European Journal of Pediatrics

https://doi.org/10.1007/s00431-023-05055-4

REVIEW

Cardiac arrest and cardiopulmonary resuscitation

in pediatric patients with cardiac disease: a narrative
review

Francesca Sperotto1 · Addison Gearhart1 · Aparna Hoskote2 · Peta M. A. Alexander1 ·

Jessica A. Barreto1 · Victoria Habet1 · Eleonore Valencia1 · Ravi R. Thiagarajan1

Received: 15 March 2023 / Revised: 27 May 2023 / Accepted: 2 June 2023

© The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature 2023

Abstract
Children with cardiac disease are at a higher risk of cardiac arrest as compared to healthy children. Delivering
adequate cardiopulmonary resuscitation (CPR) can be challenging due to anatomic characteristics, risk
profiles, and physiologies. We aimed to review the physiological aspects of resuscitation in different cardiac
physiologies, summarize the current recom mendations, provide un update of current literature, and highlight
knowledge gaps to guide research efforts. We specifically reviewed current knowledge on resuscitation
strategies for high-risk categories of patients including patients with single ventricle physiology, right-sided
lesions, right ventricle restrictive physiology, left-sided lesions, myocarditis, cardiomyo pathy, pulmonary arterial
hypertension, and arrhythmias. Cardiac arrest occurs in about 1% of hospitalized children with cardiac disease,
and in 5% of those admitted to an intensive care unit. Mortality after cardiac arrest in this population remains
high, ranging from 30 to 65%. The neurologic outcome varies widely among studies, with a favorable neurologic
outcome at discharge observed in 64%-95% of the survivors. Risk factors for cardiac arrest and associated
mortality include younger age, lower weight, prematurity, genetic syndrome, single-ventricle physiology,
arrhythmias, pulmonary arterial hypertension, comorbidities, mechanical ventilation preceding cardiac arrest,
surgical complexity, higher vasoactive-inotropic score, and factors related to resources and institutional
characteristics. Recent data suggest that Extracorporeal membrane oxygena tion CPR (ECPR) may be a valid
strategy in centers with expertise. Overall, knowledge on resuscitation strategies based on physiology remains
limited, with a crucial need for further research in this field. Collaborative and interprofessional studies are
highly needed to improve care and outcomes for this high-risk population.

What is Known:
• Children with cardiac disease are at high risk of cardiac arrest, and cardiopulmonary resuscitation may be challenging due to
unique characteristics and diferent physiologies.
• Mortality after cardiac arrest remains high and neurologic outcomes suboptimal.
What is New:
• We reviewed the unique resuscitation challenges, current knowledge, and recommendations for diferent cardiac
physiologies. • We highlighted knowledge gaps to guide research eforts aimed to improve care and outcomes in
this high-risk population.

Keywords Cardiopulmonary resuscitation · Cardiac arrest · Cardiac disease · Congenital heart disease ·
Management · Pediatrics

Abbreviations list
CHD Congenital heart disease
CI Confidence interval
CPR Cardiopulmonary resuscitation
Communicated by Gregorio Milani
 ECMO Extracorporeal membrane oxygenation
Eleonore Valencia and Ravi R Thiagarajan. These
authors contributed equally to this work.
ECPR Extracorporeal cardiopulmonary
resuscitation ICU Intensive care unit
Extended author information available on the last page of the LV Left ventricle
article

Vol.:(0123456789) 13
European Journal of Pediatrics
the pre-arrest phase or during CPR (ECPR)—when
OR Odds ratio exper
Qp Pulmonary flow tise and equipment are available [4]. In 2018, these
Qs Systemic flow con siderations were similarly included in an American
ROSC Return to spontaneous circulation Heart Association Resuscitation Statement entirely
RV Right ventricle dedicated to children with cardiac disease [2].
SV Single ventricle With this narrative review, we aim to review the
physiological aspects of resuscitation in different car
diac physiologies, summarize the current
Introduction recommenda tions in the field of cardiac arrest in
children with car diac disease, provide un update of
Children with cardiac disease are at higher risk of current literature, and highlight important knowledge
cardiac arrest (cardiac arrest) compared to healthy gaps to help guide new research efforts.
children [1, 2] as a result of their propensity for Epidemiology and risk factors
myocardial dysfunction, arrhythmias, and
hemodynamic instability. In particular, children with A recent study involving a total of 3,739 hospitals in
congenital heart disease (CHD) often have abnormal 38 states participating in the Kids’ Inpatient Database
circulatory physiology including intracardiac shunts, showed that CPR occurred in about 1% of the
outflow tract obstruction, and common mixing that hospitalized chil dren with cardiovascular disease,
often leads to abnormal pressure or volume loading which corresponds to a 13-fold higher risk of cardiac
condi tions, cyanosis, or congestive heart failure. arrest compared to children without cardiac disease
Taken together, these factors can increase the risk of (odds ratio [OR] 13.8, 95% confi dence interval [CI]
cardiac arrest, espe 12.8–15.0) [1]. A recent meta-analysis estimated that
cially in the post-operative period after surgical about 5% (95% CI 4–6%) of children with a cardiac
correction or palliation. Additionally, cardiorespiratory disease admitted to a pediatric or cardiac intensive
interactions have a greater detrimental impact on the care unit (ICU) experienced at least one episode of
hemodynamics of criti cally ill children with CHD as cardiac arrest during their admission [5]. The higher
compared to healthy children, especially when estimate in the latter study may have resulted from
supporting them with invasive positive pres sure the type of popula tion included (i.e. children receiving
ventilation in the post-operative period. higher level of care in
The anatomical and physiological substrates of the ICU, compared to all hospitalized children).
CHD can also influence the effectiveness and Table 1 summarizes the main studies addressing the
success of the resuscitation strategy itself, especially epidemiology and risk factors for cardiac arrest in this
in single ventricle (SV) patients and in neonates [2]. population according to hospital setting. Common risk
The past few decades have been marked by an fac tors included younger age, lower weight and
intense effort to investigate car prematurity, genetic syndrome, univentricular
diac arrest prevention and resuscitation strategies in physiology, arrhythmias, pulmonary arterial
chil dren with cardiac disease. In 2010, the American hypertension, renal failure, sepsis, seizures,
Heart Association officially identified the pediatric mechanical ventilation or ECMO before cardiac
cardiac patient as a specific high-risk patient for arrest, recent complex cardiac surgery (Society of Tho
cardiac arrest in its resuscitation guidelines, with racic Surgeons mortality category 4–5), and factors
particular reference to the SV patient [3]. In the 2015 related to available resources and hospital
updates, the American Heart Association advised characteristics [1, 5–11]. In the cardiac catheterization
consideration of venoarterial (VA) Extracorporeal laboratory, younger patients and those undergoing
Membrane Oxygenation (ECMO) as part of the interventional procedures were at highest risk [12].
resuscitation strategy in cardiac patients – either in Similarly, in the operating room, neonatal age
 increased the likelihood of intra-operative CPR events cardiac arrest. For example, patients with hypoplastic
[13]. Knowledge of risk factors can guide a priori left heart syndrome undergoing the Stage 1 palliation
identification of patients at the highest risk, so that (i.e. Norwood operation), which entails reconstruction
contingency planning may be put in place in case of of the aorta to provide adequate systemic flow,
clini cal deterioration. removal of atrial restric
tion, and establishment of a source of pulmonary
blood flow with a right ventricle (RV) to pulmonary
Pre‑resuscitation measures artery or a modified Blalock Taussig Thomas shunt,
and resuscitation peculiarities have parallel systemic (Qs) and pulmonary (Qp)
based on physiology circulations. These par
allel circulations may be imbalanced, resulting in a
Single ventricle physiology higher

