Cardiopulmonary Resuscitation (CPR) in Children With Heart Disease

31 

Cardiopulmonary Resuscitation (CPR) in Children With Heart Disease ELIZABETH A. HUNT, MD, MPH, PH D; TIA T. RAYMOND, MD; KIMBERLY WARD JACKSON, MD; BRADLEY S. MARINO, MD, MPP, MSCE; DONALD H. SHAFFNER, MD

C

ardiac arrest is a relatively rare phenomenon in children. Although the overall incidence is rare, cardiac arrest represents a clinically important event often resulting in death or poor neurologic outcome. Out-of-hospital cardiac arrest (OHCA) is estimated to occur approximately 16,000 times per year (8 to 20 per 100,000 children annually) in the United States.1 In-hospital cardiac arrest (IHCA) has been variably estimated to occur in 5000 to 10,000 children per year, or in 0.77 per 1000 admissions (or 77 per 100,000 children). Thus IHCA occurs at least four times more frequently than OHCA.2-5 Higher survival to discharge after in-hospital6-12 compared with out-of-hospital13 arrest rates have been attributed to differences in cause of arrest and more rapid recognition and treatment by skilled caregivers in the in-hospital setting.14 Among hospitalized children, cardiac arrest is reported in 2% to 4% of all children admitted to a pediatric intensive care unit (ICU)6,15,16 and in approximately 3% to 6% of children admitted to a cardiac ICU.16,17 Cardiopulmonary resuscitation (CPR) is performed in 7 per 1000 hospitalizations of children with acquired and congenital cardiovascular disease, a rate greater than 10-fold that observed among hospitalized children without cardiovascular disease.5 Recently published data from the Pediatric Cardiac Critical Care Consortium (PC4) demonstrated a cardiac arrest rate of 3.1% among 15,908 cardiac ICU encounters (6498 medical and 9410 surgical) in 23 centers. Observed (unadjusted) cardiac ICU cardiac arrest prevalence varied from 1% to 5.5% with wide variation in cardiac arrest rates per 1000 cardiac ICU days among the 23 centers (1.1 to 10.4).17 Survival from IHCA in infants and children has significantly improved over the past four decades, from approximately 9% in the 1980s to at least 14.3% in 2000, and most recent data reports overall survival has increased nearly threefold during the past decade to 43%.6-12,18 This significant improvement in survival is despite an increase in cardiac arrests resulting from nonshockable rhythms. These improvements have been facilitated by improvements in systems and processes to prevent cardiac arrest and improvements in CPR quality, resulting in higher rates of survival during the acute resuscitation period.19-21 Notably, these improved survival rates were not accompanied by increased rates of significant neurologic disability among survivors.18 A number of factors have likely played an important role in achieving these trends. First, clinical practice guidelines over the past decade have emphasized

several aspects of the acute resuscitation chain of survival.22 These include greater vigilance and closer monitoring, which may have resulted in shorter response times. In addition, recent pediatric in-hospital publications have demonstrated a higher proportion of patients located in a monitored unit or an ICU at the time of cardiac arrest, which may have allowed earlier recognition of cardiopulmonary compromise and prompt initiation of resuscitation efforts.18,23 Additionally, patients located in an ICU may have patient management/interventions in place at the time of the arrest (e.g., central venous and arterial access, improved cardiac monitoring) and/or the presence of team members experienced with resuscitation of the cardiac patient, which may contribute to improved survival after cardiac arrest. Additionally, specialized cardiac arrest processes, including easier access to extracorporeal life support during resuscitation (extracorporeal CPR [ECPR]), may improve survival after cardiac arrest when it occurs in the ICU.24 Increased participation in hospital-specific quality improvement efforts like the American Heart Association’s (AHA’s) Get With the Guidelines—Resuscitation (GWTG-R) registry may also have led to improved survival over time. Improved patient outcomes have also been shown with the use of routine mock codes in pediatric hospitals, audiovisual feedback during resuscitation, and postevent debriefing.25-27 Additional resuscitation strategies that may have improved outcomes include earlier recognition and management of at-risk patients, better adherence to resuscitation algorithms, improved coordination between code team members, greater emphasis on quality of resuscitation (e.g., high-quality chest compressions with minimal interruptions), and postresuscitation care (e.g., multidisciplinary care).8,15,16,28,29

Risk Factors The cardiac diseases associated with sudden cardiac death can be divided into previously recognized or unrecognized diseases (Table 31.1).30 Children with repaired congenital heart disease (CHD) constitute the largest group among the patients with previously recognized heart disease. The group with previously unrecognized heart disease is more challenging because cardiac arrest may be the presenting sign of the abnormality. Children with unrecognized heart disease may have underlying structural heart disease such as 379

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TABLE 31.1  Underlying Cardiac Diagnoses in Children Presenting With Sudden Cardiac Death Patients at Risk for

Out-of-Hospital Cardiac Arrest From Cardiovascular Disease (Sudden Cardiac Death) WITH PREVIOUSLY RECOGNIZED HEART DISEASE

WITH PREVIOUSLY UNRECOGNIZED HEART DISEASE

Congenital

Acquired

Structural Heart Disease

No Structural Heart Disease

Tetralogy of Fallot

Kawasaki syndrome

Hypertrophic cardiomyopathy

Long QT syndrome

Hypoplastic left heart syndrome

Dilated cardiomyopathy

Congenital coronary artery abnormalities

Wolff-Parkinson-White syndrome

Transposition of the great arteries

Myocarditis

Arrhythmogenic right ventricular dysplasia

Primary ventricular tachycardia and ventricular fibrillation

Myocarditis

Commotio cordis

Aortic stenosis Single-ventricle palliative procedures—Fontan, Glenn, hemi-Fontan

Primary pulmonary hypertension

Marfan syndrome Eisenmenger syndrome Congenital (or postoperative) heart block Modified from Berger S, Dhala A, Friedberg DZ. Sudden cardiac death in infants, children, and adolescents. Pediatr Clin North Am. 1990;46:221-234.

hypertrophic cardiomyopathy, coronary anomalies, or arrhythmogenic right ventricular dysplasia, or nonstructural heart disease with conduction system abnormalities such as long QT syndrome, Wolff-Parkinson-White syndrome, primary ventricular tachycardia/ fibrillation, or commotio cordis. Commotio cordis is an underappreciated syndrome in which low-energy impact to the chest wall leads to ventricular fibrillation because of impact during the vulnerable period just before the peak of the T wave.31 Many of the entities in the group with previously unrecognized heart disease have an underlying genetic predisposition. Retrospective analysis of pediatric IHCAs entered into the administrative Kids’ Inpatient Database (KID) revealed survival after cardiac arrest was higher among pediatric surgical cardiac patients (52%) than among pediatric medical cardiac patients (43%); however, children with single-ventricle disease had a lower survival rate (35%) than did children with other forms of cardiovascular disease (45%).5 Variables associated with an increased risk of cardiac arrest on multivariate analysis in the KID inpatient database included age less than 1 year, heart failure, myocarditis, single-ventricle physiology, and coronary artery pathology, whereas patients undergoing cardiac surgery had decreased risk of cardiac arrest.5 Single-ventricle patients demonstrated a fivefold increased odds of arrest over CHD patients with a biventricular circulation, and they were the only group with increased odds of death after CPR, even after adjusting for age, hospital characteristics, and comorbidities.5 More recent data on the epidemiology of cardiac arrest in cardiac ICUs were explored by the PC4 registry, which analyzed 15,908 medical and surgical encounters from 23 North American centers. Medical encounters had a 50% higher rate of cardiac arrest compared with surgical encounters. On multivariable analysis, prematurity, neonatal age, any Society of Thoracic Surgeons preoperative risk factor, and Society of Thoracic Surgeons–European Association for Cardio-Thoracic Surgery mortality category 4 or 5 had the strongest association with surgical encounter cardiac arrest. In medical encounters, independent cardiac arrest risk factors were acute heart failure, prematurity, lactic acidosis greater than 3 mmol/

dL, and invasive ventilation 1 hour after admission. Return of spontaneous circulation occurred in 64.5%, and ECPR was used in 27.2% of cardiac arrest events. Unadjusted survival was 53.2% in encounters with cardiac arrest versus 98.2% without cardiac arrest. Medical encounters had lower survival after cardiac arrest (37.7%) versus surgical encounters (62.5%), with the Norwood surgical population noted to have less than half the survival after cardiac arrest (35.6%) compared with all others.17 The AHA’s GWTG-R multicenter registry found significantly improved hospital survival after resuscitation from an IHCA in children who have undergone cardiac surgery compared with children without cardiovascular disease. Among the cardiac patients the survival to hospital discharge in those with surgical cardiac disease (37%) was significantly higher compared with patients with medical cardiac disease (28%) (adjusted odds ratio, 1.8; 95% confidence interval [CI], 1.3-2.5; P < .001), and non–cardiac disease (23%) (adjusted odds ratio, 1.8; 95% CI, 1.4-2.4; P < .001).32 Additionally, a report from GWTG-R found that children with cardiac disease were more likely to survive to hospital discharge compared with children without cardiac disease when ECPR was used.33 The most recent multi-institutional data from the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) suggests a 2.6% cardiac arrest rate for postoperative patients and a mortality rate of 49.4% among those having cardiac arrest.34 This publication and previous literature have identified younger age, prematurity, genetic syndromes, preoperative comorbid conditions, and increased surgical complexity as risk factors for cardiac arrest in patients following cardiac surgery.16,34-36 As hospitals increasingly develop dedicated cardiac ICUs to optimize the care of this complex patient population, practitioners must understand the unique cardiac arrest incidence and outcome data of this population to improve quality of care and clinical outcomes. Specific causes of arrest, the availability of specialized invasive monitoring, and access to interventions and management subsequent to the arrest may explain the differences in survival in these three large retrospective studies.



