpes simplex virus]). (See "Neonatal
herpes simplex virus infection: Clinical features and diagnosis", section on 'Neonatal HSV'.)
● Rash comprising erythematous annular lesions or arcuate macules located primarily on the scalp and periorbital
area is one of the physical diagnostic findings of neonatal lupus, which can present with shock due to congenital
heart block. (See "Neonatal lupus: Epidemiology, pathogenesis, clinical manifestations, and diagnosis", section on
'Rash'.)
Laboratory and imaging tests
Initial routine testing — The following tests may help identify the cause, assess the severity, and guide the initial
treatment.
● Arterial blood gas – The arterial blood gas measures the degree of acidosis and hypoxemia, which are important
factors in the assessment and management of patients with shock. The blood gas does not help to distinguish
between different types of shock but rather confirms shock and assesses its severity. Blood gases are also an
important to assess the effectiveness of interventions. (See "Neonatal shock: Management", section on
'Monitoring'.)
• Metabolic acidosis is observed in most cases primarily due to accumulation of lactate.
• Respiratory acidosis may be seen, particularly in patients with apnea or primary pulmonary disease (eg,
pneumonia).
• Hypoxemia is often, but not always, observed depending on the degree of respiratory compromise and
underlying etiology (eg, cyanotic CHD and primary pulmonary disorders are likely to present with hypoxemia).
Some point-of-care blood gas analyzers permit simultaneous measurement of electrolytes and lactate, which
provides additional useful information.
● Lactate – Serum lactate is a marker of tissue perfusion and is used to measure the severity of shock (lactate levels
increase as the severity increases). Lactate is obtained at baseline and can be serially measured to follow the
response to therapy. (See "Neonatal shock: Management", section on 'Monitoring'.)
● Complete blood count (CBC) with differential − Abnormalities of any of the three cell lines may occur in neonatal
shock; however, these are nonspecific findings and may not be useful in diagnosing the underlying cause:
• Both elevated (>20,000/mm3) and depressed (<5000/mm3) total white blood cell (WBC) counts are associated
with systemic bacterial infection, which may result in septic shock. However, neither is a reliable marker for
bacterial infection. (See "Clinical features, evaluation, and diagnosis of sepsis in term and late preterm infants",
section on 'Complete blood count'.)
• Thrombocytopenia may be suggestive of disseminated intravascular coagulation due to complications from

pregnancy including fetal anoxia/birth asphyxia (distributive shock) or placental abruption (hypovolemic
shock), or neonatal septic shock [16]. (See "Disseminated intravascular coagulation in infants and children",
section on 'Other etiologies in neonates'.)
• Anemia is seen in neonatal shock due to massive blood loss or hemolysis from overwhelming sepsis.
● Blood chemistries – Basic chemistry tests include electrolytes, glucose, renal (blood urea nitrogen [BUN], and
creatinine) and liver function studies:
• As noted above, hyperkalemia and both hypo- and hyperglycemia may occur in neonatal shock.
• Serum bicarbonate is depressed as an indication of metabolic acidosis.
• Baseline renal and liver function studies should be obtained, and levels should be monitored to detect any
abnormalities indicating end-organ damage and recovery.
• Hyponatremia and hyper- and hypoglycemia are typical manifestations of distributive shock due to adrenal
insufficiency.
● Blood cultures – Blood cultures should be obtained in any infant who may have septic shock or shock without an
identifiable cause.
● Viral testing – If there is concern for HSV, appropriate samples should be collected and sent for polymerase chain
reaction and viral culture. Evaluation for HSV infection should be performed if the neonate had perinatal exposure
to HSV, has physical findings consistent with HSV (mucocutaneous vesicles (picture 1A-C)), and/or has
cerebrospinal fluid pleocytosis. In addition, the diagnosis should be considered in neonates with concerning
findings that are otherwise unexplained. These may include seizures, focal neurologic signs, abnormal
neuroimaging, sepsis-like illness (fever or hypothermia, irritability, lethargy, respiratory distress, apnea, abdominal
distension, hepatomegaly, ascites), thrombocytopenia, or elevated liver transaminases. The evaluation of
suspected HSV infection in neonates is discussed in greater detail separately. (See "Neonatal herpes simplex virus
infection: Clinical features and diagnosis", section on 'Evaluation and diagnosis'.)
● Type and cross – Blood type, antibody screen, and crossmatching should be performed for infants with
hypovolemic shock due to blood loss who will require red blood cell transfusion. (See "Red blood cell transfusions
in the newborn", section on 'Acute blood loss' and "Red blood cell transfusion in infants and children: Selection of
blood products", section on 'Newborns and young infants'.)
● Chest radiograph – Chest radiography can be useful for neonates with respiratory distress or an abnormal
cardiopulmonary examination. An abnormal chest radiograph may indicate pulmonary disease (eg, tension
pneumothorax, pneumonia) or may suggest a cardiac abnormality (eg, cardiomegaly, abnormal pulmonary
vascular markings, pulmonary venous congestion).
Additional selective testing — Additional testing, which is based upon the findings of the initial evaluation and the
infant's response to initial interventions (see "Neonatal shock: Management", section on 'Interventions for suspected
etiologies') may include:
● Echocardiography – Echocardiography should be performed if there is suspicion for a cardiac etiology of shock.
Cardiac disease may be suspected on the basis of any of the following:
• Physical findings (eg, murmur, gallop, weak or absent femoral pulses)

