995© Springer International Publishing Switzerland 2017 A. Dabbagh et al. (eds.), Congenital Heart Disease in Pediatric and Adult Patients, DOI 10.1007/978-3-319-44691-2

AA band, 88Abciximab, 180Abdominal situs, 360Abnormal aortic origin of the coronary

arteries (AAOCA), 732–733Abnormal clot

generation, 817–818stability, 819

Abnormal left coronary artery of the pulmonary artery (ALCAPA), 727–728, 730–732

Abnormal right coronary artery of the pulmonary artery (ARCAPA), 732

Absent pulmonary valve syndrome, 500–501

Absolute refractory period, 74Accessory muscle, 319Acetaminophen, 163ACHD. See Adult congenital heart disease

(ACHD)Acid-base balance

pathophysiology in postoperative surgerymechanisms, 931–932metabolic acidosis, 932–934metabolic alkalosis, 934–935respiratory acidosis, 935–936respiratory alkalosis, 936

physiology, 896–897practical approach to management of,

938–942Acidosis, 587Acquired coronary disease

coronary artery allograft vasculopathy, 734inflammatory disease, 733–734

Actin, 84Action potential

cardiac cells, 72–73cardiac pacemaker cells, 72–77

automaticity, 75–77Ca2+ channels, 72Ca2+ clock theory, 76coupled clock theory, 77M-clock theory, 75phase 0, 73phase 2, 73–74phase 3, 73–74phase 4, 73refractory period, 74–75resting membrane potential, 72

electrical network, 71in myocardial cells and pacemaker

cells, 154in normal myocardial cells, 154

Activated clotting time (ACT), 348Activated factor VII (factor VIIa), 818Activated partial thromboplastin time

(aPTT), 328, 330, 332Acute kidney injury (AKI)

aetiology and pathogenesis, 905–906

AKIN classification system, 911biomarkers

cystatin C, 913interleukin 18, 913neutrophil gelatinase-associated

lipocalin, 912–913CPB

multiorgan dysfunction, 906–907physiopathology, 907ultrafiltration, 907–909

Index

996

Acute kidney injury (AKI) (cont.)definitions, 909–910epidemiology, 904–905multi-societal database committee for

pediatric and congenital heart disease classification, 912

pRIFLE classification system, 911RIFLE classification system, 909, 911

Acute rheumatic fever (ARF), 276Acyanotic congenital heart disease

coarctation of the aorta, 372–374congenitally bicuspid aortic valve,

371–372congenitally corrected transposition of the

great vessels, 370–371dextrocardia, 370primary pulmonary hypertension, 374–376pulmonic stenosis, 374

Adenosine, 160Adenosine diphosphate (ADP) receptor

inhibitors, 171Adrenergic receptor antagonists, 147Adult congenital heart disease (ACHD)

airway and ventilatory management, 388–389

antibiotic prophylaxis, 390–391arrhythmias, 391epidemiology, 366hematocrit and perioperative transfusion

management, 390intravenous access considerations, 389mixing lesions, 368–369obstructive/stenotic lesions, 369pacemakers, 391perioperative fluid management and NPO

intervals, 389–390perioperative monitoring, 388postoperative considerations, 391–392regurgitant lesions, 369shunt lesions, 367–368syndromes and associated anomalies,

387–388Adult heart surgeon vs. pediatric heart

surgeon, 401, 403Adult hemostasis vs. neonatal hemostasis,

328–329Adventitious/abnormal sounds, 321–322Afferent limb, 101, 103–104, 106–107Afterload, ESV, 96Alagille syndrome, 54α-adrenergic agonists, used during CPR,

966–967Alpha-2 adrenergic agonists, 149Alpha blockers, 147–148Alpha-mediated vasoconstrictors, 497

Alprostadil, 140–141, 144Ambrisentan, 151American Board of Anesthesiology (ABA), 6American Board of Medical Specialties

(ABMS), 6American Heart Association, 202American Society of Anesthesiology (ASA)

score, 213Amiodarone

dosing and indication, 158usage during CPR, 972–973

Amlodipine, 146Amplatzer Duct Occluder, for patent ductus

arteriosus, 660Analgesic agents, 154–155, 162–163Anesthesiology, for pediatrics,

origins of, 5–6Anesthetic agents, 203–204

in cardiac catheterization, 206CNS monitoring, 310volatile, 165, 206

Angiogenesis, 38Angiography, 361–362Angiomas, 52Angiotensin-converting enzyme (ACE)

inhibitors, 142, 145Angiotensin II receptor antagonists

(ARBs), 142, 145Anomalous left coronary from the pulmonary

artery (ALCAPA), 585Anomalous mitral arcade, 578Antiarrhythmic agents, 153, 156–161

Vaughan Williams classification, 155Antibiotic prophylaxis, 390–391

in perioperative period, 178, 183–185Anticoagulants, oral, 170–171Antifibrinolytic agents, 178, 180, 183Antifibrinolytics, 815–817, 819Antihypertensive Agents, 142Antiplatelet agents, 171, 178–180Aorta, coarctation of.