CHAPTER 31  Cardiopulmonary Resuscitation (CPR) in Children With Heart Disease

Overview of Guidelines for Cardiopulmonary Resuscitation When managing a cardiac arrest patient in the cardiac ICU, it is important for all team members to have a shared mental model of the goals of the resuscitation. The most common approach to defining the goals of the resuscitation would be to use regionally published, evidence-based resuscitation guidelines such as the AHA, European Resuscitation Council, or Australian and New Zealand Committee on Resuscitation guidelines for basic life support (BLS) and pediatric advanced life support (PALS). These guidelines should be used as the default, and then the team can consider alterations to personalize the resuscitation based on specific aspects of the size of the individual patient, disease population, or situation not yet covered in the standard guidelines, such as how to approach arresting ICU patients with an open chest, presence of hemodynamic monitoring information from central venous lines, arterial lines, near-infrared spectroscopy (NIRS), and special considerations based on a patient’s anatomy (e.g., single ventricle). Until 2015 the AHA updated their CPR guidelines every 5 years for both BLS and PALS; it has now shifted to a rolling approach (i.e., with the intent to perform updated reviews as new literature is published). In 2010 the guidelines most notably changed the traditional sequence of basic CPR from A-B-C (airway, breathing, circulation) to C-A-B (circulation, airway, breathing) to decrease time to chest compressions and reduce decreased perfusion time.37 Differences in time to return of spontaneous circulation (ROSC) and clinical outcomes with this change are yet to be determined; however, time to chest compressions and to starting ventilation strategies have both been shown to be decreased with the C-A-B sequence.38 New to the 2015 AHA pediatric BLS guidelines is the idea of compression-only CPR—particularly in the setting of a witnessed cardiac arrest that is presumably nonasphyxial in nature or when a provider is unable or unwilling to perform assisted breaths and most likely in the out-of-hospital setting. When a witnessed sudden cardiac death event occurs, there should be high suspicion for unrecognized heart disease as the cause (see Table 31.1). In these patients, compression-only CPR has been shown to have better outcomes than no CPR at all. However, in all cases, compression-only CPR has been shown to have worse survival compared with conventional CPR for children. 39 The AHA pediatric BLS guidelines recommend conventional CPR for all pediatric cardiac arrests (regardless of cause) when bystanders are able and willing. In 2017 the first updated, rolling review of this topic reviewed the most recent evidence supporting this but again stated if rescuers were not willing or able to provide mouth to mouth, to perform compression-only CPR rather than not performing CPR.40 The parameter goals for the AHA 2015 pediatric BLS guidelines can be seen in Table 31.2.19,41-44 The AHA 2015 updated PALS CPR algorithm can be seen Fig. 31.1.41 A focus of the guidelines is to standardize and optimize CPR quality, specifically adequate compression rate and depth. For all ages the recommended compression rate is 100 to 120 per minute (same as for adults). For infants and children (up to onset of puberty), adequate depth of compressions is at least one-third of anterior-posterior chest diameter (typically 4 to 5 cm in children) with full recoil of the chest between compressions. The compressor should rotate every 2 minutes to limit provider fatigue, and interruptions in chest compressions should be minimized in an attempt

381

TABLE 31.2  The 2013 and 2015 American Heart

Association Recommendations for Metrics of CPR Performance by the Provider Team

Parameter

Infants

Children

Adults a,b

Chest compression rate

100-120 compressions per min

Chest compression depth

≥ 13 AP diameter or ≈38 mma,b

Chest compression fraction

Minimize interruptionsb, >80%a

Chest compression release

Allow full recoila,b

Ventilation rate

10a, <12 breaths per minuteb

Ventilation volume

Minimal chest risea

Epinephrine interval

3-5 mina,b

Perishock pauses

Minimize number and length of preshock and postshock pauses, <10 s eachb

≥ 13 AP diameter or ≈50 mma,b

≥50 mm, <60 mma,b

a

Recommendation from 2013 AHA consensus statement.19 Recommendation from the 2015 AHA resuscitation guidelines.41 AP, Anteroposterior. Supporting references 19, 30, 41, 43, and 44. b

to maximize perfusion to vital organs and to maintain coronary perfusion pressure (CoPP).37 Recent literature has shown that early placement of an advanced airway during CPR does not improve survival or neurologic outcome.45,46 Because of the lack of definitive evidence, the current PALS algorithm suggests one consider advanced airway placement (either a laryngeal mask airway or endotracheal intubation) during resuscitation if an experienced health care provider is present, with focus on minimizing interruptions in chest compressions. When an advanced airway is present, a ventilation rate of one breath approximately every 6 seconds (10 breaths/min) is recommended with adequate, but not excessive, chest rise.41 Hyperventilation and overdistention of the lungs should be avoided because this could impede venous return to the thoracic cavity. Presence of an advanced airway also allows continuous end-tidal carbon dioxide (ETCO2) monitoring, which can assist in guiding quality chest compressions, though specific values to guide therapy have not been established in children. This is discussed in detail later in the chapter. If no advanced airway is present, effective bag-mask ventilation should be performed at a rate of 2 breaths per 15 chest compressions (or 2 breaths for every 30 compressions in the setting of one health care provider) with focus on adequate, but not excessive, chest rise.37

Drug Therapy Vasopressors Theoretically, vasopressors are indicated in cardiac arrest to cause systemic vasoconstriction (increase blood pressure) to increase coronary blood flow, providing perfusion to the myocardium,

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PART III

1

CPR Quality

Start CPR • Give oxygen • Attach monitor/defibrillator

• Push hard (≥1/3 of anteroposterior diameter of chest) and fast (100–120/min) and allow complete chest recoil. • Minimize interruptions in compressions. • Avoid excessive ventilation. • Rotate compressor every 2 minutes, or sooner if fatigued. • If no advanced airway, 15:2 compression-ventilation ratio.

Yes

No

Rhythm shockable?

2 VF/pVT

Asystole/PEA

9

Shock Energy for Defibrillation First shock 2 J/kg, second shock 4 J/kg, subsequent shocks ≥4 J/kg, maximum 10 J/kg or adult dose

3 Shock 4

Drug Therapy CPR 2 min • IO/IV access

No

Rhythm shockable? Yes 5

Shock 10

6 CPR 2 min • Epinephrine every 3–5 min • Consider advanced airway

CPR 2 min • IO/IV access • Epinephrine every 3–5 min • Consider advanced airway

No

Rhythm shockable?

Rhythm shockable?

Yes

Yes 7

Shock

8

No

Advanced Airway • Endotracheal intubation or supraglottic advanced airway • Waveform capnography or capnometry to confirm and monitor ET tube placement • Once advanced airway in place, give 1 breath every 6 seconds (10 breaths/min) with continuous chest compressions

Return of Spontaneous Circulation (ROSC)

11

CPR 2 min • Amiodarone or lidocaine • Treat reversible causes

• Epinephrine IO/IV dose: 0.01 mg/kg (0.1 mL/kg of 1:10 000 concentration). Repeat every 3–5 minutes. If no IO/IV access, may give endotracheal dose: 0.1 mg/kg (0.1 mL/kg of 1:1000 concentration). • Amiodarone IO/IV dose: 5 mg/kg bolus during cardiac arrest. May repeat up to 2 times for refractory VF/pulseless VT. • Lidocaine IO/IV dose: Initial: 1 mg/kg loading dose. Maintenance: 20–50 mcg/kg per minute infusion (repeat bolus dose if infusion initiated >15 minutes after initial bolus therapy).

CPR 2 min • Treat reversible causes

• Pulse and blood pressure • Spontaneous arterial pressure waves with intra-arterial monitoring

Reversible Causes No

Rhythm shockable?

Yes

12

© 2015 American Heart Association

• Figure 31.1

• Asystole/PEA Æ 10 or 11 • Organized rhythm Æ check pulse • Pulse present (ROSC) Æ post-cardiac arrest care

Go to 5 or 7

• • • • • • • • • • •

Hypovolemia Hypoxia Hydrogen ion (acidosis) Hypoglycemia Hypo-/hyperkalemia Hypothermia Tension pneumothorax Tamponade, cardiac Toxins Thrombosis, pulmonary Thrombosis, coronary

  Pediatric advanced life support cardiac arrest algorithm as updated in the American Heart Association 2015 guidelines. (Reprinted with permission. Circulation. 2015;132:S526-S542. Copyright 2015 American Heart Association, Inc.)



CHAPTER 31  Cardiopulmonary Resuscitation (CPR) in Children With Heart Disease

and increase myocardial contractility of the stunned myocardium. Despite the fact that epinephrine is the key drug used during the management of pediatric cardiac arrest, and the first drug listed in every PALS algorithm, there is little human evidence that vasopressors are effective in helping to achieve return of circulation or impact clinical outcomes. One adult study did show that epinephrine administration was associated with increased return of circulation in OHCAs,47 but no such pediatric data are available. Currently the only recommended vasopressor in pediatric cardiac arrest is epinephrine (0.01 mg/kg intraosseous [IO]/intravenously [IV] or 0.1 mg/kg per endotracheal tube [ETT]) every 3 to 5 minutes.41

Antiarrhythmic Therapy When a patient experiences pulseless ventricular tachycardia or ventricular fibrillation, CPR and defibrillation (2 to 4 J/kg) should be initiated per the PALS algorithm (see Fig. 31.1). If defibrillation is not successful in converting to a perfusing rhythm, amiodarone (5 mg/kg IO/IV bolus) or lidocaine (1 mg/kg IO/IV bolus, 2 mg/ kg per ETT) may be given in addition to repeat defibrillation attempts.41 In a recent systematic review, lidocaine and amiodarone were found to be comparable, so either is acceptable in this setting.48

Sodium Bicarbonate In the past the use of sodium bicarbonate during CPR had been a common practice in an attempt to buffer the metabolic acidosis associated with poor perfusion because acidemia impairs myocardial contraction and cardiac output. In recent years its use has become controversial because some studies have associated its use with worsened outcomes.49 The administration of sodium bicarbonate during CPR is now recommended only during resuscitation for cardiac arrest specifically related to severe metabolic acidosis, hyperkalemia, and tricyclic antidepressant overdose.

383

Resuscitation profiles for the structurally normal heart and the single-ventricle palliated states are delineated in Table 31.3,50 in which the pathway of each circulation and the impact of chest compressions, chest recoil, and positive-pressure ventilation are shown.