• Cyanosis that does not improve with administration of 100 percent oxygen
• Differential in pre- and postductal oxygen saturation
• Clinical deterioration with fluid administration
• Abnormal electrocardiogram
• Abnormal chest radiograph (eg, cardiomegaly, pulmonary edema)
Consultation with a pediatric cardiologist is suggested if a cardiac etiology is suspected. Echocardiography

provides detailed assessment of the cardiac anatomy, and it is the test of choice for diagnosing congenital heart
disease (CHD). In addition, it provides an assessment of cardiac function and it can identify other potential
etiologies of shock (eg, persistent pulmonary hypertension of the newborn, tamponade). Thus, it is reasonable to
perform echocardiography in infants with persistent or severe shock even in the absence of the above findings.
(See "Diagnosis and initial management of cyanotic heart disease in the newborn", section on 'Echocardiography'.)
● Electrocardiogram (ECG) – ECG should be performed if an abnormal rhythm is noted on examination or cardiac
monitoring or if CHD is suspected. (See "Diagnosis and initial management of cyanotic heart disease in the
newborn", section on 'Electrocardiogram'.)
● Abdominal imaging – Abdominal radiographs may be useful in the evaluation of infants with a suspected
abdominal source for sepsis or shock (eg, necrotizing enterocolitis [NEC] or volvulus). Abdominal ultrasonography
can be used to detect bleeding into the abdomen or kidney. (See "Neonatal necrotizing enterocolitis: Clinical
features and diagnosis", section on 'Radiologic evaluation'.)
● Head ultrasonography (HUS) – HUS is appropriate to evaluate for intracranial hemorrhage, which may be
suspected based on clinical findings (eg, abnormal neurologic examination) or because of evidence of acute blood
loss without a clear source. (See "Germinal matrix hemorrhage and intraventricular hemorrhage (GMH-IVH) in the
newborn: Pathogenesis, clinical presentation, and diagnosis", section on 'Cranial ultrasound'.)
● Lumbar puncture (LP) – LP is a routine part of the evaluation for suspected bacterial sepsis in neonates and for
suspected herpes simplex virus infection. However, many neonates with shock are too unstable to safely undergo
LP, and therefore this testing is often deferred until the neonate is clinically stable. (See "Clinical features,
evaluation, and diagnosis of sepsis in term and late preterm infants", section on 'Lumbar puncture'.)
● Kleihauer-Betke assay – The Kleihauer-Betke assay is a method for quantifying fetal cells in maternal blood. A
positive test is indicative of fetomaternal hemorrhage, which may be a cause for hypovolemic shock [17]. (See
"Spontaneous massive fetomaternal hemorrhage", section on 'Kleihauer-Betke assay'.)
SUMMARY AND RECOMMENDATIONS
● Shock is a dynamic and unstable pathophysiologic state characterized by inadequate tissue perfusion due to
reduced oxygen delivery and/or increased oxygen consumption or inadequate oxygen utilization. If untreated it
leads to tissue/cellular damage that results in end-organ failure and, in some cases, death. (See 'Definition' above
and 'Pathophysiology' above.)
● The causes of neonatal shock are classified into four pathophysiologic mechanisms (table 1). However, neonatal
shock may be the result of more than one of these processes (multifactorial shock). (See 'Etiologic classification'
above.):
• Hypovolemic shock is characterized by impaired cardiac output due to significant fluid losses (eg, fetomaternal
bleeding). (See 'Hypovolemic shock' above.)
• Distributive shock is characterized by abnormal vascular tone, which results in maldistribution of blood flow
within the microcirculation and regional and global hypoperfusion. Sepsis is the most common cause of
distributive shock. (See 'Distributive shock' above.)
• Cardiogenic shock is characterized by reduced cardiac output due to ventricular dysfunction or arrhythmia.
(See 'Cardiogenic shock' above.)
• Obstructive shock is caused by extracardiac disorders (eg, tension pneumothorax) that result in decreased
cardiac output. (See 'Obstructive shock' above.)
● Regardless of the etiology, neonates with shock typically present with signs of poor perfusion (cool extremities,
acrocyanosis, pallor), tachycardia, and metabolic acidosis. Late signs of shock include bradycardia and
hypotension. (See 'Clinical manifestations' above.)
● Stabilization of the patient's hemodynamic status takes precedence over the diagnostic evaluation, and
resuscitation should not be delayed. However, a focused diagnostic evaluation is conducted in concert with
resuscitative efforts (algorithm 1):
• Key elements of the initial resuscitation include (see 'Initial stabilization' above):
- Stabilization of the airway and respiratory status