See Coarctation of the aorta (CoA)Aortic arches, 38–41

hypoplasia, 628–629, 680Aortic channel, 30Aorticopulmonary septum, 30Aortic outflow tract obstruction

(AOTO), 680surgical repair of DOLV with, 692

Aortic sac, 39Aortic stenosis (AS), 595

calcific bicuspid, 372forms of, 642of neonate, 595–596, 600–604valvular (see Valvular aortic stenosis)

Index

997

Aortic valvebicuspid disease, 365lesion, 627–628stenosis, 270

Apixaban, 174APTEM test, 339aPTT. See Activated partial thromboplastin

time (aPTT)Argatroban, 177Arrhythmias, 391, 603Arrhythmias, postoperative care for

bradyarrhythmias, 776surgical procedures associated with

atrial septal defect closure, 776–777atrioventricular septal defect repair,

777–778correction of transposition of the great

arteries, 780repair of univentricular heart, 779–780tetralogy of Fallot correction, 778–779ventricular septal defect closure, 777

tachyarrhythmiacause of, 770supraventricular tachycardia, 770–773ventricular tachycardia, 773, 775

Arrhythmogenic right ventricular cardiomyopathy/dysplasia, 273–274

Arterial blood, systemic desaturation of, 482Arterial cannulation, 426, 543–546Arterial line, 230–231Arterial stenosis, detection of, 300Arterial switch procedure, 526–527, 538Arterial system

aortic arches, 38arterial tree development, 39–41

Arterial-venous difference, cerebral, 305Arteriovenous malformations (AVMs), 298,

387AS. See Aortic stenosis (AS)ASD. See Atrial septal defect (ASD)Aspirin, 179Asplenia, 370Asymmetrical valve, parachute-like, 578Atenolol, 157Atracurium, 166Atresia, pulmonary, 498–500Atrial development, 19–22Atrial septal defect (ASD), 26, 366, 371,

377–378, 629anesthesia for ASD treatment, 468cardiac CT, 467cardiac MR, 467catheterization studies, 467classification, 460–462

ASD I, 461

ASD II, 461CSSD, 461PFO, 462SVASD, 461

clinical presentation and the course of the disease, 463–464

CXR, 464–465detection of, 466–467diagnosis, 463–464echocardiography, 466–467electrocardiogram, 262–263electrocardiography, 464embryology, 459–460exercise intolerance, 464imaging techniques in diagnosis of, 465intracardiac echocardiography for,

214–215pathophysiology, 460post-intervention assessment of, 466postoperative arrhythmias, surgical

procedures with, 776–777pulmonary hypertension, 464treatment, 467–468

Atrial septostomy, 524balloon, 362

Atrial syncytium, 69Atrial tachycardia (AT), 771–774Atrioventricular block (AVB), 255–256, 776Atrioventricular (AV) canal, 26–27Atrioventricular (AV) node, 32–34

development of, 32–34reentrant tachycardia, 773–774

Atrioventricular reciprocating tachycardia (AVRT), 773–774

Atrioventricular septal defect (AVSD), 28, 445

anatomy of DORV with, 677anesthetic management, 453–454associated cardiac anomalies, 450cardiac anatomy and embryology,

445–447cardiac morphology and physiology,

446–447classification, 450clinical presentation, 678

and diagnosis, 451–452complete AVSD, 450–451echocardiography, 452electrocardiogram, 264outcome, 455partial AVSD, 450pathologic findings and associated

anomalies, 447–449postoperative care for, 454reoperation, 454

Index

998

Atrioventricular septal defect (AVSD) (cont.)repair with surgical procedures,

postoperative arrhythmias, 777–778surgical repair, 452–453

Atrioventricular (AV) valveanomalies, 35and chordae tendineae, 28cushions development, 28development, 27–28

Atrium, septum development, 24–25Atropine, usage during CPR, 968–969Atypical congenital mitral stenosis, 576Auditory evoked potential (AEP), 311Auscultation, 320, 359Automated external defibrillation

(AEDs), 963Automaticity, of pacemaker cells, 75–77Autonomic control of, heart

ANS functions, 90β1-and M2 receptor activation, 91–92sympathetic and parasympathetic

receptors, 90Autonomic nervous system (ANS)

function, 89–90AVMs. See Arteriovenous malformations

(AVMs)AVSD. See Atrioventricular septal defect

(AVSD)Ayer, Philip, 6

BBacterial endocarditis, 371Bainbridge reflex, 102–103Balloon atrial septostomy, 362Balloon valvuloplasty, 371–372, 557, 603Baroreceptor reflex/carotid sinus reflex,

101–102BAV. See Bicuspid aortic valve (BAV)Benzodiazepines, in cardiac catheterization, 207Berlin Heart, for pediatric patients, 751–752β-adrenergic agonists, used during CPR,

966–967Beta blockers, 147–148β-myosin, 94β-receptor, 51

during neonatal circulation, 68β1 receptor activation

chronotropic effects, 91–92inotropic effects of, 91lusitropic effects, 92

Bezold-Jarisch reflex (BJR), 103–104Bicuspid aortic stenosis, 372

calcific, 372

Bicuspid aortic valve (BAV), 618, 627disease, 365

Bifascicular block, 259Bilateral dorsal aortae, 39Bioimpedance cardiac monitors, 238–239Biomarkers for AKI

cystatin C, 913interleukin 18, 913neutrophil gelatinase-associated lipocalin,

912–913Bispectral analysis index (BIS), 310Bivalirudin, 177Biventricular hypertrophy (BiVH),