Risk Factors for Cardiac Arrest and Death Anatomic, hemodynamic, and other comorbid factors that contribute to hemodynamic compromise and early death in the infant with single-ventricle physiology include (1) the presence of hypoplastic left heart syndrome (HLHS), pulmonary atresia with intact ventricular septum with right ventricle–dependent coronary circulation, and total anomalous pulmonary venous connection; (2) reduced cardiac function and hemodynamically significant atrioventricular and/or semilunar valve regurgitation; and (3) prematurity and the presence of a genetic disorder or syndrome.51-54 Neonates with a univentricular physiology are at higher risk of cardiopulmonary arrest secondary to (1) increased cardiac work from volume overload, (2) unbalanced systemic and pulmonary blood flow (i.e., elevated pulmonary-to-systemic blood flow ratio [Qp:Qs]), and (3) shunt thrombosis.55-57 The risk of cardiac arrest remains high until the superior cavopulmonary anastomosis is completed.58-60 The incidence of IHCA in patients with HLHS after stage I Norwood palliation is higher after modified BlalockTaussig shunt (MBTS) than after right ventricle to pulmonary artery shunt (RVPAS).59-61 However, there is no difference in hospital mortality based on shunt type.53,62,63 The Single Ventricle Reconstruction Trial revealed that one-third of infants after stage I Norwood for HLHS died or underwent heart transplantation at 12-month follow-up,59 with a nontrivial incidence of cardiac arrest and mortality occurring between 30 days after the stage I palliation and the time of the superior cavopulmonary anastomosis (CPA) procedure (interstage period). Thus the interstage period is considered a prearrest state that warrants active monitoring and intervention to improve survival.56

Calcium Routine calcium administration is not recommended during resuscitation unless there is documented hypocalcemia, calcium channel blocker overdose, hypermagnesemia, or hyperkalemia. Calcium chloride or calcium gluconate may be used; however, calcium chloride should be given through central venous access when possible (i.e., to avoid infiltrates).41 However, in a true cardiac arrest situation, risk-benefit analysis would suggest the risk of infiltrate should not outweigh rapid administration if clinically indicated. For a full description of initial dosing of drugs commonly used in the treatment of low cardiac output syndrome in an attempt to avoid a cardiac arrest and during treatment of a cardiac arrest see the Pharmacology: Typical Doses and Indications table in the AHA scientific statement on CPR in infants and children with cardiovascular disease.50

Special Considerations for Cardiopulmonary Resuscitation in Patients With Underlying Cardiac Disease Single-Ventricle Resuscitation The resuscitation of the child with single-ventricle anatomy is dependent on anatomic factors and cardiopulmonary interaction.

Resuscitation in the Patient With Patent Ductus Arteriosus or Shunted Single-Ventricle Physiology The prearrest state usually includes some combination of lactic acidosis, intestinal hypoperfusion and feeding intolerance, renal insufficiency, or ST wave changes on ECG indicative of coronary ischemia.56,57 Qp:Qs is increased in patients with large shunt size relative to body weight. In the neonate with significantly elevated Qp:Qs, increases in systemic vascular resistance (SVR) may result in rapid deterioration with extreme pulmonary overcirculation and shock (“SVR crisis”).64-66 Hemodynamic compromise in a shunt-dependent physiology can typically be reversed with inotropic support, preload modification, mechanical ventilation and sedation/ analgesia, manipulation of SVR and pulmonary vascular resistance (PVR), and anticoagulation if shunt obstruction is suspected. Recognition and Management of Shunt Obstruction.  Decreased systemic arterial oxygen saturation after the Stage I Norwood palliation may result from insufficient pulmonary blood flow, intrapulmonary shunting and pulmonary venous desaturation, and/or diminished mixed venous saturation (SvO2). Insufficient pulmonary blood flow may result from mechanical obstruction of the shunt, elevated PVR, inadequate shunt perfusion pressure, or from pulmonary vein stenosis or restrictive atrial communication.62,67

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TABLE 31.3  Resuscitation Profiles for the Structurally Normal Heart and Those That Have Undergone Single-

Ventricle Palliation Circulation Description

Circulation of Blood

Chest Compressions

Chest Recoil

Structurally normal heart

Two-ventricle series circulation without heart disease

Systemic veins— lungs— Pulmonary veins— body

1. RV compression results in PBF 2. LV compression results in SBF

Increases the transthoracic gradient from the systemic veins to the RA increasing RV filling

Decreases the transthoracic gradient from the systemic veins to the RA decreasing RV filling

Stage I Norwood or shunted physiology

Single-ventricle parallel circulation with shuntdependent PBF

Systemic veins—single V— Lungs (via shunt) or body

Single-ventricle compression results in PBF (shunt ± PVR) and SBF (SVR)

Increases filling to the preload-dependent single ventricle

Decreases filling to the preload-dependent single ventricle

Bidirectional Glenn and hemi-Fontan

Single-ventricle parallel circulation with PBF dependent on multiple arteriolar vascular beds

IVC—single V—body/ brain— SVC—lungs— pulmonary veins—body

Single-ventricle compression results in SBF

1. Predominantly fills the RA from the IVC 2. SVC flow dependent on cerebral vascular resistance and PVR

Decreases filling to the single ventricle by impeding SVC flow and IVC filling

Fontan

Single-ventricle series circulation

Systemic veins— lungs— Pulmonary veins— body

Single-ventricle compression results in SBF

1. Predominantly fills the PAs with IVC blood flow (PVR) 2. SVC flow dependent on cerebral vascular resistance and PVR

Decreases filling to the single ventricle by impeding both SVC and IVC flow

Physiology

Positive Pressure Ventilation

IVC, Inferior vena cava; LV, left ventricle; PA, pulmonary artery; PBF, pulmonary blood flow; PVR, pulmonary vascular resistance; RA, right atrium; RV, right ventricle; SBF, systemic blood flow; SVC, superior vena cava; SVR, systemic vascular resistance; V, ventricle. From Marino BS, Tabbutt S, MacLaren G, et al. Cardiopulmonary resuscitation in infants and children with cardiac disease: a scientific statement from the American Heart Association. Circulation. 2018;137(22):e691-e782.

Systemic desaturation with normal systemic blood flow and unchanged arteriovenous oxygen saturation difference (initially) is consistent with shunt obstruction. One clinical sign of shunt obstruction in patients who are intubated and mechanically ventilated, and thus have ETCO2 continuously monitored, is a sudden decline in ETCO2 with an increase arterial PaCO2.68-72 The risk of shunt thrombosis is higher in those patients after stage I Norwood palliation with a MBTS than with an RVPAS during the interstage period.62,73,74 Strategies for prophylactic anticoagulation include heparin therapy in the early postoperative period after shunt placement, followed by enteral aspirin once feeds are initiated.55 Aspirin may reduce the risk of shunt thrombosis and cardiac arrest during the first year after placement.73 Acute shunt obstruction may be treated by (1) oxygen to maximize alveolar oxygenation, (2) bolus dose heparin (50 to 100 U/kg) for rapid anticoagulation,55,57 (3) systemic vasoconstricting medications to maximize shunt perfusion pressure (e.g., phenylephrine, norepinephrine, epinephrine), (4) cardiac catheterization or surgical intervention to open shunt, and (5) use of extracorporeal life support (ECLS) to support circulation. Therapies that decrease PVR (e.g., oxygen, hyperventilation, inhaled nitric oxide) will provide little benefit if there is complete shunt occlusion. When shunt obstruction occurs with reduced oxygenation, sedation, and neuromuscular blockade, the placement of an advanced airway and mechanical ventilation at low mean airway pressure will reduce oxygen consumption and may maintain oxygen saturation while the patient is waiting for

definitive therapy to open the shunt.71 If shunt obstruction results in prolonged and severe arterial desaturation, cardiac function will be impaired quickly. In infants with acute shunt obstruction, ECPR may be beneficial if used promptly to support the myocardium during the acute obstruction and/or afterwards during myocardial recovery.62,75-79 Unique Challenges in Cardiopulmonary Resuscitation in the Patient With Patent Ductus Arteriosus or Shunted Single-Ventricle Physiology.  Cumulative data from the STS-CHSD from 2007 to

2012 revealed a 13% incidence of cardiac arrest in those patients who have undergone stage I Norwood palliation.36 When the infant with a shunted single-ventricle physiology has cardiac arrest, it is difficult to obtain ROSC. The mortality rate for infants after stage I Norwood palliation who develop cardiac arrest is five times higher than for those infants who do not suffer cardiac arrest.36 When cardiac arrest develops, providers should begin conventional highquality CPR. It is difficult to obtain effective pulmonary blood flow during resuscitation in the infant with shunted single-ventricle physiology because pulmonary blood flow is shunt dependent and is impacted by PVR and aortic diastolic pressure (for MBTS) and SVR (for MBTS and the RVPAS). Compressions generate only one-third of normal blood flow to the heart and brain during CPR in patients with normal cardiac anatomy.19 In patients with single-ventricle shunted physiology, systemic blood flow during CPR is very likely to be even lower secondary to the loss of some systemic cardiac output to the pulmonary circulation due to the parallel circulation (see Table 31.3). In the neonate with shunted physiology in cardiac

CHAPTER 31  Cardiopulmonary Resuscitation (CPR) in Children With Heart Disease



385

TABLE 31.4  Effect of Respiratory Manipulations on Circulatory Parameters at Different Stages of Palliation of

Children With Univentricular Physiology

Stage 0

1

2

Respiratory Strategy (Alveolar Gas)

SaO2

SvO2

Qp/Qs

↑ ↓

↑ ↓

↓↓

Hypocapnic89 Hyperoxic89 Hypercapnic86,87 Hypoxic

↔ ↑ ↔

↔ ↑ ↔↑

Hypocapnic82 Hyperoxic86 Hypercapnic7,20,82,85,86 Hypoxic

↓ ↑ ↑↑

Hypocapnic Hyperoxic Hypercapnic81,83 Hypoxic81,83

TPG

ΔAVO2

VO2

Lactate

CBF

↓ ↔ ↔ ↔ ↓

↔

↑

↓

rSO2S

↑ ↔

↓

↓

↑

↔ ↔

rSO2C

↑

↓

↓ ↓

↓

↑

↑

Measured parameters in multiple studies are shown. See references for details. Stage 0: Uncorrected/unpalliated ductal dependent parallel pulmonary and systemic circulations. Maintenance of ductal patency is necessary for systemic perfusion and prostaglandin E1 is indicated. No human experimental data exist for measures such as hyperoxic or hypocapnic alveolar gas strategies that tend to reduce pulmonary vascular resistance, and such strategies should generally be avoided without significant monitoring of systemic oxygen delivery. The greatest improvement in systemic oxygenation occurs with induction of hypercapnic ventilation. Stage 1: Post surgical palliation of parallel circulation with relief of arch obstruction and limitation of pulmonary blood flow with a systemic-to-pulmonary artery shunt. Hypercapnia can improve cerebral more than systemic oxygen delivery. Stage 2: Post superior cavopulmonary anastomosis. The cerebral and pulmonary circulations are in series, and hypercapnia can improve systemic arterial oxygen saturation and systemic oxygen delivery by increasing cerebral blood flow, SVC flow, and therefore pulmonary blood flow. Stage 3: Post superior and inferior cavopulmonary anastomoses (post-Fontan). No systematic data exist for alveolar gas manipulation. AVO2D, Arteriovenous oxygen saturation difference; CBF, cerebral blood flow; NIRS, near-infrared spectroscopy; Lactate, lactate or metabolic acid change; Qp/Qs, pulmonary-to-systemic blood flow ratio; rSO2C, cerebral oxygen saturation by NIRS; rSO2S, somatic oxygen saturation by NIRS; SaO2, arterial oxygen saturation; SvO2, systemic venous (SVC) saturation; TPG, transpulmonary pressure gradient; VO2, oxygen consumption. Data from references 7, 20, and 81-90.