- Vascular access
- Initial fluid resuscitation
- Administration of empiric antibiotics
- Use of specific interventions based on the underlying etiology as determined by the concurrent diagnostic
evaluation
• Key elements of the initial diagnostic evaluation include (see 'Diagnostic evaluation' above):
- Brief review of the history (see 'History' above)

- Focused physical examination (see 'Physical examination' above)
- Basic laboratory tests (electrolytes, blood gas, complete blood count, lactate, blood culture, renal and liver
function tests, and type and cross) (see 'Initial routine testing' above)
- Chest radiograph if there are respiratory symptoms or findings

● Additional testing, which is based upon the findings of the initial evaluation and the infant's response to initial
interventions, may include (see 'Additional selective testing' above):
• Echocardiography
• Electrocardiogram
• Abdominal imaging
• Head ultrasonography
• Lumbar puncture
• Testing for fetal cells in maternal blood (Kleihauer-Betke assay)
ACKNOWLEDGMENT
The editorial staff at UpToDate would like to acknowledge Lisa Adcock, MD, who contributed to an earlier version of
this topic review.
Use of UpToDate is subject to the Subscription and License Agreement.
REFERENCES
1. Barber AE, Shires GT. Cell damage after shock. New Horiz 1996; 4:161.
2. Kristensen SR. Mechanisms of cell damage and enzyme release. Dan Med Bull 1994; 41:423.
3. Angus DC, van der Poll T. Severe sepsis and septic shock. N Engl J Med 2013; 369:840.
4. Kourembanas S. Shock. In: Manual of Neonatal Care, 6th ed, Cloherty JP, Eichenwald EC, Stark AR (Eds), Lippincott,
2008. p.176.
5. Takahashi N, Uehara R, Nishida H, et al. Clinical features of neonatal toxic shock syndrome-like exanthematous
disease emerging in Japan. J Infect 2009; 59:194.
6. Davis AL, Carcillo JA, Aneja RK, et al. American College of Critical Care Medicine Clinical Practice Parameters for
Hemodynamic Support of Pediatric and Neonatal Septic Shock. Crit Care Med 2017; 45:1061.
7. Griffin MP, Lake DE, Moorman JR. Heart rate characteristics and laboratory tests in neonatal sepsis. Pediatrics
2005; 115:937.
8. Miletin J, Pichova K, Dempsey EM. Bedside detection of low systemic flow in the very low birth weight infant on
day 1 of life. Eur J Pediatr 2009; 168:809.
9. Osborn DA, Evans N, Kluckow M. Clinical detection of low upper body blood flow in very premature infants using
blood pressure, capillary refill time, and central-peripheral temperature difference. Arch Dis Child Fetal Neonatal
Ed 2004; 89:F168.
10. Evans N. Assessment and support of the preterm circulation. Early Hum Dev 2006; 82:803.
11. LeFlore JL, Engle WD. Capillary refill time is an unreliable indicator of cardiovascular status in term neonates. Adv
Neonatal Care 2005; 5:147.
12. Schwepcke A, Weber FD, Mormanova Z, et al. Microcirculatory mechanisms in postnatal hypotension affecting
premature infants. Pediatr Res 2013; 74:186.
13. Clark DA. Times of first void and first stool in 500 newborns. Pediatrics 1977; 60:457.
14. Reid J. Neonatal subgaleal hemorrhage. Neonatal Netw 2007; 26:219.
15. Kilani RA, Wetmore J. Neonatal subgaleal hematoma: presentation and outcome--radiological findings and factors
associated with mortality. Am J Perinatol 2006; 23:41.
16. Kermorvant-Duchemin E, Laborie S, Rabilloud M, et al. Outcome and prognostic factors in neonates with septic
shock. Pediatr Crit Care Med 2008; 9:186.
17. Markham LA, Charsha DS, Perelmuter B. Case report of massive fetomaternal hemorrhage and a guideline for
acute neonatal management. Adv Neonatal Care 2006; 6:197.
Topic 5039 Version 30.0
GRAPHICS
Blood pressure values for extremely preterm infants (gestational age ≤28 weeks) for the first 72
hours of life
Population estimate of blood pressure (BP) values for extremely preterm infants (gestational age ≤28 weeks) by postnatal age in hours.
Dashed line represents the blood pressure estimate while solid line represents the boundaries of the 95% confidence interval. Orange:
systolic BP; blue: mean arterial BP; green: diastolic BP.
Reprinted by permission from Macmillan Publishers Ltd: Journal of Perinatology. Vesoulis ZA, El Ters NM, Wallendorf M, Mathur AM. Empirical estimation
of the normative blood pressure in infants <28 weeks gestation using a massive data approach. J Perinatol 2016; 36:291. Copyright © 2016.
www.nature.com/jp.
Graphic 110007 Version 1.0
Normative blood pressure values for preterm infants over the first 28 days of life
Normative blood pressure trends for preterm infants over the first 28 days of life based on gestational age. Boxes delineate 10 th and 90 th
percentiles, with vertical black marks delineating range.
(A) Blood pressure for neonates born at 28 to 29 weeks gestation.
(B) Blood pressure for neonates born at 30 to 31 weeks gestation.
(C) Blood pressure for neonates born at 32 to 33 weeks gestation.
(D) Blood pressure for neonates born at 34 to 36 weeks gestation.
BP: blood pressure.
Reprinted by permission from: Springer: Pediatric Nephrology. Kent AL, Meskell S, Falk MC, Shadbolt B. Normative blood pressure data in non-ventilated
premature neonates from 28-36 weeks gestation. Pediatr Nephrol 2009; 24:141. Copyright © 2009. https://link.springer.com/journal/467.
Etiology of neonatal shock based on pathogenesis
Classification Disorder
Hypovolemic
Hemorrhage Fetomaternal hemorrhage