254–255Biventricular parallel circulation pattern,

50–51Blalock, Alfred, 7Blalock-Hanlon procedure, 521Blalock-Taussig shunt (BTS), 380, 386,

486, 506anesthesia issues of the patient with, 508

Bleedinggastrointestinal, 123in postoperative care (see Postoperative

bleeding)renal, 123

Blood conservation, 414–415Blood flow, mechanisms of, 959–960Bloodless cardiac surgery, 414–415Blood pressure, 229

early postnatal period, 51during neonatal circulation, 68

Blood pump, CPB, 430–431Blood-related products, 181–182Blood vessels, development of, 38Blue boy, 7Bosentan, 151Bovine aortic arch, 629Branchial arches, 38Bronchial sounds, 321Bronchospasm, 122Bronchovesicular sounds, 321Brukhonenko, Sergei, 8BTS. See Blalock-Taussig shunt (BTS)Bulbus cordis, 16Bumetanide, 146Bundle branches, 34

CCa2+ channels, 72Ca2+ clock theory, 76CAD. See Coronary artery disease (CAD)Calcific bicuspid aortic stenosis, 372

Index

999

Calcium channel blockers (CCBs), 145–146Calcium chloride, 133Calcium (Ca2+) homeostasis, 92–93

excitation-contraction coupling by, 77–79

Calcium-induced calcium release (CICR), 78

Calcium, usage during CPR, 970–971Calmodulin, 79Cannulation, arterial, 426Capnography, 222, 324–325Capnometry, 324–325Captopril, 145Cardiac action potential, 93Cardiac and congenital surgery, for pediatrics,

6–8Cardiac anomaly, 370, 629

AVSD, 450of coarctation of the aorta, 627–629congenital, 502

Cardiac arrest, 124Cardiac arrhythmias, postoperative care for

bradyarrhythmias, 776surgical procedures associated with

atrial septal defect closure, 776–777atrioventricular septal defect repair,

777–778correction of transposition of the great

arteries, 780repair of univentricular heart, 779–780tetralogy of Fallot correction, 778–779ventricular septal defect closure, 777

tachyarrhythmiacause of, 770supraventricular tachycardia, 770–773ventricular tachycardia, 773, 775

Cardiac catheterization, 361–362, 561anesthetic agents, 206anesthetic considerations, 203–204benzodiazepines, 207congenital mitral regurgitation, 585dexmedetomidine, 208goals of, 201–202hybrid procedures, 209intraoperative management, 204–206ketamine, 208laboratory

complications, 208–209environment, 200–201intracardiac echocardiography,

214–215radiation safety, 201transthoracic echocardiography,

215–216

opiate medications, 207preoperative assessment, 204propofol, 206–207vascular access and approach, 202–203ventricular septal defect, 474volatile anesthetic agents, 206

Cardiac cells, action potential in, 72–73Cardiac conducting system (CCS)

AV node, 32–34bundle branches and His bundle, 34Purkinje fiber, 34sinoatrial node, 32

Cardiac crescentcardiogenic mesoderm specification, 12heart fields establishment, 12–13heart tube, 13–15

(see also Heart tube)progenitor cells development, 11–12

Cardiac CT, atrial septal defect, 467Cardiac cushion, cellular and molecular

factors, 27Cardiac cycle, 94–95Cardiac jelly, 14, 23Cardiac loop, 15–18Cardiac magnetic resonance imaging (cMRI),

361congenital mitral regurgitation, 584

Cardiac MR, atrial septal defect, 467Cardiac output, 51, 97, 234

during neonatal circulation, 68Cardiac pacemaker cells, action potential in,

72–77automaticity, 75–77Ca2+ channels, 72Ca2+ clock theory, 76coupled clock theory, 77M-clock theory, 75phase 0, 73phase 2, 73–74phase 3, 73–74phase 4, 73refractory period, 74–75resting membrane potential, 72

Cardiac reflexesbainbridge reflex, 102–103baroreceptor reflex/carotid sinus reflex,

101–102Bezold-Jarisch reflex, 103–104chemoreceptor reflex, 104–105Cushing reflex, 106oculocardiac reflex, 106valsalva maneuver, 105

Cardiac surgery, 327protocol of test, 350

Index

1000

Cardiac surgery, in pediatric patientsanalysis of program assistance

biomedical engineering support, 988–989

on-site surgical and interventional activity, 986–987

role of education, 987–988cardio-thoracic surgery network, 982collection of data, 990–991consequences of chronic congenital heart

disease, 989data monitoring, 991–993human resources, 983infant mortality rate, 981sustainable congenital heart center

developmentmodels of assistance, 983–985pediatric assistance program, 985–986risk adjustment for congenital heart

surgery model, 986–987teamwork culture with nurse empowerment

development and horizontal hierarchy, 990

Cardiac tachyarrhythmia. See TachycardiaCardiac transplantation, 562Cardiac tumors, 277

electrocardiogram, 277Cardiac work

cardiac output, 97ejection fraction, 97–100stroke volume, 96–97

Cardinal veins, 47inflow by, 14

Cardiomegaly, 360, 464, 474Cardiomyopathy

arrhythmogenic right ventricular cardiomyopathy/dysplasia, 273–274

dilated, 273hypertrophic, 273restrictive, 274–275

Cardiopulmonary bypass (CPB), 298, 331, 375

for AKImultiorgan dysfunction, 906–907physiopathology, 907ultrafiltration, 907–909

blood characteristics during, 426–428blood pumps, 430–431components of, 425controlling the host response, 437–438machine, 200metabolism during, 434–436oxygenator, 431–432

for postoperative bleeding, 809–811primary function of, 434priming and hemodilution, 432–434systemic inflammation during, 436–437tubing in, 429–430vascular access, 426–429