arrest the systemic blood flow (SBF) often has low oxygen content and in the presence of reduced coronary perfusion will result in coronary ischemia. As a result, the neonate with shunt-dependent single-ventricle physiology who undergoes a cardiac arrest with prolonged CPR is at particularly high risk for significant end-organ injury, including neurologic injury. Due to the limitations of conventional CPR in the infant with shunt-dependent physiology, it is important to consider other resuscitation measures early in the resuscitation, including use of a pacer for bradycardia (particularly if pacing wires are already available), treatment of arrhythmias, treatment of possible shunt thrombosis, urgent opening of the sternum (in the immediate postoperative period), and early activation of ECLS, if available in the institution. In the infant with shunted single-ventricle physiology, prolonged in-hospital resuscitative efforts that include ECPR may be successful, whereas out-of-hospital resuscitation efforts are typically not successful.76,80

Superior Cavopulmonary Anastomosis and Fontan In the child with a superior CPA circulation with low cardiac output or respiratory insufficiency or failure, strategies to improve venous return to the superior CPA will increase pulmonary blood flow and improve systemic saturation and systemic oxygen delivery. Ventilatory strategies that promote relative hypoventilation to increase cerebral blood flow and minimize intrathoracic pressure have been shown to be most useful. Although spontaneous breathing

is preferable to augment the extrathoracic to intrathoracic pressure gradient and increase pulmonary blood flow and stroke volume in the superior CPA or Fontan physiology, judicious mechanical ventilation is typically well tolerated in circumstances of respiratory insufficiency or failure or low cardiac output. Positive pressure ventilation lowers systemic afterload and wall stress to the systemic ventricle for the child with poor ventricular function or atrioventricular valve regurgitation; however, positive pressure ventilation will decrease pulmonary blood flow and ventricular preload. Modulation of inotropic support, afterload reduction, and positive pressure ventilation is complex and must be individualized for each child in an effort to avoid cardiac arrest. Table 31.4 delineates the effect of respiratory manipulations on circulatory parameters at different stages of palliation of children with univentricular physiology.50 Unique Challenges in Cardiopulmonary Resuscitation in Patients With a Superior Cavopulmonary Anastomosis or Fontan.  Only

1% of patients undergoing a Fontan procedure will develop postoperative cardiac arrest, but 40% of those who arrest will not survive.36 When cardiac arrest develops in patients who have had either of these surgeries, providers should begin conventional high-quality CPR. There are important physiologic differences between singleventricle patients with a superior CPA and a Fontan that impact resuscitation (see Table 31.3).50 In these populations, chest compressions create systemic blood flow; however, pulmonary blood flow can be minimal, resulting in reduced oxygenation and preload to the systemic ventricle, creating a vicious cycle that limits cardiac

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output. Full recoil in these populations is particularly important (i.e., chest recoil can result in blood flow through the superior CPA and lungs, as well as from the inferior vena cava [IVC] into the systemic venous atrium, providing important preload to the single ventricle for the next compression). In contrast, chest recoil in Fontan physiology results in filling of the total cavopulmonary connection from the superior vena cava (SVC) and IVC. During CPR, cardiac output in patients with a superior CPA may be further reduced when there is hemodynamically significant insufficiency of the atrioventricular or semilunar valves. A further concern in patients with a superior CPA is the reduction in cerebral blood flow and risk of neurologic injury during chest compressions secondary to the elevation in SVC and cerebral venous pressure, minimizing the necessary gradient to perfuse the brain.91

Standard Versus Open Chest Cardiopulmonary Resuscitation There are no data to support changing the AHA-recommended CPR technique in children with CHD, although the effectiveness of chest compressions may be compromised if the patient has an open chest. In addition, there are no data to support changing the techniques for cardioversion and defibrillation in children with CHD.22 In defibrillators with pads that contain an accelerometer to measure quality of chest compressions, mattress deflection can confound the measurement of chest compression depth. Thus, when possible, the recommendation would be to use anteriorposterior positioning of these pads such that more accurate measurement of chest compression depth can be made between the pads. These pads can be placed over a sternal dressing to measure quality of chest compressions but will not be able to defibrillate. If the child has dextrocardia, defibrillator pads should be placed over the right side of the chest to keep the heart between the defibrillation pads. If the child’s chest is small, placing the pads in the anteriorposterior position will prevent overlap. This can even be done with paddles for very small infants (i.e., turn the infant on his or her side and place paddles on the chest and back to avoid paddles touching each other). For patients with an open sternum or sternal dressing that require defibrillation, paddle and pad positions may need to be modified, or internal paddles can be used under sterile conditions. In the setting of an open sternum and internal paddles, the dose for defibrillation is 10 to 20 J in adults92,93 and 0.6 to 0.7 J/kg in children.

Monitoring Effectiveness of Resuscitation During Cardiac Arrest Cardiac arrest is a no-flow or low-flow (during CPR) state that risks ischemic injury to critical organs if perfusion is not promptly restored. CPR is the providers’ attempt to restore perfusion and prevent ischemic injury. The prevention of ischemic injury to critical organs depends on early recognition of inadequate perfusion, early institution of resuscitation efforts, the effectiveness of the resuscitation efforts, and the ability to restore adequate spontaneous (or extracorporeal) circulation. Patients with serious cardiovascular disease at risk for cardiac arrest are usually monitored in intensive care, where early recognition and intervention for cardiac arrest are possible. After the early recognition and intervention for cardiac arrest, the intensivist needs to be able to monitor the effectiveness of resuscitation so that efforts can be maximized while attempting

to achieve ROSC or initiate ECPR. Monitoring the effectiveness of resuscitation efforts is a rapidly developing field that focuses on maximizing the provider’s performance and optimizing the patient’s response.

Maximizing the Provider’s Performance— Human Feedback and Human Factors There is increasing recognition of the cognitive load involved in managing a cardiac arrest. It is nearly impossible to ensure exquisite CPR while simultaneously following the rhythm-specific PALS algorithm, identifying and treating reversible causes, and activating ECPR. This is particularly true in the cardiac ICU setting, where teams can be large (including nurses, respiratory therapists, pharmacists, intensivists, and surgeons), there are many streams of information relevant to the cardiac arrest (human voices, bedside monitor with multiple physiologic parameters, NIRS monitor, ETCO2 monitor, defibrillator feedback), and the anatomic and physiologic considerations of the cardiac arrest may be complex. To address this, Hunt et al. developed a model that explicitly distributes the workload of a resuscitation and intentionally cognitively unloads the team leader. Hunt introduced the role of the “quality CPR coach,” who is responsible for coaching the team members performing CPR (i.e., compressions and ventilations) to perform within the AHA guidelines (see Table 31.2) or other physiologic goals such as diastolic blood pressure, CoPP, or ETCO2 defined by the team leader and to periodically update the team leader. The goal is for the team leader to be able to focus on advanced life support (identifying the cardiac rhythm and following the appropriate PALS algorithm and considering and treating possible reversible causes) while another team member can focus on ensuring very high-quality basic life support. Another approach that can decrease the cognitive load of the leader and the team is to optimize and standardize the ergonomics of the room. Potential benefits include (1) optimizing sight lines (i.e., enable compressors to see the arterial line diastolic blood pressure or the depth of compressions reflected on the defibrillator directly in front of them), (2) improved communication, and (3) a shared mental model of how the room should be organized and a decrease in the amount of effort to “figure it out” during an emergency, for example, to enable the team to know ahead of time how to set up the room for ECPR. This entire approach is similar to a pit crew in auto racing or a dance team that creates choreography and practices with each other before important events.

Maximizing the Provider’s Performance— Mechanical Feedback Monitoring the effectiveness of the provider’s performance involves feedback on the quality of the resuscitation efforts. This “mechanical” feedback can be used by the providers to meet recommendations for CPR quality. The AHA identifies the five components of high-quality CPR as providing chest compressions of adequate rate, ensuring chest compressions of adequate depth, allowing full chest recoil between compressions, minimizing interruptions in chest compressions (i.e., maximize “compression fraction”), and avoiding excessive ventilation.44 The recommendations from the AHA for metrics of CPR performance are listed in Table 31.2. Performing high-quality CPR improves the outcome from cardiac arrest.19 Providing resuscitation efforts without having a system of ongoing training, practice, feedback, and debriefing often fails to promote

CHAPTER 31  Cardiopulmonary Resuscitation (CPR) in Children With Heart Disease



387

TABLE 31.5  Studies That Examine the Effect of Audiovisual Feedback During Cardiopulmonary Resuscitation on

Quality Metrics and on Patient Outcome

Study

CCR (/min) NFB vs. FB

CCD (mm) NFB vs. FB

CCF (%) NFB vs. FB

CCL (%) NFB vs. FB

ROSC (%) NFB vs. FB

STD (%) NFB vs. FB

GNO (%) NFB vs. FB

Kramer-Johansen et al.,94 2006

121 vs. 109

34 vs. 38

52 vs. 56

0 vs. 0

17 vs. 23

3 vs. 4

NR

N = 358, OH

P .001

P .001

P .08

P .08

P .3

P .7

Abella et al.,25 2007

104 vs. 100

42 vs. 44

77 vs. 80

NR

40 vs. 44

9 vs. 9

N = 156, IH

P .16

P .47

P .26

P .58

P .97

Hostler et al.,95 2011

108 vs. 103

38 vs. 40

64 vs. 66

15 vs. 10

45 vs. 44

12 vs. 11

10 vs. 10

N = 1586, OH

P .001

P .005

P .02

P .001

P .96

P .21

P .85

Bobrow et al.,96 2013

128 vs. 106

45 vs. 55

66 vs. 84

Not %

25 vs. 22

9 vs. 14

7 vs. 11

NS

OR 2.7 (1.2-6.4)

