Topjian et al
RESUSCITATION OF THE PATIENT WITH
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Pediatric Basic and Advanced Life Support: 2020 AHA Guidelines for CPR and ECC
Preoperative and Postoperative Stage I
A SINGLE VENTRICLE
The complexity and variability in pediatric congenital
heart disease pose unique challenges during resuscita-
tion. Children with single-ventricle heart disease typi-
cally undergo a series of staged palliative operations.
The objectives of the first palliative procedure, typically
performed during the neonatal period, are (1) to cre-
ate unobstructed systemic blood flow, (2) to create an
effective atrial communication to allow for atrial level
mixing, and (3) to regulate pulmonary blood flow to
prevent overcirculation and decrease the volume load
on the systemic ventricle (Figure 14). During the second
stage of palliation, a superior cavopulmonary anasto-
mosis, or bidirectional Glenn/hemi-Fontan operation, is
performed to create an anastomosis, which aids in the
redistribution of systemic venous return directly to the
pulmonary circulation (Figure 15). The Fontan is the fi-
nal palliation, in which inferior vena caval blood flow
is baffled directly to the pulmonary circulation, thereby
making the single (systemic) ventricle preload depen-
dent on passive flow across the pulmonary vascular
bed (Figure 16).
Neonates and infants with single-ventricle physiology
have an increased risk of cardiac arrest as a result of (1)
increased myocardial work as a consequence of volume
overload, (2) imbalances in relative systemic (Qs) and pul-
monary (Qp) blood flow, and (3) potential shunt occlu-
sion.1,2 Depending on the stage of repair, resuscitation
may require control of pulmonary vascular resistance,
oxygenation, systemic vascular resistance, or ECLS.
S516 October 20, 2020
Palliation (Norwood/Blalock-Taussig
Shunt or Sano Shunt)
Recommendations for the Treatment of Preoperative and
Postoperative Stage I Palliation (Norwood/Blalock-Taussig Shunt or
Sano Shunt)
COR LOE Recommendations
1. Direct (superior vena cava catheter) and/or
indirect (near infrared spectroscopy) oxygen
saturation monitoring can be beneficial to
2a B-NR
trend and direct management in the critically
ill neonate after stage I Norwood palliation or
shunt placement.3
2. In the patient with an appropriately restrictive
shunt, manipulation of pulmonary vascular
resistance may have little effect, whereas
lowering systemic vascular resistance with the use
2a C-LD
of systemic vasodilators (α-adrenergic antagonists
and/or phosphodiesterase type III inhibitors), with
or without the use of oxygen, can be useful to
increase systemic oxygen delivery (DO2).4,5
3. For neonates prior to stage I repair with
pulmonary overcirculation and symptomatic
low systemic cardiac output and delivery of
oxygen (DO2), it is reasonable to target a Paco2
2a C-LD of 50–60 mm Hg. This can be achieved during
mechanical ventilation by reducing minute
ventilation or by administering analgesia/
sedation with or without neuromuscular
blockade.6,7
4. ECLS after Stage I Norwood palliation can be
2a C-LD
useful to treat low systemic DO2.8,9
5. In the situation of known or suspected shunt
obstruction, it is reasonable to administer
oxygen, vasoactive agents to increase shunt
2a C-EO
perfusion pressure, and heparin (50–100 U/kg
bolus) while preparing for catheter-based or
surgical intervention.2
Two diagrams of
a heart, showing
aortic arch recon-
struction using a
Norwood repair.
Figure 14. Stage I palliation for single One diagram
ventricle with a Norwood repair and either shows a Blalock-
Taussig Shunt
a Blalock-Taussig Shunt from the right from the right
subclavian artery to the right pulmonary subclavian artery
to the right
artery or a Sano shunt from the right ven- pulmonary
tricle to pulmonary artery. artery; the other
diagram shows
a Sano shunt
from the right
ventricle to the
pulmonary artery.
Circulation. 2020;142(suppl 2):S469–S523. DOI: 10.1161/CIR.0000000000000901