Severe bleeding (eg, subgaleal hemorrhage, umbilical cord rupture, internal bleeding)
Twin-twin transfusion
Non-hemorrhage Third spacing from acute intestinal injury (eg, volvulus, necrotizing enterocolitis)
Gastrointestinal fluid loss from congenital chloridorrhea
Polyuria due to congenital diabetes insipidus
Distributive
Sepsis
Non-sepsis Adrenal insufficiency

Hydrops fetalis
Neonatal toxic shock syndrome
Cardiogenic
Cardiomyopathic Myocardial ischemia/hypoxemia

Myocarditis
Congenital cardiomyopathy
Arrhythmogenic Congenital complete heart block

Tachyarrhythmia (eg, SVT, VT)
Structural Congenital heart disease:

Hypoplastic left heart syndrome
Critical aortic stenosis
Critical coarctation of the aorta
Interrupted aortic arch
Obstructed total anomalous pulmonary venous connection
Obstructive
Pulmonary vascular Severe pulmonary hypertension

Pulmonary embolus
Mechanical Tension pneumothorax

Pericardial tamponade
Constrictive pericarditis
SVT: supraventricular tachycardia; VT: ventricular tachycardia.
Common critical* congenital heart defects and their association with cyanosis and dependence upon the ductus
arteriosus
Cyanosis? Ductal-dependent?
Left-sided obstructive lesions
Hypoplastic left heart syndrome Yes Yes
Valvar AS
Critical AS Cyanosis or differential cyanosis ¶ Yes
Moderate to severe AS No No
COA
Critical COA Differential cyanosis ¶ Yes
Moderate to severe COA No No
Interrupted aortic arch Differential cyanosis ¶ (pattern of cyanosis varies Yes

based upon type)
Right-sided obstructive lesions
Tetralogy of Fallot Variable Possibly Δ
Tetralogy of Fallot with pulmonary atresia Yes Yes (unless multiple or large aortopulmonary
collaterals are present)
Pulmonary atresia with intact interventricular Yes Yes