OR 2.7 (1.0-6.9)

N = 484, OH

NR

Couper et al.,97 2015

121 vs. 114

45 vs. 53

78 vs. 84

18 vs. 14

35 vs. 50

16 vs. 17

16 vs. 14

N = 249, IH

P .02

P .001

P .002

P .75

P .26

P .28

P .60

Couper et al.,97 2015

126 vs. 116

50 vs. 49

78 vs. 82

14 vs. 12

39 vs. 42

17 vs. 13

15 vs. 11

N = 400, IH

P .001

P .38

P .003

P .91

P .60

P .83

P .86

Couper et al.,97 2015

120 vs. 116

46 vs. 54

80 vs. 84

14 vs. 12

52 vs. 56

19 vs. 20

18 vs. 18

N = 746, IH

P .08

P .001

P .001

P .87

P .66

P .73

P .80

Couper et al.,97 2015

122 vs. 115

46 vs. 54

79 vs. 83

15 vs. 12

45 vs. 51

18 vs. 18

17 vs. 16

N = 1395, IH

P .005

P .009

P .002

P .98

P .03

P .35

P .85

Note that the AHA guidelines increased the CCD from ≥38 mm to ≥50 mm in the 2010 recommendations. CCD, Chest compression depth; CCF, chest compression force; CCL, chest compression leaning; CCR, chest compression rate; FB, feedback; GNO, good neurologic outcome; IH, in hospital; OH, out of hospital; NFB, no feedback; NR, not reported; NS, not significant; OR, odds ratio; ROSC, return of spontaneous circulation; STD, survival to discharge. Data from references 25 and 94-97.

and ensure quality CPR. The inclusion of real-time audiovisual feedback about the quality (mechanics) of CPR has been effective in improving most CPR metrics in simulation (manikin) and actual clinical scenarios. Audiovisual feedback about CPR mechanics has been used to improve the quality of resuscitation efforts. Studies that have investigated CPR quality in actual clinical scenarios with and without audiovisual feedback are presented in Table 31.5.25,94-97 Several of the studies show improvements in chest compression rate, depth, fraction, and leaning after audiovisual feedback is enabled. One shows that these metrics improved without feedback over the same time frame.97 Most of these studies fail to show any improvement in outcome (ROSC, survival to discharge [STD], or neurologic function) despite the addition of audiovisual feedback and improvement in quality, though they were not designed with adequate power to do so (see Table 31.5). Because it appears that real-time monitoring of the performance metrics of CPR has the potential to improve the quality of resuscitative efforts, it is important for us to consider how to optimize the use of these tools. It is important for caretakers in cardiac intensive care to focus on delivering chest compressions at the rate, depth, and release outlined in Table 31.2 and with minimal interruptions. Real-time feedback about performance metrics can be delivered by audio or visual output from resuscitation aids and/or from quality CPR coaches. It is important that providers be familiar with and not overwhelmed or distracted by the audiovisual input provided as they focus on the quality of their chest compression delivery.

Optimizing the Patient’s Response— Physiologic Feedback The use of real-time physiologic feedback during CPR involves monitoring the patient’s response to the resuscitation efforts and has the potential to improve outcome. Using physiologic feedback has important differences from using mechanical feedback. Mechanical feedback is based on recommendations that are meant to be generally applicable to any cardiac arrest and other than slight alterations in the guidelines related to the patient’s age, do not include guidance on how to individualize or optimize efforts based on the patient’s underlying anatomy or physiologic response. The use of real-time physiologic feedback during CPR allows changes to resuscitation technique to be studied for effects on physiologic metrics. The 2013 AHA consensus statement on CPR quality and improving resuscitation outcomes recommends the use of physiologic monitoring during CPR. The metrics include coronary perfusion pressure (CoPP), diastolic blood pressure (DBP), and ETCO2 level monitoring.19 The CoPP is estimated at the bedside by the following parameters (CoPP = diastolic blood pressure − central venous pressure), please see further discussion below on more advanced and accurate calculations. Studies involving physiologic feedback have focused on prognostication and provide little evidence or guidance about how to use this feedback to optimize resuscitative efforts. There are some animal data to support the timing of vasopressor administration

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to improve myocardial perfusion during CPR (see hemodynamicdirected CPR). The most commonly studied methods of physiologic monitoring include hemodynamic, ETCO2, cerebral regional oxygen saturation (rScO2), electrocardiogram amplitude spectral analysis, blood gas and blood lactate levels, and cardiac and vascular ultrasonography. These physiologic monitoring methods differ by whether they involve invasive techniques, are continuous or intermittent, are real-time or delayed, and whether they interfere with resuscitative efforts. These methods also differ by whether they represent myocardial, cerebral, or systemic perfusion and whether they can be used for prognostication, to determine futility, to detect ROSC, or to adjust resuscitative efforts. The study of the use of these methods during CPR is complicated by variations in measurement and reporting of clinical targets based on initial, average, 20-minute, peak, final, or trending values. Average values may be useful in research but are the most difficult to use in real time. Peak, 20-minute, and final values tend to be most useful when used for futility determinations. Trends in the values may ultimately be the most important clinically but are rarely reported in studies and deserve further attention. Explanations of these physiologic feedback techniques and the available information about their use are provided in the following sections. Hemodynamic Monitoring During Cardiopulmonary Resuscitation.  Hemodynamic monitoring during CPR involves the use of

arterial and/or central venous pressure (CVP) catheters to measure the systolic blood pressure (SBP), DBP, SvO2) and derived CoPP. Calculation of the CoPP requires both arterial and central venous access and uses the formula CoPP = DBP − the venous relaxation (diastolic) pressure. The CoPP is considered the most useful surrogate for myocardial blood flow during CPR. The next most useful surrogate for myocardial blood flow during CPR is the DBP, which requires arterial access. The SBP also requires arterial access and is a less well-described metric of systemic perfusion. Measurement of the SvO2 requires central venous access and is a surrogate of systemic perfusion. All four parameters used for hemodynamic monitoring during CPR require invasive monitoring and may be applicable to children in the cardiac ICU. It is not recommended to interrupt chest compression delivery to place invasive lines for the sole purpose of determining these measurements during CPR. However, if an arterial line can be placed during a cardiac arrest without compromising CPR, the real-time values may be very helpful in directing high-quality compressions. If the lines are already in place, these measurements may be used for optimal direction of high-quality compressions. This is important because it allows the team to maximize coronary perfusion while avoiding overzealous compressions with depths that increase risk of unintended consequences. The CoPP, DBP, and SBP can be measured continuously and in real time. The SvO2 is measured intermittently unless an oximetric catheter is in place. The calculation of the CoPP during CPR requires both the DBP from the arterial line and the diastolic venous pressure from the central venous line. Because most bedside monitors display the CVP only as a mean value (i.e., does not report the CVP systolic and diastolic values), renaming the “CVP” within the bedside monitoring parameter menu as the “pulmonary artery” on the monitor allows the display of diastolic venous values to be available for CoPP calculation. For any of these four parameters the initial value may indicate the severity of the injury before starting CPR. Initial values that are low may indicate a worse preresuscitation status, whereas high initial values represent a less severe and potentially more recoverable status. Late values that are high or increasing represent a good response, whereas late values

TABLE 31.6  Parameters Used for Physiologic

Feedback During Cardiopulmonary Resuscitation and Approximate Targets

Perfusion Area

Parameter

Target

Coronary

Arterial relaxation pressure Myocardial perfusion pressure Amplitude spectral area

>25 mm Hg >20 mm Hg >13 mV Hz

Cerebral

Cerebral regional oxygen saturation

>30%

Systemic

End-tidal carbon dioxide Mixed venous saturation Blood lactate levels

>20-25 mm Hg >30% <9-12 mmol/L

that remain low or are decreasing may represent a poor response to CPR and lower likelihood of ROSC and thus support strong consideration of ECPR. Prognostication.  In an early study of adults with OHCA a maximal CoPP of greater than 15 mm Hg predicts 50% survival.98 No data are available for CoPP and survival in children. In a query of the AHA GWTG-R registry the use of DBP to monitor CPR quality was associated with improved ROSC in adults.99 Futility.  The early study of adults with OHCA also found that a maximal CoPP of less than 15 mm Hg was associated with no survival.98 Detection of Return of Spontaneous Circulation.  Rapid increase in arterial blood pressures may represent that ROSC has occurred, but no specific values have been suggested for holding chest compression delivery to determine if ROSC is present. Direction of Resuscitative Efforts.  A series of preclinical studies have led to the recommendation to use CoPP, DBP, SBP, and SvO2 to monitor and optimize chest compression quality and titrating vasopressor therapy. Precise numeric targets have not been established, but approximate values are listed in Table 31.6. Specific target values for blood pressure have not been established in children. Rescuers in advanced care settings with access to invasive arterial blood pressure monitoring can use targets based on expert consensus.19,41 In swine with ventricular fibrillation (VF)-induced cardiac arrest the adjustment of chest compression depth to obtain a CoPP above 30 mm Hg increased the rate of ROSC and postresuscitation myocardial function.100 In an adult OHCA study the intraarrest placement of femoral catheters into the aorta and inferior vena cava allowed adjustment of the chest compression rate and depth and administration of intraaortic epinephrine to improve the CoPP from 8 to 25 mm Hg.101 In a series of studies in 10- to 30-kg swine the use of hemodynamic-directed CPR (HD-CPR) has been compared with standard CPR in both VF and asphyxial arrest models of 7 minutes’ duration.102-108 The HD-CPR group received chest compressions adjusted to a depth to produce a SBP above 100 mm Hg, and epinephrine and vasopressin were titrated to produce a CoPP above 20 mm Hg. The standard CPR group received a chest compression depth consistent with current guidelines, and epinephrine was dosed at 4-minute intervals. The HD-CPR group consistently used a shallower chest compression depth than the standard group yet had an increased DBP, CoPP, rate of ROSC, and survival at 24 hours and improved neurologic outcome. The improvements in these parameters occurred in both VF and asphyxial arrest. In a retrospective review of 3 years of