Cardiopulmonary interaction, 322–323Cardiopulmonary resuscitation (CPR), 122

cardiovascular physiology and shock, 954endotracheal medication administration,

964with hyperkalemic cardiac arrest, 975mechanics of, 955

airway, 956breathing, 956–958circulation, 957, 959

medications used duringadministration of glucose, 971–972α-and β-adrenergic agonists, 966–967atropine, 968–969calcium administration, 970–971epinephrine, 967lidocaine, 973–974role of amiodarone, 972–973sodium bicarbonate, 969–970vasopressin, 968

monitoring during, 964–966perioperative cardiac arrest, 974–975pulmonary vascular resistance and blood

flow regulation, 954–955rate and duty cycle, 960vascular access and fluid administration,

964Cardio-thoracic surgery network (CTSNet),

982Cardiovascular physiology and shock, 954Carotid artery, 40Carotid duct, 40Carotid sinus reflex, 101–102Carvedilol, 148Catheter

based ligation, 408insertion, addendum on invasive

monitoring, 542Caudal pole, 12Cedars algorithm, mechanical circulatory

support for single-ventricle patients, 753–754

Cefazolin, 185Cefotaxime, 185Cefuroxime, 185Celiac arterial trunk, 42Cellular model, of coagulation, 328

Index

1001

Central diabetes insipidus (DI), 856–857Central nervous system (CNS), CoA, 630Central nervous system management, in

postoperative careclassification of deficits, 834impact of outcome, in pediatric cardiac

surgery, 829intraoperative risk factors for injuries in

neonates and children, 830, 833–834

neurocognitive and neurodevelopmental disorders, 838–839

neurologic injuries, 836–838postoperative risk factors for injuries in

neonates and children, 834–836preoperative risk factors for injuries in

neonates and children, 830–832white matter injuries with periventricular

leukomalacia, 839–844Central nervous system monitoring

anesthesia level, 310electroencephalography

amplitude integrated, 296–297applications in perioperative period,

289–290considerations, 29610/20 electrode system, 290–294history, 289interpretation, 294limitations, 295–296report, 295

evoked potentials, 310–311jugular oxygen saturation, 305

anatomic approach, 306–308catheters and technique, 306limitations, 308–309

near infrared spectroscopy, 284–289pain assessment, 280–282sedation assessment, 282–284, 310transcranial Doppler

indications, 299–300limitations, 303principles, 297–299signal acquisition of, 300–303

Central venous lines, 231–233Cephalad pole, 12Cerebral metabolic rate for oxygen (CMRO2),

435–436Chamber hypertrophy

biventricular, 254–255left ventricular, 253–254right ventricular, 252–253

Chemoreceptor reflex, 104–105

Chest pain, 358Chest physiotherapy, postoperative

respiratory management, 797Chest radiography, 360

congenital mitral regurgitation, 582–583

congenital mitral stenosis, 582Chest X-ray (CXR), 360, 490,

564, 625atrial septal defect, 464–465ventricular septal defect, 474

Child life counseling, 417Children’s Hospital of Eastern

Ontario Pain Scale (CHEOPS), 280–281

Children’s Hospital of Pennsylvania (CHOP), 4

Children’s hospitals, 4–5Chlorothiazide, 147Chordae, 574Chromosomal anomalies, 630–631Chronic cyanosis, 390Chronic hypoxemia, 486Chronic obstructive lesions, 369Chronic postsurgical pain

definition, 871–872management of CPTP and post-sternotomy

pain, 875–876neuropathic pain, 872pain after sternotomy, 873–874pain after thoracotomy, 874–875

Chronic post-thoracotomy pain (CPTP), 874–875

Chronic respiratory failure and tracheostomy, 797–798

Circulatory alterationsafter birth, 50–52before and after birth, 49–50

Cisatracurium, 166Classic septum, 24Clindamycin, 185Clonidine, 149, 164Clopidogrel, 179Clot firmness, in postoperative bleeding

treatmentcryoprecipitate, 820fibrinogen concentrate, 820fresh frozen plasma, 821packed red blood cells, 821–822platelets, 820–821ROTEM tracing, 819

cMRI. See Cardiac magnetic resonance imaging (cMRI)

Index

1002

CoA. See Coarctation of the aorta (CoA)Coagulation

cellular model of, 328clinical evaluation and preoperative

laboratory testing, 330congenital heart disease and, 329–330stages, 328, 333–335

Coagulation factors (cF), 328, 332Coagulation monitoring, 327

integration in clinical care, 347–351laboratory-based coagulation test, 331–333TEG®/ROTEM®, 333

Coagulation test, 331–333Coagulopathy, 331Coarctation of the aorta (CoA), 53–54,

372–374, 617–618anesthesia for

intraoperative anesthesia management, 637–640

postoperative care, 640preoperative evaluation, 636–637

cardiac anomalies of, 627–629clinical features, 622

adult features, 625infancy period, 624neonatal period, 623–624older childhood and adolescence

period, 624–625prenatal period (fetal period),

622–623clinical outcome, 641–642echocardiography, 626echo protocol, 626–627electrocardiogram, 271embryology and anatomic features,