septum
PS
Critical PS Yes Yes
Severe PS No No
Tricuspid atresia Yes Possibly Δ
Severe neonatal Ebstein anomaly Yes Possibly ◊
Parallel circulations
Transposition of the great arteries Yes § Yes
Other
TAPVC Yes No ¥
Large VSD No No
AV canal defect No No
Truncus arteriosus Yes No
AS: aortic stenosis; COA: coarctation of the aorta; PS: pulmonic stenosis; TAPVC: total anomalous pulmonary venous connection; VSD: ventricular septal defect; AV:
atrioventricular; CHD: congenital heart disease; RV: right ventricle; PDA: patent ductus arteriosus.
* Critical CHD refers to lesions requiring surgery or catheter-based intervention in the first year of life. The most common lesions are listed in this table; however, there are
other less common congenital heart lesions that may require intervention within the first year of life.
¶ In these lesions, the upper one-half of the body (preductal) is pink and the lower one-half (postductal) is cyanotic.
Δ Infants with tetralogy of Fallot or tricuspid atresia may have ductal-dependent circulation if there is severe RV outflow tract obstruction (ie, critical pulmonary stenosis or
atresia).
◊ In cases of severe Ebstein anomaly with extreme cyanosis, a PDA may be necessary to maintain pulmonary blood flow until pulmonary vascular resistance drops.
§ Reversed differential cyanosis (ie, oxygen saturation higher in the lower than upper extremity) may occur if there is coexisting coarctation of the aorta or pulmonary
artery hypertension.
¥ Some patients with obstructed TAPVC may require a PDA to maintain systemic cardiac output. However, a PDA may also increase the degree of cyanosis.
Spectrum of hypoplastic left heart syndrome
The spectrum of HLHS.