CHAPTER 31  Cardiopulmonary Resuscitation (CPR) in Children With Heart Disease

their group’s animal experiments, Morgan et al. determined that a DBP less than 34 mm Hg was a threshold that was highly associated with nonsurvival.107 The evolving data in this field are intriguing because they suggest that compression depth may not be as important as vasopressor administration in achieving target blood pressure goals and that there are potential benefits of individualized resuscitation. It is possible that in children who have hearts or vascular tone that are very responsive to chest compressions or vasopressors, the team might be able to minimize the compression depth needed and/or the amount of vasopressor needed. Alternatively, when the patient is less responsive, it is possible that by using more vasopressor the team may be able to minimize the depth of compressions needed and thus has the potential to decrease complications. In cardiac intensive care, hemodynamic monitoring during CPR, when the appropriate vascular catheters are in place, may provide real-time information about the effectiveness of resuscitative efforts, myocardial perfusion, and the likelihood of ROSC. Adjusting resuscitative efforts, especially vasopressor administration, holds promise for improving these measurements, myocardial perfusion, and clinical outcomes. However, there are few human data and no current guidelines to clearly outline which physiologic parameters are the most important, what the goals for each parameter should be, and if the goals should vary by age, size, or anatomy. Thus this is a high-priority area of future research. End-Tidal Carbon Dioxide Monitoring During Cardiopulmonary Resuscitation.  ETCO2 monitoring during CPR involves the

measurement of the carbon dioxide level at the end of expiration. The ETCO2 level during CPR is a measure of pulmonary blood flow in low-flow states and has been shown to correlate with cardiac output (systemic perfusion). Capnography during CPR has been used to confirm endotracheal tube placement and maintenance, to monitor for ROSC, and to assess the effectiveness of CPR. Capnography is preferred to capnometry because visualization of the waveform helps verify the integrity of the values. ETCO2 measurements can be made continuously and in real time. As described in the following discussion, ETCO2 levels during CPR are highly correlated with pulmonary and systemic perfusion. The use of ETCO2 monitoring during CPR has traditionally been described in regard to use with an endotracheal tube. Although little information is available about the reliability of ETCO2 measurements to assess the effectiveness of CPR with mask ventilation, we would still recommend placement of quantitative ETCO2 capnography in line with face mask and bag until an advanced airway is placed. At a minimum, presence of an ETCO2 waveform and numeric values with bag-mask ventilation confirm the airway is open and can confirm both some degree of ventilation and cardiac output are taking place. Studies report different ETCO2 measurements (initial, average, final, and 20 minute) in relation to a cardiac arrest. The initial value is important for early prognostication and evaluation of efforts. Definition of initial measurement varies by study (the first value after intubation, the value 1 minute after intubation, and the value six breaths after intubation are some of the variations in study design). The average value during an arrest is difficult to use clinically. A 20-minute value may be useful for termination of efforts. The final value may be problematic to interpret because of difficulty separating when ROSC occurred, and so the final value may be falsely elevated. In 30 adults with OHCA and ETCO2 level above 20 mm Hg, between 5 and 10 minutes of CPR had the strongest correlation with ROSC.109 A systematic review showed that the slope of the ETCO2 trend line and the cumulative

389

maxETCO2 were the most consistently significant differences between patients with and without ROSC.110 Several factors have an influence on ETCO2 levels during CPR. The cause of the cardiac arrest may influence early ETCO2 levels. Hypercarbia produced during asphyxia may take some time to wash out during CPR before the ETCO2 level represents the effectiveness of resuscitation. In adults with asphyxial arrest the ETCO2 levels after 5 minutes of CPR were useful.111 In animals with asphyxia the ETCO2 level normalized after five breaths112 or 1 minute of CPR.113 Bicarbonate and epinephrine administration during CPR may also influence the ETCO2 level. Bicarbonate administration is associated with a transient increase in the ETCO2 level. Epinephrine administration has been variably reported to transiently increase, decrease, or not affect the ETCO2 level during CPR. Prognostication.  Two studies that evaluated the frequency of use of ETCO2 monitoring in adults with cardiac arrest found an association between the use of ETCO2 monitoring during CPR and ROSC.107,114 A 2013 systematic review concluded that there was a strong correlation between ETCO2 levels during CPR and outcome. They found that 15 of 18 studies confirmed association of ETCO2 level with ROSC and 5 confirmed association of ETCO2 level with STD.110 A 2015 systematic review of 27 studies found that the mean ETCO2 level in patients with ROSC was 26 mm Hg and without ROSC was 13 mm Hg. They suggest that developing a target ETCO2 level during CPR has the potential to improve the rate of ROSC or decrease ischemic organ injury and that the potential target for prognostication may be higher than previously believed.115 In adults with IHCA an initial ETCO2 level (after six manual ventilations) above 25.5 mm Hg was predictive of sustained ROSC and STD but not of favorable neurologic outcome.116 Futility.  An ETCO2 level during CPR of less than 10 mm Hg immediately after intubation or after 20 minutes of CPR has been associated with extremely poor chances of ROSC.110 Although proposed cutoff values for ETCO2 have been considered to assist in predicting outcome, this is complicated because the value may be affected by confounders.117 A confounder that is common clinically is the presence of pulmonary edema fluid in the endotracheal tube, which leads to a falsely low ETCO2 level. There are other potential causes of low ETCO2 levels that can be potential confounders (i.e., still have potential for recovery if the underlying issue is addressed). For example, an infant with a clotting BT shunt will likely have a low ETCO2 level due to hypoperfusion to the lungs and associated poor cardiac output or cardiac arrest that may be reversible with medical management, including a high-dose heparin bolus and ECPR. A similar presentation can occur for a large pulmonary embolism as the cause for cardiac arrest in older children and adults. Finally, profound hypovolemia can also be associated with low ETCO2 levels that may be the reversible cause of a cardiac arrest.118-120 Recommendations for the use of ETCO2 level in assessing futility apply only to intubated patients, and although an ETCO2 level of less than 10 mm Hg is strongly associated with mortality, ETCO2 values should not be used as a mortality predictor in isolation.41 If the ETCO2 level is much lower than expected despite optimizing quality of CPR, it is important to consider these potentially treatable alternate causes before considering futility. Detection of Return of Spontaneous Circulation.  An abrupt increase in ETCO2 level during CPR can be the first sign of return of native circulation when CPR efforts are ongoing and may be an indication to stop chest compressions and check the rhythm and presence of a pulse. The size of the increase that

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indicates ROSC is not known, but author experience suggests a jump from the 20s to the 40s is highly suggestive of ROSC and warrants a coordinated pause to assess for a spontaneous pulse. Alternatively, when the ETCO2 is consistently staying at a value below 25 with no other signs of improved cardiac output (i.e., no improvement in color, no spontaneous movement, no jump in arterial blood pressures,) the authors would avoid stopping chest compressions for pulse checks as very low yield, in order to minimize no-flow time. Direction of Resuscitative Efforts.  When the ETCO2 level during CPR is consistently less than 15 mm Hg, efforts should focus on improving CPR quality (quality of chest compressions and avoiding overventilation).20 In adults with IHCA and OHCA the ETCO2 values during CPR were significantly associated with chest compression depth but not rate.121 The use of the ETCO2 level (in isolation from other feedback) to optimize chest compression depth and rate in a neonatal swine was as effective as standard CPR with chest compressions optimized by marker, video, and verbal feedback after a short arrest interval.122 In a long arrest interval ETCO2-guided CPR provided increased ROSC compared with standard CPR in the same preclinical mode.122 In cardiac intensive care, ETCO2 level monitoring during CPR is widely available for intubated patients and should be used to continuously confirm airway access and to reflect and guide quality of CPR. For hospitals in limited-resource areas that do not have ETCO2 monitoring available for every intubated patient, we advise owning even a single portable ETCO2 device to be taken and used in the event of any cardiac arrest in the hospital. This has been a successful strategy as a first step or long-term strategy for cardiac arrest response teams. As an indicator of systemic perfusion, it is recommended as the second choice for monitoring the effectiveness of CPR behind hemodynamic monitoring.19 Cerebral Regional Oxygen Saturation Monitoring During Cardiopulmonary Resuscitation.  Cerebral regional oxygen satura-

tion monitoring during CPR involves the use of a NIRS emitter and sensor applied to the patient’s forehead. NIRS is not dependent on pulsatile flow and can be used in low-flow states. The measured values represent the saturation in primarily venous (70% venous) blood and are a surrogate for regional brain perfusion. Cerebral regional oxygen saturation is noninvasive and can be used continuously and in real time. This monitoring may already be in place after a cardiac surgical procedure because cerebral NIRS monitoring is often used intraoperatively and continued postoperatively. Monitoring rScO2 during CPR may provide a surrogate for regional brain perfusion during CPR. In a 2016 systematic review of 19 studies examining the initial, mean, and highest levels of rScO2 during CPR the highest levels were too heterogeneous a subgroup for meta-analysis. The initial level was associated with STD and favorable neurologic outcome, a combination of mean and initial levels was associated with STD and favorable neurologic outcome, but the mean level alone was not associated with STD or neurologic outcome.123 The mean levels were more likely to be associated with ROSC than initial levels. NIRS is mainly a trend monitoring technique, and trends during CPR may offer the most important prognostic value.124 Prognostication.  In adults in cardiac arrest, survival has been associated with mean rScO2 levels,125 an increase in rScO2 level of more than 20% during CPR,126 an increase from baseline, and percentage of time with rScO2 level above 40%.127 In adults with IHCA, maintaining the rScO2 level above 50% improved survival with a favorable neurologic outcome, and peak rScO2 (>65%) was associated with ROSC.128 A 2015 systematic review of nine studies

of adults with OHCA and IHCA found that higher initial and averaged NIRS levels were associated with ROSC.124 In adults with OHCA going onto ECPR, no change in rScO2 level after initiation of ECPR was a better prognosis for neurologic outcome than if the level improved on ECPR.129 Futility.  In adults with IHCA or OHCA a persistently low (<25% to 30%) rScO2 may predict futility.123,125,130 Detection of Return of Spontaneous Circulation.  In adults with OHCA without endotracheal intubation with standard chest compressions, the use of NIRS in the emergency department was associated with ROSC.131 Direction of Resuscitative Efforts.  In adult IHCA, during four separate episodes of low-quality CPR, no increase in NIRS was observed despite efforts to improve the quality of CPR.132 In cardiac intensive care, monitoring rScO2 levels during CPR may be useful when available and is easy to apply if not already applied. It may be useful to predict outcome or futility and to detect ROSC. The usefulness of rScO2 to direct resuscitative efforts is still not clear. Blood Gas and Lactate Level Monitoring During Cardiopulmonary Resuscitation.  Blood gas and lactate level monitoring