Patient with SV physiology have the highest risk of

13
European Journal of Pediatrics

Studies addressing risk factors for cardiac arrest in pediatric patients with cardiac disease according to setting

Predictors / Risk factors for Defnition of cardiac N patients with cardiac Exclusion criteria

cardiac arrest

arrest arrest/N total (%)

Multivariable model:

ICD-9 code 3709/ 498,610 (0.7) Not defned

RISK: age<1 yr OR 2.1

(1.8–2.5), Children’s hospital

OR 1.4 (1.2–1.7), small

hospital OR 1.3 (1.01–1.6),

heart failure OR 2.3 (2–2.6),

pulmonary arterial hyperten sion OR 2.0 (1.7–2.3), SV

OR 2.4 (CI 2–2.8), cardio myopathy OR 2.1 (1.8–2.6),

heart transplant (prior) OR 1.4

(1.01–1.9), acute pericarditis

OR 2.4 (1.7–3.2), coronary

artery disease OR 2.8 (2–3.8),

myocarditis OR 2.7 (2.0–3.5),

bacterial endocarditis OR 1.9

(1.2–2.9). PROTECTIVE:

CHD OR 0.6 (0.5–0.8).

Univariate analysis: Any event characterized

2182/ 2182 (100) Patients with “altered

Center volume: OR 1.13 by either pulselessness

code status”

(1.07–1.19). Categori cal analysis: low volume or critically com promised perfusion

center OR 0.67 (0.55–0.82), treated with external

low-medium volume center chest compression and/

OR 0.73 (0.65–0.82), high medium volume center 0.69 or defbrillation

(0.61–0.77), reference: high

volume center.

Multivariable model: NS
Table 1
Population Study design, Author, year

Setting

and period

Hospitalized patients
Hospitalized cardiac Retrospective analysis of Lowry AW et al. (2013)

patients prospective data, KID

[1]
Registry,

Multicenter

(38 USA States)

2000,2003,2006

Hospitalized cardiac Retrospective analysis of Gupta P, et al. (2014)

patients with at least prospective data, VPS

[6]

1 episode of cardiac (NACHIRI) Registry,

arrest Multicenter

(108 USA Centers),

2009–2013

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European Journal of Pediatrics

Predictors / Risk factors for cardiac arrest

Multivariable model:

For SURGICAL patients:

premature neonate OR 5.04

(2.98–8.54), term neonate OR

3.77 (2.54–5.60), infant OR

2.48 (1.69–3.63), underweight

OR 1.56 (1.17—2.08), any

chromosomal abnormality/

syndrome OR 1.36 (1.04–

1.78), any STS preop. risk

factor OR 2.14 (1.68–2.74),

STS mortality category 4–5

OR 3.92 (2.94–5.22).

For MEDICAL patients: prema ture neonate OR 3.15 (1.54–

5.37), medical condition OR

2.20 (1.56–3.34), acute heart

failure OR 2.23 (1.47–3.19),

lactate>3 mmol/L within

2 h of P-CICU admission OR

3.00 (1.86–4.86), MV 1 h post

P-CICU admission OR 2.61

(1.70–3.82)

chest def VT or comprom cy arrest sions

Defnitio Cardiopu
to
n of lmonary com brillation acute ise assisted requiring and/or
cardiopul
pression for respiratorrequiring ventilatio chest
cardiac arrest defbrillati
monary
arrest requiring s and/or pulseless y emergen n leading compres
on

d) Table Author, encounte

1
rs
N year

patients
Intensiv
with
e care
cardiac
unit
arrest/N
setting
total (%)
492/ patients
Exclusio surgical)
15,908
(medical
n criteria
CICU
and PC4

prospecti
Registry,
ve data,
Populati

on None
Multicent
P-CICU
er
pediatric
(23 USA
Study
Retrospe [7] Centers),
,
design ctive 2014–20
g analysis 16
Settin
of Alten
and et al.
(3.1) ***
period (2017)

(continue
13
European Journal of Pediatrics

Predictors / Risk factors for

cardiac arrest

Multivariable model:

RISK: younger age OR 0.73 (CI

0.56–0.96), female OR 1.18

(CI 1.01–1.38), development

disorder OR 1.71 (CI 1.16–

2.51),high complexity opera tions OR 1.81 (CI 1.51–2.16),

MV before surgery OR 2.79

(CI 2.33–3.35), higher PIM-2

score OR 1.28 (CI 1.20–1.36),

SV anatomy OR 1.3 (CI

1.08–1.57), pulmonary arte rial hypertension OR 1.8 (CI

1.4–2.3), acute lung injury

OR 1.50 (CI 1.27–1.77), renal

failure OR 2.92 (CI 2.29–

3.71), chylothorax OR 1.65

(CI 1.11–2.47), arrhythmia

OR 2.69 (CI 2.29–3.16), sei zures OR 3.60 (CI 2.82,-4.59),

brain hemorrhage OR 2.13

(CI 1.27–3.57), MV after sur gery OR 1.52 (CI 1.07–2.16),

hemodialysis catheter in place

OR 1.98 (CI 1.13–3.46).

PROTECTIVE: younger Age

(>28d,<1 years) OR 0.73 (CI

0.56–0.96), higher weight OR

0.73 (CI 0.88–0.00), arterial

line OR 0.58 (CI 0.35–0.96),

attending intensivist OR 0.35

(CI 0.26–0.47)

cardiac patients cardiac arrest/N total (%) Any character

Defnitio
N with arrest
n of event ized
 736/ by either ness or promised with compres defbrillati

critically perfusion external sion and/ on

26,909
pulseless (2.7)
com treated chest or
r
e o I y D di al S ot
1
n C si pr a
E is
U s o c
S cl A li
x r of s s
t ol
e G p u
c
a u e o C st
u r
l d p ct
g at
d
m t iv e
u e s e
y is a e
ry
s si e d e
d ( d
u nt
i o t at
N
e n, al a, d
o A
P . V e in
s C r
n - ( P
i H P
C 2 S
c 2
R rs
I 0 e
g
I)
r C 1 D
n R ) 0
U 6
i ) of
, e
p
la A s
t A gi
e [8] 0
c st M
di d
e u k
at ry o u
o
r of , 9
( t ri c ul
s s
c i c
h u r r
ur
o a R –
m
o gi ti
n et
P e u
c ( g
t r r
nt 2
al
i o o
at c
, p r
n s e
p io 6
at 0
u y p
u ie n, e
e e
e gi ry
nt p 2
d ct 1
l
) s o
a iv nt
T a wi st c
r e n 4
U
a th c
t Setting a
b C a e
n STS-EA
and
l i H
al r CTS
period
 13
European Journal of Pediatrics

Predictors / Risk factors for cardiac arrest Defnition of cardiac arrest N patients with cardiac arrest/N total (%)

Univariate analysis: Cessation of efective

1843/ 70,270
Female sex (p=0.003), lower cardiac mechanical

(2.6)

age (p<0.0001), lower function

weight(p<0.0001), prematu rity (p<0.0001), congenital

disorders (p<0.0001), preop.