618–619etiology and mechanism of the disease,

619–620noncardiac anomalies of, 630–631pathologic findings, 620–622TGA with, 532–533therapeutic approaches, 632

balloon dilatation, 633–634stenting, 634–636surgical correction, 632–633

Colloids, 916–918Color-flow Doppler echocardiography, 583COMFORT Scale, 281–282Compliance, resistance, oxygenation, and

pressure (CROP) index, 791Computerized tomographic angiography

(CTA), 606for coronary artery anomalies, 736

Computerized tomography (CT), 361

Conal septal deviationTaussig-Bing anomaly, 537–538TGA/VSD and LVOT obstruction,

534–536Conduction abnormalities

atrioventricular block, 255–256intraventricular conduction defect,

256–259Conduction intervals

PR interval, 248–249QT interval, 249–250

Conduction system, anatomy of DOLV, 689Conductive cells, 32, 71Congenital anomaly

cardiac, 502noncardiac, 503

Congenital cardiac diseasemedical history, 357–358para clinical diagnostic tests

cardiac catheterization and angiography, 361–362

chest radiography, 360cMRI, 361computerized tomography, 361echocardiography, 360–361electrocardiography, 360

physical examination, 358–359signs and symptoms, 359

Congenital cardiac structural defects categories, 366–367

Congenital heart disease (CHD)aortic valve stenosis, 270atrial septal defect, 262–263atrioventricular septal defect, 264coarctation of aorta, 271Ebstein’s anomaly, 270great arteries, transposition of, 271–273lifetime surgical impact of, 407patent ductus arteriosus, 264–266pulmonary valve atresia, 268pulmonary valve stenosis, 267–268tetralogy of Fallot, 269total anomalous pulmonary venous

connection, 267tricuspid atresia, 267truncus arteriosus, 267ventricular septal defect, 264

Congenital heart patientevaluation coordination, 399–400guiding care for, 407referral and evaluation of, 399surgical and cardiologic interventional

issues, 401–403Congenitally bicuspid aortic valve, 371–372

Index

1003

Congenitally corrected transposition of the great arteries (CCTGA), 271

Congenital mitral regurgitationclinical features, 580–581diagnosis, 582–585

Congenital mitral stenosis, 579–580chest radiography, 582echocardiography, 582

dynamic 3D transthoracic, 582transesophageal, 582

electrocardiography, 581Congenital mitral valve anomalies, 573

anesthetic management, 587–588classification, 574–576

anatomical, 574, 576complex congenital MV lesions,

577–579surgical, 577

clinical features, 579congenital mitral regurgitation,

580–581congenital mitral stenosis, 579–580

diagnosis, 581congenital mitral stenosis, 581–582

embryology, 573–574management, 586

timing of surgery, 586mitral regurgitation, 589surgical outcomes, 590–592surgical techniques, 589–590

Congenital pulmonic valve stenosis, 555anatomy, 555–556anesthetic considerations, 557–558presentation, 556–557treatment, 557

Conotruncal defects, 79disorders due to, 13

Conotruncal ridge, 30Conotruncal segment, 16–18Continuous renal replacement therapy

(CRRT)advantages and disadvantages, 926clot formation, 927conventional criteria for, 923–924ECMO, 928extracorporeal blood purification therapy,

926haemofiltration and haemodialysis, 927vs. peritoneal dialysis, 926

Contractile function, 93–94. See also Mechanical (contractile) function

Contractile proteins, 81myosin, 82–83titin, 83–84

Contractile system, fetal cardiac. See Fetal cardiac contractile system

Contractility, 96Conus cordis, 30–31Conus cordis/conus arteriosus, 16Conventional coagulation test, 333–334Conventional surgical technique, 408Coronary arteries

DOLV anatomy, 688–689double-outlet right ventricle (DORV)

anatomy, 676Coronary arteries formation, 44–45Coronary artery allograft vasculopathy

(CAAV), 734Coronary artery anomalies (CAA), 501

ALCAPA, 727–728anatomical illustration of, 727–728anesthesia, 741case studies, 742classifications, 729–730clinical algorithm, 738–739echocardiography, 734

catheterization laboratory, 738computerized tomographic

angiography, 736MRI, 737–738stress sestamibi perfusion scan, 735transesophagic echocardiography, 735,

737transthoracic echocardiography,

735–736EKG, 734embryology, 729outcomes, 741–742pathology

AAOCA, 732–733acquired coronary disease, 733–734ALCAPA, 730–732ARCAPA, 732coronary fistulas, 733myocardial bridges, 733stenosis/atresia of the left main

coronary artery, 733surgical management, 738–740

Coronary artery disease (CAD), 372Coronary perfusion, 528, 559Coronary sinus septal defect (CSSD), 461Coronary sinus septum, 25Coupled clock theory, 77CPBCardiopulmonary bypass (CPB)Crista terminalis, 21Critical aortic stenosis

of neonate, 595–596, 600–604surgical techniques for the repair of, 602

Index

1004

Cryoprecipitate, 182Crystalloids, 918–919CSSD. See Coronary sinus septal defect

(CSSD)Cumulative stressors, 417Cushing reflex, 106CXR. See Chest X-ray (CXR)Cyanosis, 359, 482Cyanotic congenital cardiac disease, 482Cyanotic congenital heart disease, 379