involves the determination of pH, pO2, or pCO2 and lactate levels by blood sampling during CPR. Low pH or lactate levels may be associated with long periods of no or low flow. Trends in any of these values during CPR may reflect systemic perfusion produced by resuscitative efforts. Blood gas and lactate level monitoring during CPR is invasive and requires a vascular catheter or IO access. Continuous and real-time analysis using these methods are not available. Prognostication.  In adults with OHCA an intraarrest pCO2 level of less than 75 mm Hg (either arterial or venous) on arrival to the emergency department was associated with sustained ROSC.133 Other blood gas variables and the lactate level were not predictive of ROSC. The use of blood gas and ETCO2 measurements to calculate the arterial-alveolar (PaCO2-ETCO2) CO2 difference was inversely associated with survival to hospital admission.134,135 In adults with IHCA an intraarrest serum lactate level of less than 9 mmol/L measured during first 10 minutes of CPR was associated with STD and favorable neurologic outcome.136 No study was found that used blood gas or lactate levels during CPR to predict futility, detect ROSC, or direct resuscitation efforts. In cardiac intensive care, many patients often have catheters that would allow frequent sampling of these parameters during CPR. The contribution of these parameters to the management of children in arrest is unknown but likely similar to the observations from adults. The data could be additive to that from other methodologies when assessing the effectiveness of prolonged CPR efforts. Ultrasound Monitoring During Cardiopulmonary Resuscitation.  Ultrasound monitoring during CPR involves the use of

echocardiography to determine reversible causes of cardiac arrest, mechanical activity versus standstill, or blood flow during resuscitative efforts. Echocardiography, although noninvasive, may interfere with chest compression performance.137 It is real time but usually not continuous. Intermittent application is used to reduce interference with resuscitative efforts. Ultrasound monitoring during cardiac arrest can demonstrate cardiac mechanical activity and detect carotid blood flow or be used to rule out reversible causes of cardiac arrest. Prognostication.  Mechanical activity on initial ultrasound examination during cardiac arrest is associated with ROSC (84%) versus its absence (14%).138 Echocardiography use during the 10-second intervals when compressors changed was evaluated to determine reversible causes of pulseless electrical activity (PEA). It detected hypovolemia and trivial effusion but not pneumothorax

CHAPTER 31  Cardiopulmonary Resuscitation (CPR) in Children With Heart Disease



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Mechanical Support—Extracorporeal Life Support and Extracorporeal Cardiopulmonary Resuscitation

or tamponade. Treatment based on findings did not improve survival. True PEA did not result in survival.139 Futility.  Serial ultrasound examinations during the 10-second intervals every 2 minutes for pulse check and compressor change showed that no adult with an OHCA and cardiac standstill at the 10-minute or greater examination had ROSC.140 Detection of Return of Spontaneous Circulation.  No study was found that used ultrasound measurement during CPR specifically to detect ROSC. Many studies use ultrasonography during CPR to detect mechanical activity when pulses are not present. The distinction between inadequate and adequate mechanical activity is usually made based on hemodynamics. Direction of Resuscitative Efforts.  The feasibility of collection of 5 minutes of common carotid blood flow measurements during chest compressions has been shown in adults with OHCA. The relevance of this technique to the performance of CPR is not known.141 Diagnosis of the Cause of Cardiac Arrest.  The finding of right ventricle dilation by echocardiography during CPR in a preclinical study was not useful to determining pulmonary embolus.142 Reversible causes, including hypovolemia, tension pneumothorax, tamponade, pulmonary embolism, and myocardial activity, can be detected using ultrasonography in 10-second intervals during compressor change.139,143 In cardiac intensive care, ultrasonography or echocardiography is often available and may be considered during CPR but risks potential harm from interruption of chest compressions. Suspicion of an ultrasound-detectable cause is recommended before use because of the lack of evidence that it changes outcome. Tamponade, hypovolemia, and hemothorax or pneumothorax may be more common in cardiac intensive care and increase the usefulness of intraarrest ultrasonography.

Since Bartlett and others started using extracorporeal membrane oxygenation (ECMO) to support children with CHD in the 1970s, ECMO has evolved from an extraordinary, last resort, lifesaving intervention to a standard of care in many pediatric centers with thousands of patients supported to date.130,144,145 Indications for ECMO now include not only cardiovascular and/or respiratory failure but also sustained cardiac arrest. Extracorporeal CPR (ECPR or rescue ECMO) is the initiation of ECMO support while active chest compressions are taking place. ECPR was first described in 1992, and since then its use has increased significantly. 146,147 Improvement in outcomes following in-hospital pediatric cardiac arrest has been attributed in part to the impact of ECMO as a rescue strategy when prolonged conventional CPR cannot restore spontaneous circulation.33,148-150 Pediatric patients who receive ECPR for refractory cardiac arrest have survival to hospital discharge rates ranging from 33% to 42% in general ICU patients151-154 and from 23% to 55% in cardiac ICU patients.153-155 The AHA has recognized a role for ECPR in the 2015 pediatric advanced life support guidelines for CPR and emergency cardiovascular care. These guidelines recommend that ECPR may be considered for pediatric patients with cardiac diagnoses who have IHCA in settings with existing ECMO protocols, expertise, and equipment.41 The recent AHA scientific statement “Cardiopulmonary Resuscitation in Infants and Children With Cardiac Disease” outlines cannulation strategies (Table 31.7) for ECPR and mechanical circulatory support device strategy in rapidly deteriorating children with heart disease (Fig. 31.2).50

TABLE 31.7  Cannulation Strategies for Extracorporeal Cardiopulmonary Resuscitation

General Principles for Efficient Use of ECLS to Support CPR 1. 2. 3. 4. 5.

Venoarterial ECLS should be utilized in all cases. Knowledge of venous anatomy and previously occluded vessels is critical for successful and timely deployment of ECLS. Central (transthoracic) cannulation may be considered in patients who have undergone a recent sternotomy. Peripheral (percutaneous) cannulation may be preferred for patients without recent sternotomy. ECLS cannulas should be large enough to provide complete cardiac output (CI greater than 2.5 L/min/ m2). If ECMO flow is limited by inadequate venous drainage, secondary drainage sites should be considered. PERIPHERAL CANNULATION

CENTRAL CANNULATION

Physiology

Venous

Arterial

Venous

Arterial

Comments

Biventricular circulation

Internal jugular or femoral

Common carotid or femoral

Systemic venous atrium

Aorta

Left atrial decompression may be required.

Single ventricle or shunted physiology

Internal jugular

Common carotid

Systemic venous or common atrium

Aorta

Shunt restriction may be required. For carotid cannulation with a MBTS, cannula position may result in shunt overcirculation or occlusion.

Superior cavopulmonary anastomosis

Internal jugular and/or femoral

Common carotid

SVC and/or systemic venous or common atrium

Aorta

Additional venous cannula may be required.

Fontan

Internal jugular and/or femoral

Common carotid or femoral

Fontan baffle

Aorta

Additional venous cannula may be required. Pulmonary venous atrial drainage may be required.

CI, Cardiac index; ECLS, extracorporeal life support; ECMO, extracorporeal membrane oxygenation; MBTS, modified Blalock-Taussig shunt; SVC, superior vena cava. Modified from Marino BS, Tabbutt S, MacLaren G, et al. Cardiopulmonary resuscitation in infants and children with cardiac disease: a scientific statement from the American Heart Association. Circulation. 2018;137(22):e691-e782.

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Acute Heart Failure

Cardiac arrest with CPR

Unstable circulation

Respiratory failure

VA ECLS

VA ECLS

No respiratory failure

Cardiac arrest imminent

Cardiac arrest not imminent

VA ECLS or Temporary VAD

Temporary VAD or Durable VAD

1. Choice of VAD depends on patient size and need for LV or biventricular support. 2. Examples of temporary VAD: centrifugal pump ECLS without oxygenator, Impella, and TandemHeart. 3. Examples of durable VADs: Berlin Heart EXCOR, Thoratec PVAD, HeartMate, HeartWare, and TAH/Syncardia.

• Figure 31.2

  Mechanical circulatory support device strategy in rapidly deteriorating children with heart disease. CPR, Cardiopulmonary resuscitation; ECLS, extracorporeal life support; LV, left ventricular; TAH, total artificial heart; VA, venoarterial; VAD, ventricular assist device. (Data from references 186 through 208.)

Evidence from four observational studies of pediatric IHCA has shown no overall benefit to the use of ECPR compared to conventional CPR.12,156-158 More recently, however, data from the GWTG-R registry have shown that among pediatric patients treated with at least 10 minutes of in-hospital CPR, those receiving ECPR had greater odds of STD than patients who received continued CPR. Importantly, ECPR patients also had a greater survival with favorable neurologic outcome.24 Similar improved outcomes have been shown when ECPR is used for children with underlying cardiac disease compared to those with noncardiac disease,33 with the population of surgical cardiac diagnoses having the greatest likelihood for survival to hospital discharge.32 Historically, pediatric CPR was considered futile beyond 20 minutes’ duration or more than two doses of epinephrine.159,160 A recent report from the AHA’s GWTG-R analyzed the relationship between CPR duration and survival to hospital discharge after pediatric IHCA.161 Survival rates fell linearly over the first 15 minutes of CPR, yet patients who received ECPR had no difference in survival across CPR durations. Survival for patients receiving more than 35 minutes of conventional CPR was only 15.9% (survival for patients receiving conventional CPR for <15 minutes was 44.1%). For children with underlying cardiac disease, when ECPR is initiated in a critical care setting, long-term survival has been reported even after more than 50 minutes of conventional CPR.151 In a report from the Extracorporeal Life Support Organization (ELSO) database on 492 patients with cardiac disease who underwent ECPR,162 survival to hospital discharge was 42%, with mortality associated with single-ventricle physiology, the stage I Norwood procedure, extreme acidosis before ECMO, renal injury, neurologic injury, and duration of ECMO support. Right carotid artery cannulation was associated with decreased mortality risk and is most likely secondary to the ability to perform high-quality CPR without interruption with neck cannulation, compared to transthoracic cannulation.