LOS (p<0.0001), preop.

MV (p<0.0001), preop.

sepsis (p<0.0001), preop.

shock (p<0.0001), preop.

RI (p<0.0001), preop.

CPR (p<0.0001), CPB

time(p<0.0001), previ ous cardiothoracic surgery

(p<0.0001), STS mortality

category 4–5 (p<0.0001),

STS morbidity category 4–5

(p<0.0001).

Multivariable model: NS

Univariate analysis:

Not defned 29 cardiac arrest (cases)

Lower birth weight (−0.57; (of 343 patients post

95%CI,−0.84,−0.31 kg) cardiac surgery)

and gestational age (−1.5; (8.5)

95%CI,−2.64,−0.40 weeks),

longer preop. ventila tor days (4.1; 95%CI, 1.0,

7.2), worse postop. day 1

peak lactate (4.1; 95%CI,

2.3, 5.9 mmol/L), postop.

day 1 base defcit (−2.9;

95%CI,−5.4,−0.3),

postop. day 1 pH (−0.04;

95%CI,−0.08,−0.01), and

inotrope score (11.6; 95%CI,

3.3, 22.4)

n criteriaoutcome in with CHD y with CPR

Surgery data post CPB,≤ events

the
not cardia 6 week
Cardiac operating
classifed c s of
surgery room
into one surger age.
not y
of the Cases: at
requiring
STS least 1
CPB, NICU
EACTS Populati CPR
patients patients
Mortality on
having post
Categori event,
P-CICU
CPR cardia Control
es, pediatric
preopera c
missing s: no
patients
tively or surger
Exclusio
 d) Table and ctive prospecti Registry, 2007–20 et al. ton,
1
analysis 12 (2011)
Study period Canada)
ve data,
Gupta Single-ce
, 1996–20
design Author, et al. Multicent [10]
[9] nter 05
g r (2014) er Case–co
Settin yea
of ,
STS-CH (97 USA ntrol (Edmon
Retrospe
(continue SD Centers), Hansen

13
European Journal of Pediatrics

Predictors / Risk factors for

cardiac arrest

Univariate analysis:

Younger age (p=0.04), SV

(p<0.01), preop. MV

(p=0–03), PGE1 (p<0.001),

preop. inotropic support

(p=0.04), longer mean

aortic-cross-clamp time

(p<0.0001), longer CPB time

(p=0,0002), longer DHCA

time (P=0,0002),

higher inotropic support dur ing surgery (p<0.0001) and

higher postop. inotropic sup port (p=0.002)

Univariate analysis:

Interventional procedures

(p<0.001), younger age

(p<0.001)

Univariate analysis:

Neonatal age (p<0.001)

CHD congenital heart disease, CPB cardiopulmonary bypass, CPR cardiopulmonary resuscitation, DHcardiac

arrest deep hypothermic circulatory arrest, MV mechanical ventilation, NA not

applicable, NS not signifcant, NICU neonatal intensive care unit, OR odds ratio, P-CICU pediatric cardiac intensive care

unit, PGE1 prostaglandin E1, PICU pediatric intensive care unit,

Defnitio n of cardiac
 Absence apnea, pulses in cardiopul ventila chest tion requiring pression massage cardiover

of and lack major Sudden monary tion compres external or with sion

consciou arteries cessationcirculatio requiring sions for Any chest internal or

of
s ness, of n or external resuscita event com without
palpable cardiac
arrest

N total (%) 44 ted) c

patients 82 resuscita (of surgery

with cardiac tion 1,115 patients)

70 / 7289 40/
cardiac arrest not post
(1.0) 5213
arrest/N (48 CPR, attemp cardia
(7.3) (0.8)

or who had theater toperative, Surgeons,

Exclusion received
received STS Societ SV single
criteria resuscita Preop. preo
CPR in the y of ventricle
Patients tion drugs Not defned perative,
operat ing Thoracic
who only or MV, Not defned Postop. pos

Populati cardiac least 1 Controls DHcardia cardiac 2: c arrest, arrest with CHDcardiac undergoi surgery

on patients surgery. car diac 1: c arrest, arrest; without no undergoi catheteri Patients ng
P-CICU
with CHDCases: atarrest; no Controls cardiac zation
with Patients ng with CHDcardiac
pediatric DHcardia
post

(continued) Table 1

Study design, Author, year

Setting

and period

Case control Suominen et al. (2001)

Single-center (Helsinki, [11]

Finland),

1990–1994

Cardiac catheterization laboratory setting

Retrospective, Odegard KC et al.

cardiac arrestS and car diac arrest Registry,

(2014)