Eisenmenger syndrome, 384–385single-ventricle physiology and complex,

385–387tetralogy of Fallot, 379–381transposition of the great arteries, 382–384

Cyanotic heart disease, 330, 366Cyclooxygenase (COX) inhibitors, 408Cystatin C, for AKI, 913Cystic medial necrosis, 621Cytokines, proinflammatory, 437Cytomegalovirus (CMV), 390Cytosol proteins, 79

DDabigatran, 173Daily fluid balance, monitoring of, 920Dalteparin, 175Deep hypothermia with circulatory arrest

(DHCA), 431, 435–436Defibrillation. See Electric countershockDefinitive kidney, 43De Motu Cordis, 7Dendritic cells (DCs), 126–127Dennis, Clarence, 8Desensitization, of platelets, 437Desflurane, 165Desmopressin, 182Dexmedetomidine, 164

in cardiac catheterization, 208Dextrocardia, 370

isolated, 360DHCA. See Deep hypothermia with

circulatory arrest (DHCA)Diabetes mellitus (DM), 357

perioperative management fordefinition, 857for emergency surgery, 859–860glucose monitoring, 858intraoperative care, 858–859postoperative care, 859treatment with insulin, 858types, 857

Diastole, 95

Diastolic dysfunction, 78Diastolic filling (phase 1), 95DiGeorge syndrome, 388, 390, 494Digoxin, 133, 161Dilated cardiomyopathy, 273Diltiazem, 160Dipyridamole, 180Direct thrombin inhibitors, 176Disopyramide, 156Diuretics, 145–146Dobutamine, 132

contraindications, 139dosage, 138indications, 138mechanism of action, 138pharmacological properties, 136for right ventricle failure, 722side effects, 138

Dodrill, Forest Dewey, 8Dodrill-GMR heart pump, 9Dofetilide, 158Dopamine, 132

adverse effects, 129clinical effects of, 125–126considerations for children, 129dose and administration, 127, 129immunologic and neurohormonal effects,

126–128indications, 127mechanism of effect, 124–125precautions, 129for right ventricle failure, 723time of effect, 127warnings and contraindications, 129

DORV. See Double-outlet right ventricle (DORV)

Double-outlet left ventricle (DOLV), 686anatomy

conduction system, 689coronary arteries, 688–689patent ductus arteriosus, 688right-and left-sided obstructive

lesions, 688ventricular septal defect, 687–688

clinical cases, 693–694clinical presentation

DOLV with noncommitted/remote VSD, 691

right ventricular dysfunction, 691subaortic VSD with/without POTO,

689–690subpulmonic VSD with/without

AOTO, 690with supracristal VSDs, 690

Index

1005

clinical vignettes, 685–686embryology, 686–687outcomes, 692–693surgical repair

aortic outflow tract obstruction, 692patent pulmonary outflow, 692single-ventricle palliation, 692with subaortic VSDs, 691

Double-outlet right ventricle (DORV), 35, 538, 672

anatomywith atrioventricular septal defect, 676classification, 674–675coronary arteries, 676with doubly committed VSD and PS,

675with remote VSD with/without PS,

676with subaortic VSD, 674–675with subpulmonary VSD with/without

PS, 675–676anesthetic management of clinical cases

TOF-type DORV, 681VSD-type DORV, 680–681

clinical presentationwith AVSD, 678with remote VSD, 678TGA-type, 677VSD-type and TOF-type, 677

clinical vignettes, 671–674embryology, 673–674outcomes, 680surgical repair

TGA-type DORV with and without PS, 679

VSD-type and TOF-type, 678–679Doubly committed VSD

anatomy of DORV with, 674–675and PS, 675

Down’s syndrome, 387D-TGV. See D-transposition of the great

vessels (D-TGV)D-transposition of the great arteries (D-TGA),

382–383, 518, 532D-transposition of the great vessels (D-TGV),

370Duchenne muscular dystrophy (DMD), 275Ductal theory, 53, 620Ductus arteriosus, 40

abnormalities of, 52during fetal circulation, 66during neonatal circulation, 67saturated and desaturated blood

mixture, 50

Ductus tissue theory, 53Ductus venosus, during fetal

circulation, 66Duty cycle, 960Dysrhythmia ECG, 764Dystrophies

Duchenne muscular, 275myotonic muscular, 275

Ee-Aminocaproic acid (EACA), 815–817Ebstein’s anomaly, 270, 371, 381–382, 566

anatomy, 567anesthetic considerations, 569–570presentation, 567–568treatment, 568–569

Ebstein’s malformation, of mitral valve, 574

ECG. See Electrocardiography (ECG)Echocardiography

atrial septal defect, 466–467coarctation of the aorta, 626congenital cardiac disease, 360–361congenital mitral stenosis, 582coronary artery anomalies, 734

catheterization laboratory, 738computerized tomographic

angiography, 736MRI, 737–738stress sestamibi perfusion scan, 735transesophagic echocardiography,

735, 737transthoracic echocardiography,

735–736for pulmonary hypertension, 700for right ventricle failure, 719–720transesophageal, 361transthoracic, 452ventricular septal defect, 474–476