Long-term neurologic outcome data are lacking in survivors of ECPR. In the ELSO database, acute neurologic injury was reported in 22% of the 682 patients who had ECPR.163 Acute neurologic injury was defined in the ELSO database as brain death, cerebral infarction, or intracranial hemorrhage determined by ultrasonography or computerized tomography imaging of the head; 11% had brain death, 7% cerebral infarction, and 7% intracranial hemorrhage, with an in-hospital mortality for patients with acute neurologic injury of 89%. However, this manuscript did not report any medium-term (at hospital discharge) or long-term (following index hospitalization) neurologic outcome data as functional outcome at discharge, and long-term follow-up after index hospitalization was not collected by ELSO. Although recent papers have reported high percentages of children with good neurologic outcome at the time of discharge,33,152,162 this most likely does not represent true long-term neurologic injury because the majority of survivors are young infants. A small cohort study of 51 pediatric cardiac patients is the only study reporting detailed neurocognitive outcomes of ECPR survivors and found that global intelligence, vocabulary, and adaptation skills were significantly lower than the population mean with 24% having intellectual disability.164 These results highlight the need for further studies to identify improved ECPR protocols and techniques, as well as early intervention programs for these children to optimize their long-term outcomes. It is also important to consider that ECPR is usually provided to those patients with refractory cardiac arrest, and these children were likely to die if this support was not provided.

Post Cardiac Arrest In past decades, efforts to resuscitate a child would cease when the child either had ROSC, the team determined it was futile to continue, or the parents asked for the resuscitation to stop. In the era of ECPR there is a fourth option: the team may have achieved “circulation,” albeit via the ECMO circuit, but no spontaneous



CHAPTER 31  Cardiopulmonary Resuscitation (CPR) in Children With Heart Disease

pulse. This is now commonly referred to as return of circulation (ROC), rather than ROSC. If a child was hemodynamically unstable or in a coma, then critical care would continue, but there was not an organized framework for “post–cardiac arrest care” (PCAC). This period has become increasingly complex and nuanced, and efforts to improve processes of care during the post–cardiac arrest period are having an important impact on optimizing neurologically intact survival. The science in this area continues to evolve, but it is clear there are two distinct issues to address regardless of whether the child is resuscitated. The first is postevent debriefing, and the second is performing organized PCAC.

Post–Cardiac Arrest Debriefing The literature continues to accumulate that debriefing teams on their performance after a cardiac arrest has a positive impact on subsequent resuscitation performance and may impact survival as well.165-167 There is also evidence to suggest that combining real-time feedback during the cardiac arrest with postevent debriefing is synergistic with a stronger impact than either element alone.166 A common element to debriefings associated with improved performance is the use of objective performance data. This allows the discussion to move beyond a reflection of the team’s subjective experience to a discussion framed around any performance gaps and/or exploring things that went exceptionally well. Despite these data, a survey of a nationally representative sample of US hospitals published in 2014 revealed that only 34% of hospitals report conducting post–cardiac arrest debriefings.168 Research is being conducted as to how to optimize debriefing (i.e., hot debriefings [immediately after the event], cold debriefings [separated in time from the event once data have been accumulated and reviewed—e.g., 1 week later], or a combination of the two). Although there is less evidence on the role of the hot debriefing, the authors’ experience is that it can be important for several reasons. Traditionally teams have focused on the fact that it can be used to diffuse the situation emotionally and help the team get back to work knowing there will be time for further discussion about what went well and what needs further discussion at a subsequent cold debriefing. The time can be used to create the agenda for that follow-up meeting. In addition, a hot debriefing can be used to make sure that any broken equipment or used-up medication is replaced or restocked before any subsequent events, as well as to clarify and refine the plan for any subsequent cardiac arrests that the child may have. For example, should the child receive ECMO immediately to avoid a second arrest; in the event of ECPR, can the room be optimized for ergonomics (i.e., move the child so the surgeon has easy access to perform the cannulation); have all reversible causes been addressed, or should the parents be approached about limiting further resuscitation efforts? Finally, the postevent hot debriefing is an excellent time to discuss the PCAC goals as defined in the next section.

Post–Cardiac Arrest Care Ongoing clinical instability in the postresuscitation period often occurs, necessitating continuous cardiopulmonary monitoring and careful management to allow recovery time, optimize systemic perfusion to preserve end-organ function, and optimize outcomes. Precipitating causes of the arrest should be identified and treated promptly to avoid further deterioration and recurrent arrest. Placement of arterial access is helpful for blood gas sampling and

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blood pressure monitoring. If IO access was placed for resuscitation, more stable venous access is warranted with subsequent removal of the IO as soon as possible. An indwelling urinary catheter is helpful to follow urine output closely as both a surrogate marker to adequate cardiac output and possible renal injury. Laboratory values, including renal function, electrolytes, complete blood count, coagulation studies, venous and arterial blood gases, and lactate, may help guide postresuscitation care, particularly when abnormal values are identified that may have contributed to the cardiac arrest. A chest x-ray should be obtained to assess appropriate ETT placement and to assess for cardiopulmonary pathology (including heart size, parenchymal lung disease, pneumothorax, and/or pleural effusions.) Myocardial dysfunction and vascular instability are common after arrest, and inotropic support is often required. An echocardiogram to assess for inadequate ventricular filling, myocardial function, and cardiac tamponade can guide therapy for fluid resuscitation, inotropic support, or need for pericardiocentesis, respectively. Hypotension (less than fifth percentile for age) should be avoided in the postresuscitation period because hypotension has been associated with lower likelihood of survival with favorable neurologic outcome.169 There are no data currently to recommend specific vasoactive medications for blood pressure support, and such infusions should be tailored to patient-specific physiology and cause of cardiac arrest. Typically 100% oxygen is administered during CPR, especially in the hospital setting. After return of circulation, monitoring of oxygen saturations and PaO2 is prudent because both hyperoxemia and hypoxemia after cardiac arrest have been associated with worse outcomes.170,171 Postischemic hyperoxemia contributes to oxidative stress and cellular injury, decreased cardiac output, and decreased oxygen delivery to the cerebral and coronary vascular beds.172-174 Hypoxemia limits oxygen delivery to tissues that have just experienced ischemic time. The AHA currently recommends targeting normoxemia (defined as a PaO2 level between 60 and 300 mm Hg) and avoidance of hyperoxemia and hypoxemia. In the absence of arterial blood gas information, one should wean supplemental oxygen as tolerated for a pulse oximetry saturation of 94% to 100% (or patient-specific goal saturations) to avoid hyperoxemia.41 Few data exist to support a specific PaCO2 range in the postarrest period. Given that PaCO2 plays an important role in the regulation of cerebral flow, it is sensible to avoid hyperventilation and hypocapnia because this may decrease cerebral blood flow and potentiate neuronal ischemia. In one observational pediatric study, both hypocapnia and hypercapnia post arrest were associated with higher mortality.171 At this time the AHA recommends targeting PaCO2 for the specific patient’s condition and limiting significant hypocapnia or hypercapnia.41 Glucose level should be measured during and after resuscitation, particularly in infants because they have a higher glucose requirement and lower glycogen stores. The authors have experienced patients who have been hyperglycemic at the beginning of the arrest with progression to “unmeasurably low values” during prolonged arrests. Thus we would recommend checking the glucose level every 20 to 30 minutes during a prolonged resuscitation attempt (i.e., ECPR). Hypoglycemia should be treated promptly. Hyperglycemia is common in the postarrest period, but with few data to support tight glycemic control, the AHA does not currently have a target glucose range recommendation. Current AHA guidelines recommend continuous temperature monitoring following cardiac arrest with the primary focus on

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avoiding fever (>38°C) because fever has been associated with poor outcomes.41,175 Therapeutic hypothermia following arrest in attempt to prevent neurologic injury has been a controversial topic. Though therapeutic hypothermia has been shown to improve neurologic outcomes in neonates with asphyxia176 and adults after OHCA with an initial rhythm of ventricular fibrillation,177 there are conflicting data in infants and children. One study showed improved survival in infants and children after cardiac arrest,178 whereas others have shown worsened mortality and functional outcome.179 A recent large multicenter randomized controlled trial evaluating the effects of therapeutic hypothermia (target temperature 33°C) versus therapeutic normothermia (target temperature 36.8°C) for 120 hours post–cardiac arrest showed no difference in survival with a favorable neurologic outcome at 1 year for both the in- and out-of-hospital populations.180,181 Thus for patients who remain comatose after cardiac arrest, it is not clear whether hypothermia improves outcomes, but clearly fever should be avoided. Thus it is currently recommended to maintain 5 days of normothermia (36°C to 37.5°C) or 2 days of hypothermia (32°C to 34°C) followed by 3 days of normothermia.41

Prognostication Post Arrest Practitioners must consider multiple factors when trying to predict outcome because no sole criterion for prognostication of neurologic outcome and survival after cardiac arrest exists. Many factors have been associated with poor neurologic outcomes and/or increased mortality after pediatric cardiac arrest, including age greater than 1 year, longer duration of CPR,182 persistent hypotension after resuscitation,169 persistent lactate level elevation after 12 hours,183 high and persistently elevated levels of serum neuronal biomarkers

(neuron-specific enolase and S100B),184 poor pupillary response at 12 to 24 hours,182 and discontinuous or isoelectric tracing on electrocardiogram within 7 days of arrest.185

Conclusion In conclusion, over the past two decades survival from pediatric cardiac arrest has improved dramatically, particularly in the inhospital setting, from 15% to nearly 50%. The reasons for this are multifactorial, and include but are likely not limited to the use of ECPR, personalizing the care delivered to that child’s age and also the child’s underlying cardiac anatomy and physiology, and applying a multitude of approaches to improving the quality of care we deliver. Teams are now systematically addressing the quality of CPR delivered during the event with real-time feedback with bedside devices and quality CPR coaches, postevent debriefing, and meticulously managing the PCAC period. Unfortunately, there is evidence of great variability between hospitals, suggesting there are still lives to be saved. In the upcoming decades we need to continue to explore physiologic targets to optimize coronary and cerebral perfusion while minimizing harm from compressions, use human factors to improve team dynamics and room ergonomics, refine approaches to PCAC, and improve our ability to prognosticate on outcomes. Although avoiding a cardiac arrest is always best, we now know that many lives can be saved with exquisite CPR, and we must continue to focus on optimizing outcomes for these most vulnerable of patients.

References A complete list of references is available at ExpertConsult.com.



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CHAPTER 31  Cardiopulmonary Resuscitation (CPR) in Children With Heart Disease

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