(A) Mitral atresia and aortic atresia. This is the most extreme form of HLHS. The LV is diminutive or absent. The
ascending aorta is severely hypoplastic. Systemic output is ductal-dependent with retrograde flow in the
ascending aorta.
(B) Mitral stenosis and aortic atresia. The ascending aorta is severely hypoplastic, and systemic output is ductal-
dependent. Depending on the degree of mitral stenosis, LV pressures may be subsystemic, systemic, or even
suprastemic. The degree of ventricular hypertrophy is also variable, but there is usually severe LV systolic
dysfunction.
(C) Mitral stenosis and aortic stenosis. There is considerable variability in the size of the LV, amount of antegrade
flow, and degree of LV systolic and diastolic dysfunction, depending on the degree of obstruction from the
combined effect of the mitral and aortic stenosis. The ascending aorta is larger than in patients with aortic
atresia. There is overlap in the spectrum of these patients and those with critical aortic stenosis, allowing some
patients to be considered for a two-ventricle repair.
HLHS: hypoplastic left heart syndrome; LH: left ventricle.
Reproduced with permission from: Tweddell J, Hoﬀman G, Ghanayem N, et al. Hypoplastic left heart syndrome. In: Moss and
Adams' Heart Disease in Infants, Children and Adolescents: Including the Fetus and Young Adult, 7 th ed, Allen H, Driscoll D,
Shaddy R, et al (Eds), Lippincott Williams & Wilkins, Philadelphia 2008. Copyright © 2008 Lippincott Williams & Wilkins.
www.lww.com.
Critical coarctation of the aorta
Coarctation of the aorta is a narrowing of the descending aorta. The narrowing typically
is at the isthmus, the segment just distal to the left subclavian artery. In critical
coarctation, the narrowing is severe and blood flow to the descending aorta is
dependent on a PDA. When the PDA closes, neonates with critical coarctation develop
heart failure and/or shock. On physical examination, femoral pulses are weak or absent.
PDA: patent ductus arteriosus.
Interrupted aortic arch variants
Interrupted aortic arch is classified according to location of the interruption. In type A, the
interruption occurs distal to the left subclavian arterial origin. In type B, interruption
occurs between the origins of the left common carotid and left subclavian arteries. In type C,
interruption occurs proximal to the origin of the left common carotid artery. Blood flowing in the
arteries proximal to the interruption is fully oxygenated, while blood flowing distal to the
interruption is cyanotic. Type B is the most common form (>50%), followed by type A
(approximately 40%). Type C is rare (<5%).
RS: right subclavian artery; RCC: right common carotid artery; LCC: left common carotid artery; LS: left
subclavian artery; PDA: patent ductus arteriosus.
Modiﬁed from: Celoria GC, Patton RB. Congenital absence of the aortic arch. Am Heart J 1959; 58:407.
Diagram of common types of totally anomalous pulmonary venous connection (TAPVC)
(A) Supracardiac TAPVC to the left innominate vein. The individual pulmonary veins form a horizontal pulmonary venous confluence (HVC) that connects to the left
innominate vein by way of a vertical vein.
(B) Cardiac TAPVC to the coronary sinus (CS).
(C) Infracardiac TAPVC to the portal vein. The pulmonary veins form a vertical confluence that descends below the diaphragm and typically joins the portal vein (PV).
Pulmonary venous blood then drains into the inferior vena cava (IVC) via the ductus venosus or the hepatic sinusoids. The individual pulmonary veins may join the
vertical vein at different levels.
(D) Mixed-type TAPVC. In this example, the left pulmonary veins (LPV) connect to the left innominate vein (LIV), and the right pulmonary veins (RPV) connect with the
CS.
TAPVC: totally anomalous pulmonary venous connection; HV: hepatic vein; LA: left atrium; RA: right atrium; SMV: superior mesenteric vein; SV: splenic vein.
Adapted with permission from: Allen HD, Driscoll DJ, Shaddy RE, Feltes TF. Moss and Adams' Heart Disease in Infants, Children, and Adolescents: Including the Fetus and Young Adult, 7 th
ed, Lippincott Williams & Wilkins, Philadelphia, 2008. Copyright © 2008 Lippincott Williams & Wilkins. www.lww.com.
Blood pressure for full-term neonates over the first 4 days of life
Systolic, mean, and diastolic blood pressure values obtained from healthy term neonates (n = 406) in
the newborn nursery.
Reprinted by permission from Springer: Pediatric Nephrology. Kent A, Kecskes Z, Shadbolt B, Falk MC. Normative
blood pressure data in the early neonatal period. Pediatr Nephrol 2007; 22:1335. Copyright © 2007.
https://www.springer.com/journal/467.
Initial evaluation and management of neonatal shock
O 2 : oxygen; HR: heart rate; BP: blood pressure; BUN: blood urea nitrogen; LFTs: liver function tests; CBC: complete blood count; HSV: herpes simplex virus; CXR: chest
radiograph; ECG: electrocardiogram; ID: infectious disease; RBC: red blood cell; PGE1: prostaglandin E1; CSF: cerebrospinal fluid.
* The amount and rate of infusion may vary depending on the gestational age of the neonate and the suspected type of shock (eg, hypovolemic, distributive, or
cardiogenic). An initial fluid bolus of 10 to 20 mL/kg isotonic crystalloid is appropriate for most neonates presenting in shock. However, fluid boluses should be
administered cautiously in extremely preterm infants (gestational age <28 weeks) or if there is suspicion for a cardiac etiology of shock. Refer to UpToDate's topic on
management of neonatal shock for additional details.
¶ In neonates presenting with shock, HSV infection should be suspected as a possible etiology if the neonate had perinatal exposure to HSV, has exam findings consistent
with HSV (mucocutaneous vesicles), and/or has CSF pleocytosis. In addition, the diagnosis should be considered in neonates with concerning findings that are otherwise
unexplained. These may include seizures, focal neurologic signs, abnormal neuroimaging, sepsis-like illness (fever or hypothermia, irritability, lethargy, respiratory distress,
apnea, abdominal distension, hepatomegaly, ascites), thrombocytopenia, or elevated liver transaminases. Refer to separate UpToDate content on neonatal HSV infection
for additional details on evaluation and treatment of neonatal HSV infection.
Δ Empiric antibiotic therapy is provided to all neonates with shock because sepsis is the leading cause of neonatal shock. Antimicrobial coverage should include agents
active against organisms that most commonly cause neonatal sepsis (ie, group B streptococcus and Escherichia coli). The combination of ampicillin and gentamicin
provides adequate coverage for these organisms until culture results are available. Local antibiotic resistance patterns should also be considered. If there is clinical
suspicion for HSV infection, the empiric antimicrobial regimen should include acyclovir. For additional details on choice of empiric regimen and duration of therapy, refer to
separate UpToDate content on evaluation and treatment of neonatal sepsis and HSV.
◊ An adequate response to therapy is signaled by improvement in pulses, skin perfusion (capillary refill <4 second), and vital signs (including rise in BP if initially
hypotensive, decrease in HR if initially tachycardic, or rise in HR if initially bradycardic), and resolution of acidosis. For additional details on the goals of therapy and
monitoring response, refer to UpToDate's topic on evaluation and management of neonatal shock.
§ Vasoactive agents commonly used in the management of neonatal shock include dopamine, epinephrine, dobutamine, and milrinone. The choice is based in part on the
type of shock (eg, distributive versus cardiogenic). Dopamine is both a vasopressor and inotrope and it is the most commonly used agent in neonates. Dobutamine is
predominantly an inotrope and it is commonly used for management of neonatal cardiogenic shock. For additional details, refer to UpToDate's topic on evaluation and
management of neonatal shock.
¥ Cardiac disease may be suspected based upon the presence of a murmur or gallop, cyanosis, irregular heart rate, weak or absent femoral pulses, a differential in pre-
and post-ductal oxygen saturation, and/or chest radiograph findings (eg, cardiomegaly, pulmonary edema).
‡ Primary adrenal insufficiency (eg, congenital adrenal hypoplasia) may be suspected based on physical examination findings (ambiguous genitalia) and laboratory
findings (hyperkalemia, hyponatremia, hypoglycemia). Secondary or relative adrenal insufficiency may be suspected on the basis of shock that is refractory to fluid boluses
and vasoactive therapy. Refer to separate UpToDate content on adrenal insufficiency and neonatal shock for additional details.
† See separate UpToDate topics for additional details of the initial evaluation and management of neonatal shock, evaluation and management of neonatal sepsis,
management of suspected cyanotic heart disease, and management of arrhythmias in infants.
** In some cases, the source of bleeding may be evident based on the history and physical examination (eg, history of intrapartum hemorrhage, obvious bleeding source
on examination such as a subgaleal hematoma). In cases where the source is unclear, additional evaluation may include cranial ultrasound to evaluate for intracranial
hemorrhage and testing for fetal cells in a maternal blood sample (Kleihauer-Betke assay) to assess for fetomaternal hemorrhage.
Characteristics of different forms of congenital adrenal hyperplasia that may present with atypical genitalia
Atypical genitalia Incidence