ECMO. See Extracorporeal membrane oxygenation (ECMO)

Edema, pulmonary, 360, 562Edwards SAPIEN valve, 410–411EFE. See Endomyocardial fibroelastosis

(EFE)Efferent limb, 102, 104–107EIH. See Exercise-induced hypertension

(EIH)Eisenmenger syndrome, 366, 368, 375,

384–385Ejection fraction (EF), 97–100EKG, for coronary artery anomalies, 734Electric countershock, 960–961

Index

1006

Electrocardiogram (ECG)cardiac tachyarrhythmias

supraventricular tachycardias, 260ventricular tachycardias, 260–262

cardiac tumors, 277cardiomyopathy

arrhythmogenic right ventricular cardiomyopathy/dysplasia, 273–274

dilated, 273hypertrophic, 273restrictive, 274–275

chamber hypertrophybiventricular, 254–255left ventricular, 253–254right ventricular, 252–253

conduction abnormalitiesatrioventricular block, 255–256intraventricular conduction defect,

256–259conduction intervals

PR interval, 248–249QT interval, 249–250

congenital heart diseaseaortic valve stenosis, 270atrial septal defect, 262–263atrioventricular septal defect, 264coarctation of aorta, 271Ebstein’s anomaly, 270great arteries, transposition of, 271–273patent ductus arteriosus, 264–266pulmonary valve atresia, 268pulmonary valve stenosis, 267–268tetralogy of Fallot, 269total anomalous pulmonary venous

connection, 267tricuspid atresia, 267truncus arteriosus, 267ventricular septal defect, 264

dystrophiesDuchenne muscular dystrophy, 275myotonic muscular dystrophy, 275

heart rate, 246inflammatory conditions

acute rheumatic fever, 276Kawasaki disease, 275–276myocarditis, 276pericarditis, 276

morphologyP wave, 250QRS complex, 250–251ST segment, 251–252T wave, 251

QRS axis, 247–248rhythm, 247

Electrocardiography (ECG), 360atrial septal defect, 464congenital mitral regurgitation, 583congenital mitral stenosis, 581for pulmonary hypertension, 699ventricular septal defect, 474

10/20 Electrode system, 290–294Electroencephalography (EEG)

amplitude integrated, 296–297applications in perioperative period,

289–290considerations, 29610/20 electrode system, 290–294history, 289interpretation, 294limitations, 295–296report, 295spectrogram, 296

Electrolyte managementcalcium levels, 930magnesium, 930–931physiology

bicarbonate, 896calcium, 894–895chloride, 895ECS, plasma and ICS fluid

composition, 891–893Gamble gram, 893ionic balance, 892magnesium, 895phosphorus, 895potassium, 894sodium, 893–894

potassium levels, 929–930sodium overload, 928–929

Electrophysiologic devices, 412–414Emblem S-ICD subcutaneous

defibrillator, 412Embolism, detection of, 300Embryology, cardiovascular system

angiomas, 52arterial system

aortic arches, 38arterial tree development, 39–41

atrial development, 19–22atrioventricular valve, 27–28

anomalies, 35cardiac conducting system

AV node, 32–34bundle branches and His bundle, 34Purkinje fiber, 34sinoatrial node, 32

cardiac crescent, 11–13cardiac loop, 15–18

Index

1007

circulatory alterationsafter birth, 50–52before and after birth, 49–50

coarctation of the aorta, 53–54coronary arteries formation, 44–45ductus arteriosus abnormalities, 52heart tube formation, 13–15intersegmental branches, 43lateral branches development, 42–43limb arteries formation, 44lymphatic system

anomalies, 56–57formation of, 48

lymph sacs and lymphatic ducts, 48–49

outflow tract septation anomaliesgreat vessels transposition, 36tetralogy of Fallot, 37truncus arteriosus, 36

semilunar valveformation, 31stenosis of, 35

septum developmentatrioventricular canal, 26–27interventricular septum, 29primitive atrial septum, 24–25truncus arteriosus and conus cordis,

30–31sinus venosus, 18–19thoracic duct development, 49vascular formation, 38vena cava anomalies

inferior vena cava, 54–55superior vena cava, 55–56

venous system formationinferior vena cava, 47–48

vitelline and umbilical arteries, 42Emotional health dysfunction, 280Enalapril, 145End-diastolic volume (EDV), 96Endocardial cushions, 23–24, 387Endocarditis, bacterial, 371Endocardium, 14Endocrine problems, perioperative

management forin diabetic patients

definition, 857for emergency surgery,

859–860glucose monitoring, 858intraoperative care, 858–859postoperative care, 859treatment with insulin, 858types, 857

patients on steroids requiring surgeryacute physical/emotional stresses, 852dosing glucocorticoids, 854with non-suppressed HPA axis,

852–853surgical procedures, 854–855time period for impaired HPA axis, 852with uncertain status of HPA axis, 853using inhaled steroids, 853using intra-articular or spinal

glucocorticoid injections, 854using topical steroids, 853

in patients with metabolic disorder, 860–861

fatty acid oxidation defects, 864homocystinuria, 864hypoglycemia, 861maple syrup urine disease, 863methylmalonic and propionic

academia, 862–863mucopolysaccharidosis, 864–865phenylketonuria, 861urea cycle defects, 863–864

pituitary gland incentral adrenal insufficiency, 855–856central diabetes insipidus, 856–857central hypothyroidism, 856