[12]
Single-Center (Boston,
USA),
2004–2009
Operating room setting
Retrospective, Odegard KC et al.
Single-center (2007)
(Boston, USA), [13]
2000–2005
Qp than Qs, especially in the late neonatal and early
infant periods [14]. Additional risks include low
cardiac output syndrome in the early postoperative
period, during which myocardial work and oxygen
demand are increased, and occlusion of the shunt,
the sole source of pulmonary blood flow. Each of
these conditions increases the risk of rapid
cardiovascular collapse with the need for resuscitation
[2–4]. In this cohort, the rate of in-hospital cardiac
arrest has been described to be up to 12.7% in the
acute postoper ative period [9], with a mortality or
transplant rate of 31% at 12 months [15].
Interestingly, the incidence of cardiac arrest was
significantly lower in the presence of a Sano shunt
compared to a BTT shunt [15]. Risk of cardiac arrest
also increase in the presence of a genetic syndrome,
ven
tricular dysfunction, or significant atrioventricular valve
regurgitation (AVVR) [16–18]. Given this unique
physiol ogy, resuscitation in these patients represents
a challenge. Main interventions that may help prevent
a cardiac arrest include: (1) early recognition and
treatment of low car diac output syndrome: correction
of acidosis, reduction of metabolic demand with
sedation and paralysis, adoption of a low mean
airway pressure ventilation strategy, initia tion of
inotropic support, maintenance of an open chest, and
early deployment of VA-ECMO support when there is
significant instability or inadequate response to
medical management; (2) careful balancing of the two
circulations through manipulation of pulmonary and
13
European Journal of Pediatrics
systemic vascular resistance, with avoidance of
hyperventilation and hyper oxygenation or use of
systemic vasodilators [19], and (3) early recognition
and treatment of shunt obstruction, with
anticoagulation or interventional procedures [2, 18]. In
the event of cardiac arrest, it is important to consider
chest compressions in these patients will provide flow
to paral lel circulations, with the majority of flow
directed to the lower vascular resistance district
(generally the lungs) and consequently decreased
flow to other vital organs, includ ing the brain [2].
Adequate chest recoil and target of low mean airway
pressure ventilation can improve filling of the
preload-dependent SV and the consequent
compression related output (Fig. 1) [2].
The second stage of palliation is a superior cavopulmo
nary anastomosis (more commonly the bidirectional
Glenn), which facilitates passive pulmonary blood
flow from the superior vena cava to the pulmonary
arteries and volume unloading of the SV. Completion
of palliative single ventri cle circulation for many
patients includes the Fontan opera tion, in which all
the systemic return (i.e., inferior vena cava) is baffled
to the pulmonary circulation, placing the two cir
culations in series. These patients are preload
dependent, meaning that preload determines
pulmonary blood flow, pulmonary venous return to the
SV, and ultimately systemic cardiac output.
Pulmonary blood flow is highly impacted by
pulmonary vascular resistance and the
transpulmonary gradient. The common atrial pressure
can be affected by AVVR, end-diastolic pressure, and
 atrioventricular dissyn chrony. Therefore,
hypovolemia, increased pulmonary vas cular
resistance, elevated common atrial pressure, ventricu
lar dysfunction, and arrhythmias can result in
Fig. 1 Pre-arrest phase characteristics and cardiopulmonary resuscitation strategies according to cardiac physiology. CPR:
cardiopulmonary resuscitation
13
European Journal of Pediatrics
phase, these patients may benefit from inotropic
support, afterload reduction, and gentle positive
pressure ventilation (when mechanical ventilation is
needed) [2, 20, 21]. In par ticular, ventilatory
strategies that target mild hypercarbia and the
minimal mean airway pressure necessary to maintain
functional residual capacity can be useful to increase
cer ebral and systemic arterial oxygenation [2, 22].
Negative pressure ventilation has been shown to
have some benefit, although it is not available in
many centers [23]. Given their in-series circulations,
chest compression for both Glenn and Fontan
circulations will augment systemic flow but not pul
monary blood flow. This reduction of pulmonary blood
flow will limit oxygenation and preload to the SV,
resulting in low stroke volume and thus cardiac output
with chest com pressions. Optimal compression with
adequate chest recoil to allow preload and pulmonary
blood flow are therefore fundamental (Fig. 1),
although outcomes often remain poor even when
high-quality resuscitation is performed [2, 9, 24].
Right‑sided lesions and right ventricle
restrictive physiology
inadequate pulmonary blood flow and ultimately
compromise cardiac output, increasing the risk of
cardiac arrest. In the prearrest
Patients undergoing correction of right sided lesions
(i.e. pulmonary stenosis, tetralogy of Fallot,
double-outlet RV with pulmonary stenosis, truncus
arteriosus) are at high risk for RV systolic and
diastolic dysfunction, with consequent increased risk
of cardiac arrest. Patients at highest risk are mostly
those who have been exposed to prolonged abnor
mal pressure or volume loading conditions, and those
with residual lesions. Restrictive RV physiology,
characterized by doppler demonstration of persistent
antegrade diastolic blood flow into the pulmonary
artery in late diastole [25], is frequently seen
postoperatively. A poorly compliant RV is associated
with elevated RV end diastolic pressure, and
systemic venous hypertension. Patients are therefore
preload dependent, and lack of adequate preload
reduces RV stroke volume, left ventricle (LV) preload,
and cardiac output [26, 27]. Alternatively, fluid
overload can be deleterious, and especially in the
setting of capillary leak related to cardio pulmonary
bypass [28]. This population is also at a high risk of
developing arrhythmias, such as supraventricular
tachycardia, junctional ectopic tachycardia, and
ventricu
lar tachycardias; the decrease in cardiac output due to
the arrhythmias per se or due to the atrioventricular
dissyn chrony in a dysfunctional RV can lead to rapid
collapse and cardiac arrest. These patients may
benefit from gentle fluid administration to maintain
preload, low mean airway pres sure ventilation
strategies [29, 30], preservation of atrioven tricular
synchrony, initiation of inotropic agents to support RV
systolic dysfunction, and administration of pulmonary
vasodilators to decrease RV afterload [26]. In patients
with RV restrictive physiology, the presence of an
atrial septal communication allows right to left
shunting which may
be helpful to reduce the RV wall stress and preserve
LV preload. Additionally, maintaining an open sternum
for the first few postoperative days may improve
hemodynamics by reducing strain on the RV.
If cardiac arrest occurs, chest compressions may
inad equately fill a restrictive RV with diastolic
dysfunction or lead to obstruction of the RVOT.
Furthermore, chest com pressions can exacerbate
residual pulmonary regurgitation and worsen cardiac
output. High-quality CPR with adequate chest recoil
to optimize RV filling and avoid excessive ven tilation
to maintain low RV afterload are key factors for suc
cessful resuscitation in these patients (Fig. 1).
Patients may also need their chest reopened urgently
to enable open chest cardiac massage and improve
hemodynamics [2].
Left‑sided lesions
Both severe mitral stenosis or regurgitation can cause
left atrial hypertension and pulmonary arterial
hypertension. In the postoperative period, the
reactivity of the previously hypertensive pulmonary
bed – exacerbated by the inflamma
tion secondary to the cardiopulmonary bypass—can
precipi tate pulmonary arterial hypertension crises.
compressions is reduced due to obstruction. In all
these scenarios, high-quality CPR with adequate
compression depth and recoil is crucial (Fig. 1) and,
when ineffective, early consideration of VA-ECMO
may offer the best chance of survival.
Myocarditis and cardiomyopathy
Decompensated heart failure secondary to a baseline
myo cardial disease – such as myocarditis or
cardiomyopathy— can often lead to cardiac arrest [2,
36, 37]. In patients with chronic dysfunction, an
intercurrent illness or procedural sedation may be
Additionally, given the abnormal LV loading
conditions, these patients are at higher risk for
postoperative low cardiac output syndrome and
cardiac arrest [2, 31, 32]. Prevention of arrest includes
close monitoring of cardiac output, left atrial pressure,
and arrhythmias. Afterload reduction, inotropic
support, con trolled positive pressure ventilation, and
arrhythmia control remain key principles for the
management of these patients.
Neonates with critical aortic stenosis present with
fixed obstruction and a hypertrophied or dilated LV
with decreased contractility, especially when
fibroelastosis is present [33, 34]. Elevated LV end
diastolic pressure can translate into left atrial
hypertension and pulmonary edema. These patients
will benefit from a prostaglandin infusion to maintain
systemic perfusion and percutaneous transcatheter
balloon valvuloplasty. Postintervention monitoring of
car diac output using clinical and laboratory
parameters such us heart rate, peripheral perfusion
assessment, urine out
put, and serum lactate, as well as support with
inotropes are important [35]. Persistence of low
cardiac output syndrome despite adequate treatment
of critical stenosis should prompt the assessment of
adequacy of the left-sided structure to sup
port a biventricular circulation, with possible
conversion to a univentricular approach [33, 34].
Cardiac output generated by chest compressions
for severe mitral stenosis or mitral regurgitation is
limited by the elevated left atrial pressure and
pulmonary vascular resistance, which limit effective
pulmonary blood flow and ultimately systemic cardiac
output. Additionally, a stiff or dysfunctional LV may
impair filling. In cases of significant aortic valve
disease, cardiac output generated during chest
13
European Journal of Pediatrics
enough to further decrease the cardiac out put and