Elevated steroid
Disorder Defective gene XY
Adrenal crisis (general
XX (virilization) metabolites
(undervirilization) population)
21-hydroxylase CYP21A2 + – + 1:11,000 to 1:23,000 17-OHP

deficiency
11-beta- CYP11B1 + – Rare 1:100,000 DOC; 11-deoxycortisol

hydroxylase May also have mild
deficiency elevation of 17-OHP
3-beta- HSD3B2 + (mild or absent) + + Rare DHEA; 17-

hydroxysteroid hydroxypregnenolone
dehydrogenase May also have mild
deficiency elevation of 17-OHP
17-alpha- CYP17A1* Typical female + No Rare DOC; corticosterone

hydroxylase genitalia, but no
deficiency puberty
P450 POR ¶ + + + Very rare 17-OHP,

oxidoreductase progesterone, and
deficiency pregnenolone
Lipoid congenital STAR Typical female + (typical female + Very rare None
adrenal genitalia, but no external genitalia)
hyperplasia puberty
P450scc deficiency CYP11A1 Typical female + (typical female + Very rare None
genitalia, but no external genitalia,
puberty sometimes with
clitoromegaly)
CYP21A2: steroid 21-hydroxylase; 17-OHP: 17-hydroxyprogesterone; CYP11B1: steroid 11-beta hydroxylase; DOC: deoxycorticosterone; HSD3B2: 3-beta-hydroxysteroid
dehydrogenase 2; DHEA: dehydroepiandrosterone; CYP17A1: steroid 17-hydroxylase, 17,20 lyase; POR: cytochrome P450 oxidoreductase; STAR: steroid acute regulatory
protein; P450scc: cytochrome P450 side-chain cleavage enzyme; CYP11A1: cytochrome P450 side-chain cleavage gene.
* CYP17A1 encodes an enzyme that catalyzes both the 17-hydroxylase reaction, which forms 17-hydroxysteroids, and the 17,20-lyase reaction, which cleaves 21-carbon 17-
hydroxysteroids to 19-carbon 17-keto androgen precursors.
¶ POR encodes a co-factor for 17-alpha-hydroxylase, 21-hydroxylase, and aromatase.
Eye vesicles in neonate with herpes simplex virus infection
Neonate with HSV infection of the eye, showing characteristic coalescing vesicles on an
erythematous base on eyelid and surrounding skin. Ophthalmologic evaluation of the
eye should also be performed to determine if keratitis or keratoconjunctivitis is
present.
HSV: herpes simplex virus.
Courtesy of Jenny Ravenscroft, MD, and Gail J Demmler-Harrison, MD, Texas Children's Hospital.
Neonatal herpes simplex virus scalp vesicles
Scalp lesions of neonate with skin, eye, and mouth neonatal HSV infection associated
with fetal scalp monitor. Gram-stained smear and bacterial cultures were negative, and
the lesions did not respond to topical and systemic antibiotics. Viral cultures grew HSV
type 2, and the lesions responded to intravenous acyclovir.
HSV: herpes simplex virus
Courtesy of Jane Troendle-Atkins, MD, and Gail J Demmler-Harrison, MD, Texas Children's Hospital.
Neck vesicles in neonate with herpes simplex virus infection
The early untreated skin lesions associated with neonatal HSV infection are
characteristically clear vesicles on an erythematous base, often touching or "kissing,"
or coalesced in groups of vesicles. Culture of the clear fluid aspirated or swabbed from
the vesicles will readily grow HSV in 24 to 48 hours, and slides made from cells scraped
from the base of the lesion will show HSV viral antigens by DFA.
HSV: herpes simplex virus; DFA: direct immunofluorescence assay.
Courtesy of Gail J Demmler-Harrison, MD, Texas Children's Hospital.
Contributor Disclosures
Beau Batton, MD Nothing to disclose Richard Martin, MD Consultant/Advisory Boards: Windtree [Surfactant inhalation trial];
CareFusion in automated oxygenation control trial [STAMINA]. Melanie S Kim, MD Nothing to disclose Carrie Armsby, MD,
MPH Nothing to disclose
Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these are addressed by vetting
through a multi-level review process, and through requirements for references to be provided to support the content. Appropriately
referenced content is required of all authors and must conform to UpToDate standards of evidence.
Conflict of interest policy