induce significant clinical deterioration. This can
manifest as insufficient cardiac output, arrhythmias,
and cardiac arrest. Conversely, patients with
fulminant myocar ditis may present with relatively
preserved systolic function and absence of
cardiomegaly on chest radiography but will have
rapid deterioration. Although the probability of myo
cardial recovery is generally good in children with
fulmi nant myocarditis, prevention of cardiac arrest
and support of any rapidly evolving myocardial
dysfunction is imperative, including early initiation of
mechanical circulatory support to allow full
myocardial recovery [38]. Careful arrhythmia
monitoring, avoidance of electrolyte abnormalities or
aci dosis, end-organ function monitoring, and careful
attention to an excessive or inadequate preload
status are fundamen tal for the prevention of cardiac
arrest in these populations. In hypertrophic
cardiomyopathy, any reduction of preload should be
avoided. In the pre-arrest phase, patients may ben efit
from inotropic support, and positive pressure
ventilation. Finally, VA-ECMO should be considered
early in case of
refractoriness to conventional medical therapies.
[36–38]. Unique challenges in CPR in these patients
include the dramatic reduction in coronary blood flow
and myocardial perfusion with cardiac arrest in the
setting of an already dysfunctional LV, with decreased
likelihood of return of spontaneous circulation
(ROSC). Additionally, in a patient with hypertrophic
cardiomyopathy, adequate ventricular preload and
coronary perfusion pressure to support cardiac output
and myocardial perfusion may be compromised.
Management of tachyarrhythmias can also be
extremely challenging in these patients [36, 37]. Once
cardiac arrest occurs, VA-ECMO should be
considered for early institu tion [36, 37].
Pulmonary Hypertension
Elvated pulmonary vascular resistance and pulmonary
arte rial hypertension is an underappreciated cause of
morbid ity and mortality in children with congenital and
acquired heart disease. An estimated 2% to 5% of all
pediatric
patients following cardiac surgery develop pulmonary
arterial hypertension, and up to 5% experience
pulmonary hypertensive crisis postoperatively,
especially following atrioventricular septal defect
repair (14%) and in patients with trisomy 21 (10%)
[39–41]. The in-hospital mortality rate among patients
with pulmonary arterial hypertension crises is as high
as 20% [40]. Pulmonary arterial hyperten
sion crises can be precipitated by various stimuli
(pain, anxiety, endotracheal tube suctioning, hypoxia,
and aci dosis) and can rapidly lead to acute RV
failure, acute LV preload reduction, and cardiac
arrest. Medical interven tions to prevent and treat
pulmonary arterial hypertension crises include
administration of analgesia, sedation, muscle
13
European Journal of Pediatrics
suture lines or accessory pathways [45]. Prolonged
epi sodes of SVT can cause deterioration of cardiac
function, with onset of congestive heart failure and
relaxants, and avoidance or correction of hypoxia,
hyper carbia, and metabolic acidosis. The use of
inhaled pulmo nary vasodilators (e.g. oxygen
supplementation and inhaled nitric oxide) and
prostacyclin analogs in the acute phase, as well as
the use of phosphodiesterase-5 inhibitors (e.g.
sildenafil) and endothelin receptor antagonists (e.g.
bosen tan) chronically, represent the current
therapeutic approach. Inotropic and vasoactive
pharmacotherapies can be used to support RV
function and maintain adequate coronary perfusion
pressure to avoid ischemia secondary to systemic
hypotension. In severe refractory cases to medical
therapy, pulmonary artery decompression
procedures, such as an atrial septostomy or
placement of a Potts shunt, can signifi cantly reduce
the risk of pulmonary arterial hypertension crises [39,
40, 42, 43].
Conventional resuscitation and medications are
often unsuccessful at restoring pulmonary blood flow,
and thus cardiac output. Correcting possible triggers
for pulmonary arterial hypertension crises and
avoidance of hyperventila
tion are crucial. In cases refractory to high-quality
CPR, rapid mobilization of ECMO may allow for the
best chance of survival by providing a bridge to
heart/lung transplanta tion or spontaneous recovery of
RV function.
Arrhythmias
Patients with cardiac disease are at higher risk of
conduc tion anomalies and arrhythmias compared to
the general population [2]. Children with CHD after
cardiac surgery are at heightened risk of complete
heart block, which is generally not well tolerated
especially in the setting of low cardiac output
syndrome. Temporary pacing is used to restore
atrioventricular synchrony in some cases, and
implantation of permanent pacemaker is considered
for patients with heart block who fail to recover sinus
rhythm within the first postoperative week to 10 days
[44]. Supraventricular tachycardia (SVT) is the most
common tachyarrhythmia in children, and it may be
even more common in patients with CHD given
presence of atrial
collapse. Syn chronized cardioversion or adenosine
should be rapidly considered in hemodynamically
unstable patients. Adeno sine has been shown to be
an effective therapy for SVT; other treatments for SVT
refractory to adenosine include procainamide,
esmolol, or amiodarone [45, 46]. Another arrhythmia
with the potential to induce rapid circulatory failure
and cardiac arrest is junctional ectopic tachycardia
(JET), an automatic rhythm that originates from the
atrio ventricular node or high in the His-Purkinje
system most commonly observed in the early
postoperative period (up to 8% of children after
cardiac surgery) [47–49]. Overdrive pacing,
anti-arrhythmic therapies such as procainamide, and
strategies to decrease oxygen demand using sedation
and analgesia, and temperature control have been
demon strated to be effective treatments [50].
Ventricular arrhyth mias including torsade de pointes
in patients with long QT syndrome, ventricular
tachycardia (VT) and ventricular fibrillation (VF) are
rare in children. Initial resuscitation is targeted to
eitiology, including magnesium sulfate for torsade de
pointes, lidocaine or amiodarone for monomor phic
VT, while direct current cardioversion should be con
sidered early for pre-arrest polymorphic VT [2, 4].
Finally, lidocaine can be considered in pediatric
patients with VF/ pulseless VT in-hospital cardiac
arrest, which seems to be associated with an
increased likelihood of ROSC [51].
ECMO and ECPR
Venoarterial ECMO can provide mechanical circula
tory support during and after resuscitation in children
who experienced cardiac arrest refractory to conven
tional medical therapies or CPR. The use of ECMO in
the form of ECPR is becoming frequent, both in
surgical and medical cardiac patients, specifically for
in-hospital cardiac arrest [2, 52–54]. Recent pooled
data showed that in centers with ECMO expertise,
22% (95%CI: 14–33%) of pediatric patients with
cardiac disease underwent ECPR [5]. In a nationwide
study comparing cardiac patients who did or did not
undergo ECPR, Lasa et al. demonstrated that
patients receiving ECPR had higher odds of survival
to discharge (OR 2.80; CI 2.13–3.69) and survival with
favorable neurological outcome (OR 2.64; CI
1.91–3.64) than patients who received CPR only [55].
This associa tion persisted when analyzed by
propensity score-matched cohorts [55]. Overall, the
use of extracorporeal strategies to support CPR has
been rapidly adopted in many centers and its
utilization is growing consistently as the ECMO
expertise increases worldwide.
Mortality after cardiac arrest
and neurologic outcome
Despite an overall improvement of the survival rate
after in-hospital cardiac arrest in the general pediatric
popula tion in the last decade (threefold
improvement), the mortal ity rate for patients with
cardiac disease who experienced cardiac arrest
remains high (30% to 65%) [2, 7, 9, 56–63]. Among
studies on children admitted to an ICU who experi
enced a cardiac arrest, the pooled in-hospital mortality
was recently calculated at 51% (95%CI: 42–59%) [5].
Mortality also remains high among patients rescued
with ECPR, with reported rates from 44 to 65% [62,
64–69]. Table 2 summa rizes studies addressing the
mortality rates and risk factors for mortality in
pediatric cardiac patients following cardiac arrest.
Main reported risk factors are univentricular physiol
ogy, comorbidities, higher vasoactive-inotropic score,
longer CPR, cardiac arrest during the weekend,
limited nurse expe rience, and – in post-surgical
patients—surgical complexity (Society of Thoracic
Surgeons [STS] mortality category 4–5, or highest
Risk Adjustment for Congenital Heart Surgery-1
[RACHS-1] score). Admission to a CICU was reported
to decrease the risk of mortality [8–10, 57–59, 61–63,
70–73].
Neurologic outcome after cardiac arrest varies
widely among studies. A favorable neurologic
outcome at discharge, generally defined as a
pediatric cerebral performance cat egory (PCPC) of
1–3 [74], has been reported in a variable range from
64 to 95% of survivors [55, 75–78]. Neurologic
dysfunction following ECPR remains high: among sur
vivors of ECPR in the THAPCA trial, about 29% of chil
dren<6 years of age experienced persistent severe
cognitive deficits and 40% experienced at least
moderate neurologic injury at 12 months [75], with
similar results reported from other studies [62, 64–66,
76, 79].
Research gaps and future directions
Research gaps in the field of resuscitation in children
with acquired and congenital heart disease are
numerous. As highlighted in this review, the neonatal
and pediatric car diac population have unique risk
factors, for which spe cific resuscitation strategies are
crucial. A recent large multicenter collaborative study
has shown that a low technology cardiac arrest
prevention bundle consisting in a structured risk
assessment performed by the care team by
physiology was effective in reducing the aggregate car
diac arrest rate by 30% compared with control
hospitals [80]. Education efforts and further quality
improvement studies are needed worldwide to
confirm these results and improve outcomes.
 Researchers are also investigating ded icated the highest risk of cardiac arrest [81, 82]; efforts
resuscitation techniques for SV patients, who have should