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Neonatal shock: Etiology, clinical manifestations, and evaluation

Shock is classified based on the underlying pathophysiologic mechanism:

● Hypovolemic (see 'Hypovolemic shock' below)

Causes of hemorrhagic shock in the neonate include:

● Fetomaternal hemorrhage (see "Spontaneous massive fetomaternal hemorrhage")

• Interrupted aortic arch (figure 5).

• Ventricular tachycardia. (See "Causes of wide QRS complex tachycardia in children".)

• Full-term infants (figure 7)

Decreased peripheral perfusion — Signs of decreased peripheral perfusion include:

• Poor feeding due to lethargy and/or respiratory distress.

• Abdominal distension as a manifestation of ileus.

Other less common laboratory features include:

It generally includes the following interventions:

● Provide respiratory support as needed. (See "Mechanical ventilation in neonates".)

• Prolonged rupture of membrane

• Maternal fever during labor

• Group B streptococcal (GBS) bacteriuria during the pregnancy

• Previous delivery of an infant affected by GBS disease

However, certain findings may point to a specific etiology. These include:

Laboratory and imaging tests

• Metabolic acidosis is observed in most cases primarily due to accumulation of lactate.

• Thrombocytopenia may be suggestive of disseminated intravascular coagulation due to complications from

• Serum bicarbonate is depressed as an indication of metabolic acidosis.

• Physical findings (eg, murmur, gallop, weak or absent femoral pulses)

Consultation with a pediatric cardiologist is suggested if a cardiac etiology is suspected. Echocardiography

SUMMARY AND RECOMMENDATIONS

- Stabilization of the airway and respiratory status

- Initial fluid resuscitation

- Administration of empiric antibiotics

- Brief review of the history (see 'History' above)

- Chest radiograph if there are respiratory symptoms or findings

• Testing for fetal cells in maternal blood (Kleihauer-Betke assay)

Use of UpToDate is subject to the Subscription and License Agreement.

14. Reid J. Neonatal subgaleal hemorrhage. Neonatal Netw 2007; 26:219.

Topic 5039 Version 30.0

Graphic 110007 Version 1.0

BP: blood pressure.

Graphic 110015 Version 5.0

Etiology of neonatal shock based on pathogenesis

Hemorrhage Fetomaternal hemorrhage

Non-sepsis Adrenal insufficiency

Cardiomyopathic Myocardial ischemia/hypoxemia

Arrhythmogenic Congenital complete heart block

Structural Congenital heart disease:

Pulmonary vascular Severe pulmonary hypertension

Mechanical Tension pneumothorax

SVT: supraventricular tachycardia; VT: ventricular tachycardia.

Graphic 110373 Version 1.0

Left-sided obstructive lesions

Hypoplastic left heart syndrome Yes Yes

Critical AS Cyanosis or differential cyanosis ¶ Yes

Critical COA Differential cyanosis ¶ Yes

Moderate to severe COA No No

Interrupted aortic arch Differential cyanosis ¶ (pattern of cyanosis varies Yes

Right-sided obstructive lesions

Tetralogy of Fallot Variable Possibly Δ

Pulmonary atresia with intact interventricular Yes Yes

Critical PS Yes Yes

Tricuspid atresia Yes Possibly Δ

Severe neonatal Ebstein anomaly Yes Possibly ◊

Transposition of the great arteries Yes § Yes