13
European Journal of Pediatrics
:
arrest Univariat Recurren able model NA
(0.6–0.9) able
, CPR OR
Predictor associatee t arrest model: RISK: SVPROTEC model:
S cardiac 0.6
(p<0.001 N
s/ risk d OR 1.7 TIVE: NSNA
analysis: ) surgery (0.5–0.8)
Multivari
factor for Mortality (1.2–2.6)
Age<1 yr
NA Multivari prior to Multivari
able OR 0.7
cardiac Late
s
factors cardiac with g to (29.8) EPCR) 739 e: (49.2%) (56.2) e: per 100
Short
for arrest in cardiac setting (362 (39.1) At 34 cardiac
Studies
term No-ROS
mortality pediatric disease Outcome deaths, At discharg (95%CI admissio
addressi At
mortality C*: 929
s
after patients accordin measure 201 discharg e 2083 27–44) n
ng risk
563 At 24 h: discharg

Defnition Pulseles compres procedur charac or external

N
of s in sions>1 e code terized critically chest
patients
cardiac hospital min by either comprom compres
with 2182/
arrest cardiac 3709/ ised sion
cardiac 2182
arrest 498,610 perfusion and/or
(100)
arrest/N 1889/188 pulseless
requiring (0.7) treated defbrillati
9 Any
total (%) 9 (100) chest ness
ICD- with on
event

Exclusio out-of arrest, in NICU arrest implante or defned with status”

n criteria hospital newborn delivery patients, resolved d “altered

Not
cardiac s room, cardiac with defbrillat Patients code
DNR,
Hospitali with cardiac zed car zed car at least 1 arrest

zed car at least 1 arrest diac diac episode

Populatio diac episode patients patients of
n Hospitali
patients of Hospitali with cardiac

Table 2
-
Study design, Set ting and period Author, year

Hospitalized patients

Retrospective Gupta P et al.

analysis of (2016) [60]

prospective

data, GWTG-R

(AHA),

Multicenter

(157 USA Centers)

2000–2010

Retrospective Lowry et al.

analysis of (2013) [1]

prospective data,

KID Registry,

Multicenter

(38 USA States)

2000,2003,2006

Retrospective Gupta et al.

analysis of (2014) [6]

prospective data,

VPS (NACHIRI)

Registry, Multi center

(108 USA Centers),

2009–2013

13
European Journal of Pediatrics

Predictors/ risk factor for

cardiac arrest associated

Mortality

Late
Multivariable model (Out come: survival): NA

RISK for cardiac-surgical:

renal failure OR 0.1 (CI

0.03, 0.3), heart failure

OR 0.5 (CI 0.3, 0.8), beds

n<300 OR 0.4 (CI 0.2,

0.9), teaching hospital,

OR 0.3 (CI 0.09, 0.8),

longer CPR, OR 0.6 (CI

0.5, 0.7)

PROTECTIVE for

cardiac-surgical: Age

1 month-1 year OR

2.7 (CI 1.6, 4.4), Age

1 year-8 year, OR 2.6 (CI

1.4, 4.9), ECPR OR 2.5

(CI 1.3, 4.5)

RISK for cardiac medi cal: cardiac arrest in the

Emergency Department,

OR 0.3 (CI 0.1, 0.6),

metabolic/electrolyte

abnormality OR 0.4 (CI

0.1, 0.96), atropine OR

0.4 (CI 0.2, 0.7), longer

CPR duration OR 0.7 (CI

0.6, 0.8)

PROTECTIVE for cardiac medical: arrhythmia OR

2.6 (CI 1.5, 4.3), airways

compromise OR 8 (CI

2.5, 26), ECPR OR 3.8

(CI 1.4, 5.8)

measure cardiac mortality C* 632 (52.1) ECPR) At

s
arrest/N 1214/ (481 At 24 h discharg
N
Short 1214 death, 594 e 821
patients total (%)
term (100) 151 (48.9) (67.6)
Outcome No-ROS
with

arrest mechani the central unrespon

Defnition Cessatio cal absence pulse, siveness

of n of car activity of apnea,

cardiac diac with palpable and

Exclusio DNR, zed car in at of obstetric patient, by an

out-of diac least 1 cardiac s implante

n criteria shock
hospital, patients episode arrest (medical d
Populatio )
surgical defbrillat
NICU, with the room,
n and
Hospitali newborn delivery or
 d) Table year prospecti Centers),
2
Retrospe ve 2000–20
Study
ctive data, 08
design,
Ortmann GWTG-R
Set ting (AHA),
et al.
Overlap
and analysis Multi
data
period center
of (2011)
(265
Author,
[72] USA
(continue

13
European Journal of Pediatrics

Predictors/ risk factor for cardiac arrest associated Mortality Late

Univariate analysis: NA

Unrepaired lesions

compared with palliated

or completely repaired

lesions (p=0.006),

longer total duration of

resuscitation (p=0.001),

larger number of

drugs (p=0.046), and

more rounds of drugs

(p=0.038)

Univariate analysis: NA

Younger age (p<0.001),

male (p=0.001), lower

weight (p<0.001),

prematurity (p<0.001),

chromosomal/genetic

syndrome (p<0.001),

need for ECMO/VAD

(p<0.001), higher

RACHS-1 category

(p<0.001)
 Univariate analysis: NA

No epinephrine infu sion pre-cardiac arrest

(p=0.02 for CHD medi cal patients, p=0.03 for

surgical patients), no arte rial line pre-cardiac arrest

(p=0.02 for surgical

patients), longer cardiac

arrest duration (p=0.02

for surgical patients),

higher number of epi nephrine doses (p<0.01

for surgical patients)

e e
measure mortality death (no42 C* 41 At 24 h
s outcome 64 (30.1) 49 (54.4)
Cardiac time (33.1) (46.0) 25 (27.8)
Short defned At 23 At
arrest defned): ECPR (18
term discharg No-ROS ECPR) discharg
Outcome related 7/68 – no deaths,

arrest/N (4.3) over 5,947 s)

N patients
total (%) 127/ 127 unique (150/5,947
with 90 (of 150
(100) 211/ 4983 admission =2.5%)
cardiac events

compres Cessation l cardiac

Defnition
Administra sions of car diac activity mas sage CPR≥2 mi
of cardiac
tion or death mechanica requir ing for≥1 min n
arrest
of chest

Exclusion criteria

Cardiac arrests in

the pediatric and

neonatal inten sive care units or

on the ward

Children with

medical cardiac

conditions,

children who

sufered cardiac

arrest follow ing procedures

as cardiac

catheterization,

cardiac arrest

prior to cardiac

surgery, DNR

Multiple events

in the same

patient, events
with incomplete

documentation,

cardiac arrest

outside the

CICU
n
diac at least 1 ia arrest surgery who at least 1
Hospitali
patients episode of related post experien cardiac
zed car P-CICU P-CICU
Populatio with anesthes cardiac cardiac ced
patients patients arrest

(continued) Table 2
-
Study design, Set ting and period Author, year

Retrospective Ramamoorthy

analysis of et al. (2010) [73]

prospective data,

POCA Registry,

Multicenter

(79 USA Centers),

1994–2005

Intensive care unit setting

Retrospective, Dagan et al. 2019

Single-center [58]

(Melbourne,

Australia),

2007–2016

Retrospective, Dhillon et al.

Single-center (2018) [59]

(Texas, USA),

2011–2016

13
European Journal of Pediatrics
Predictors/ risk factor for Outcome measures

cardiac arrest associated

Mortality

Late
Short term mortality

Univariate analysis: NA No ROSC*

Diastolic BP≥25 mmHg 72 (63.7) (39 deaths,

for infants or≥30 mmHg 33 ECPR)

for children (cohort At discharge 56

surgical patients only, (49.6)

p=0.018)

Multivariable model:

NA At discharge 229

RISK: ECMO OR 3.04 (CI (31.1)

2.02, 4.57), SV anatomy

OR 1.60 (CI 1.04, 2.46),

renal failure OR 2.78

(CI 1.70, 4.54), brain

hemorrhage OR 3.09 (CI

1.10, 8.62), hemodialy sis catheter in place OR

3.42 (CI 1.05, 11.15).

PROTECTIVE: younger

age (<28 days) OR 0.47

(CI 0.28, 0.81), presence

of Cardiac PICU OR 0.48

(CI 0.25, 0.92)

Multivariable model:

NA At discharge 910

Low volume centers (49.4)

(<150 case/y) OR 2.0

(1.52–2.63), low-medium

volume centers (150–250

case/y) OR 1.39 (1.09–

1.77), STS mortality

category 1–3 in low and

in medium volume cent ers (OR 2.29 (1.19–4.41)

and 1.88 (1.12–3.18));

STS mortality category

4–5 in low and medium–

low volume centers (OR

2.0 (1.37–2.9) and 1.41

(1.03–1.94))

patients 26,909 perfusion chest (2.6)

with (2.7) treated compres Cessatio

cardiac total (%) Any sion n of

arrest/N 113/ 113 event and/or efective

)
Defnition (100 charac defbrillati cardiac

of
CPR for terized or on mechani

at least by either critically 1843/ cal

cardiac 1 min
with
pulseless comprom 70,270 function
arrest
736/ external
N ness ised

Exclusion criteria

Patients for which

frst compres sion was not

captured on the

waveform data,

or compression

start and stop

could not be

determined

ICU readmission,

lack of surgical

documentation,

surgical closure

of isolated PDA

or surgery not

listed in STS EACTS

Surgery not clas sifed into one of

the STS-EACTS

Mortality Cat egories, missing

outcome
 ion patients surgic ve BP during U with cardiac U with

(medical al) with arteria monitorin line prior CPR patient CHD surgery patien CHD cardiac
PICU or y
Populat and invasi l g and s post ts post surger
P-CICU P-CIC P-CIC

(continued) Table 2
Study design, Set ting and period Author, year

Prospective, Multi center Yates et al. (2019)

[57]

(PICqCPR study,

USA centers, CPC CRN network),

2013–2016

Retrospective Gupta et al. (2016)

analysis of [8]

prospective data,

VPS (NACHRI)

Registry,

Multicenter

(62 USA Centers)

2009–2014

Retrospective Gupta et al. (2014)

analysis of [9]

prospective data,

STS-CHSD Reg istry, Multicenter

(97 USA Centers),

2007–2012

13
European Journal of Pediatrics

Predictors/ risk factor for cardiac arrest associated Mortality Late

Univariate analysis: NA

Lower mean arterial BP

 before the cardiac arrest

(p=0.04)

Multivariable model: NA

Arrest during weekend OR

4.4 (1.2–15.5), experience

of primary nurse<1 yr

OR 9.4 (1.6–55.0),

VIS> =20 OR 6.4

(1.8–22.9)

Multivariable model on all At 1 month

cohort, 11 (37.9)

not on patients with cardiac At 2 years 17

arrest only: (58.6)

Minutes of chest compres sion OR 1.04 (CI 1.01,

1.06)

Univariate analysis: NS At 6 months:

21 (65.6)

Univariate analysis: At 6 months

Lower pre-arrest MAP 20 (58.8)

(p=0.0003), Lower At follow-up

arterial pH (p<0.02), (median

Higher epinephrine doses 21 months)

(p<0.001), Higher bicar bonate dose (p=0.005), 21 (61.8)

Longer CPR duration

p<0.001)

AHA American Heart Association, BP blood pressure, cardiac arrest cardiac arrest, CHD congenital heart disease,

CPB cardiopulmonary bypass, CPR cardiopulmonary resuscitation, DNR do

not resuscitate, ECPR ECMO- cardiopulmonary resuscitation, MAP mean arterial pressure, MV mechanical ventilation,

NA not applicable, NS not signifcant, NICU neonatal intensive care unit,

OR odds ratio, P-CICU pediatric cardiac intensive care unit, PICU pediatric intensive care unit, Preop. preoperative,

Postop. postoperative, RACHS-1 Risk Adjustment for Congenital Heart Sur gery-1, ROSC return of spontaneous

circulation, STS Society of Thoracic Surgeons, SV single ventricle, VAD ventricular assist device VIS vasopressor

inotropic score

Outcome measures

Short term mortality

At discharge 37

(62.7)

No-ROSC*

27 (16.5) (17 death,

10 ECPR)
At discharge 53

(52.0)

No-ROSC*

17 (8 death, 9

ECPR) (58.6)

No-ROSC* 18

(56.2)

(14 deaths, 4 ECPR)

At discharge:

18 (56.2)

No-ROSC

11 (32.4)

At discharge:

20 (58.8)

cardiac P-CICU (4.6) arrest cardiac 32 (38 (5.9)

N
arrest/N 59/529 102 (of admis (cases) patients surgery) events) /
patients 29
total (%) (11.2) 2,230 sion) (of 343 post (8.5) 32
with cardiac (100) 34/ 575

Defnition of cardiac arrest Exclusion criteria

Not defned Not defned

Event requiring Not defned

active chest

compressions for

any duration

Not defned Cardiac surgery

not requiring

CPB, patients

having CPR

preoperatively or

in the operating

room

Cessation of DNR patients

circulation and

respiration that

required CPR

for>2 min

Chest compres sions or the Not defned

absence of a

palpable sponta neous pulse that

was not resolved

with only airway

intervention

*for the purpose of this table, ECPR was considered as no return to spontaneous circulation (no-ROSC)

Population

P-CICU

patients<7 years

of age, post car diac surgery

P-CICU patients

with at least 1

episode of car diac arrest

NICU patients

post cardiac

surgery with

CPB,≤6 weeks

of age. Cases:

at least 1 CPR

event, Controls:

no CPR events

P-CICU patients

with at least 1

episode of car diac arrest

P-CICU patients

with CHD and

age<12 months

post-cardiac

surgery
-
Study design, Set ting and period
Single-center (Teh ran, Iran),

2001–2002

Retrospective,

Single-center (Ann

Arbor, USA)

2006–2008

Case–control,

Single-center

(Edmonton,

Canada)

1996–2005

Retrospective,

Single-center
(Miami, USA),
1995–1997
Retrospective, Sin gle-center (New
York, USA),
1994–1998
(continue Ahmadi et al.
d) Table et al. (20 (2012) Hansen
2 (2011)
13) [73] et al.
Author, [61]
Gaies
year
13
European Journal of Pediatrics
continue in that direction. Overall, specific
resuscitation strategies – with respect to chest
compressions, ventila tion strategies, and
pharmacologic approaches—should be investigated
for children with different physiologies. Further
studies are also needed to investigate the role of
specific drugs for which the level of evidence is low,
such as bicarbonate and calcium [83, 84].
Collaborative stud ies and consensus are needed to
better define, predict, and manage low cardiac output
syndrome. Furthermore, given that current knowledge
on pulmonary arterial hyperten sion, myocarditis, and
cardiomyopathies are still mainly based on adult data,
dedicated pediatric studies are war ranted. Finally,
researchers should continue to investigate the role of
ECMO as resuscitation strategy, particularly
regarding the quality of CPR prior to and during
cannula tion, timing of deployment, and type of circuits
used in this setting.
Conclusions
Children with cardiac disease are at higher risk of
cardiac arrest as compared to healthy children. Main
risk factors include neonatal age, genetic syndrome,
SV physiology, arrhythmias, pulmonary arterial
hypertension, comorbidi
ties, ECMO support before cardiac arrest, and recent
com plex cardiac surgery. Preventive and
resuscitation strategies strictly depend on physiology.
Outcomes following cardiac arrest in this population
remain poor and prevention is piv otal. Knowledge on
physiology-based resuscitation strat egies and its
importance should be widely spread and its impact
investigated in dedicated studies. Collaborative and
interprofessional studies are needed to address
et al.
(2000) Rhodes
(1999)
[62] et al.
[10] [63]
Parra
research the substantial knowledge gaps in this field
to improve care and outcomes for children with
cardiac disease.
Acknowledgments The authors thank Kai-ou Tang, MA,
Medical Illustrator at Boston Children’s Hospital, Harvard
Medical School, for her artistic contribution to Figure 1.
Authors’ contributions: Francesca Sperotto conceptualized
and designed the study, reviewed the literature, collected the
data, and wrote the first draft of the manuscript. Addison
Gearhart contributed to the literature review and to write the
first draft of the manuscript. Jessica A. Barreto and Victoria
Habet contributed to the literature review and data collection.
Ravi R, Thiagarajan and Eleonore Valencia reviewed the first
draft of the manuscript. Aparna Hoskote and Peta Alexander,
as well as all the other authors, commented on previous
versions of the manuscript and contributed with significant
intellectual content. All authors read and approved the final
manuscript.
Funding The authors declare that no funds, grants, or other
support were received during the preparation of this
manuscript.
Statements and declarations
Competing interests The authors have no relevant financial
or non financial interests to disclose.
Ethics approval not applicable.
Consent to participate Not applicable.
Consent to publish Not applicable.
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Authors and Affiliations
82. Stromberg D, Carvalho K, Marsden A et al (2021) Standard
CPR versus interposed abdominal compression CPR in
shunted single ven tricle patients: Comparison using a lumped
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https://doi.org/10.1017/S1047951121003917
83. Cashen K, Reeder R, Ahmed T et al (2022) Feature Articles
Sodium Bicarbonate Use During Pediatric
Francesca Sperotto1 · Addison Gearhart1 · Aparna Hoskote2 · Peta M. A. Alexander1 ·
Jessica A. Barreto1 · Victoria Habet1 · Eleonore Valencia1 · Ravi R. Thiagarajan1
🖂 Francesca Sperotto
francesca.sperotto@childrens.harvard.edu
1
Department of Cardiology, Boston Children’s
13
Cardiopulmonary Resuscitation: A Secondary Analysis of
the ICU-RESUScitation Project Trial. Pediatr Crit Care
Med 23:784–792
84. Dhillon G, Kleinman M, Staffa S et al (2022) Calcium Admin
istration During Cardiopulmonary Resuscitation for
In-Hospital Cardiac Arrest in Children With Heart Disease
Is Associated With Worse Survival—A Report From the
American Heart Associa tion’s Get With The
Guidelines-Resuscitation (GWTG-R) Regis. Pediatr Crit
Care Med 23:860–871
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2
Cardiac Intensive Care Unit, Heart and Lung Directorate,
Great Ormond Street Hospital for Children, NHS
Foundation Trust, London, UK