Endomyocardial fibroelastosis (EFE), 600Endothelin receptor antagonists, 701–702, 704Endothelin receptor blockers (ET blockers),

153End-systolic volume (ESV), 96–97Enflurane, 165Enoxaparin, 177Epicardial cells, 44Epicardial echocardiography, perioperative

imaging, 223–224Epicardium, 14Epinephrine, 132

administration routes, 123cautions, 124dosage, 123–124indications, 122–123mechanism of effect, 122usage during CPR, 967

Epithelial-to-mesenchymal transition, 44Epoprostenol, 150Epsilon-aminocaproic acid, 183Eptifibatide, 180Erythrocytosis, 390Esmolol, 148, 157Esomeprazole, 170Ethacrynic acid, 146Etienne-Louis Fallot, 7–8

Index

1008

Etomidate, 164Evoked potentials, 310–311Excitation-contraction coupling (ECC)

calcium homeostasis, 77–79functioning organelles of, 79modulating mechanisms, 80

Exercise-induced hypertension (EIH), 635EXTEM test, 338Extracellular matrix (ECM), 14Extracorporeal CO2 removal (ECCO2R), 796Extracorporeal membrane oxygenation

(ECMO), 604, 610Extubation

criteria, 791–792failure, 793procedure, 792–793success, 791

FFace, Legs, Activity, Cry, Consolability Scale

(FLACC), 281Factor Xa inhibitor, 176Famotidine, 170Femoral (FV) vein, 231Fenoldopam, 144Fentanyl (opioid), 162Fetal cardiac contractile system

Ca2+ homeostasis, 92–93cardiac action potential, 93mechanical force production and

contractile function, 93–94myocardial function, 94

Fetal circulation, 49–52developmental changes, 65–66

Fibrinogenassay, 340concentrate, 182

Fibrinolysis, 328Fibroma, electrocardiogram, 277FIBTEM test, 339Fick principle, 585First heart field (FHF), 12Flecainide, 157Flow theory, 53, 620Fluid management

balance and distribution, physiology, 890–892

fluid overload, pharmacologic management of

dopamine, 921fenoldopam, 923loop diuretics, 921nesiritide, 922

practical approach to management of, 936–938

and solute movement, physiology, 892treatment course

daily fluid balance, monitoring of, 920

fluid intake calculation, 914fluid overload, 914goal-directed fluid therapy, 914–916maintenance fluid therapy, in

paediatric patients, 919–920parental nutrition, 920type of fluid solution, 916–919

Focal atrial tachycardia (AT), 771–774Fondaparinux, 176–177Foramen ovale, 25, 51

during fetal circulation, 66during neonatal circulation, 67

Frank-Starling relationship, 97–100Fresh frozen plasma, 181Frey, Maximilian von, 8Furosemide, 146FVIIA, 328

GGastrointestinal system, CoA, 630Gibbon, John, 8Glenn procedure, 562Glucose, usage during CPR, 971–972Glycoprotein IIB/IIIA inhibitors, 178Gonadal arteries, 43Gore-Tex monocusp, 492Gosling’s pulsatility index, 298Great arteries, transposition of, 271–273Great vessels transposition, 36Gross, Robert, 7

HHaloperidol, 168Halothane, 165Hammock mitral valve, 578Harvey, Lee, 7Heart disease and cerebral function, 954Heart failure, therapeutic agents against,

88–89Heart-lung machine, 8Heart rate, 246Heart rhythm, 247

electrocardiographic pattern, 247Heart transplantation

diagnostic categories for congenital heart disease patients, 748

Index

1009

multivariable analysis for risk factors, 748–749

patient characteristics, VAD type, 748–749

reasons for, 745survey report, 746–747yearly status report on, 746

Heart tubecaudal part, 16cephalic part, 15developmental phases, 14formation, 13–15looping of, 15–18ventricular loop, 16

Heat exchanger, 431Hemangiomas, 52Hematocrit, and perioperative transfusion

management, 390Hematologic system, neonatal, 329Hemiazygos vein, 48Hemodilution, CPB, 432–434Hemodynamic complications, in postoperative

carelow cardiac output

afterload due to vasoconstriction, 762–763

contractility dysfunction, 763–764dysrhythmia ECG, 764factors, 761paraclinical parameters, 761preload due to loss of intravascular

volume, 762residual defects, 762surgical-related factors, 762

postoperative bleeding and cardiac tamponade, 764–765

pulmonary hypertension after cardiac surgery, 765–766

Hemodynamic theory, 53, 620Hemoptysis, 580Hemorrhage, 327Hemostasis

neonatal vs. adult, 328–329pediatric, 328

Heparinase, 340HEPTEM test, 339High-flow nasal cannula (HFNC), 795–796High-frequency oscillatory ventilation

(HFOV), 796–797High-frequency ventilation (HFV). See

High-frequency oscillatory ventilation (HFOV)

His bundle, 32electrical activity, 71

HLHS. See Hypoplastic left heart syndrome (HLHS)

Homocystinuria, 864Homograft monocusp, 492Hospitals for children, 4–5Human physiology

acid-base balance, 896–897electrolyte balance

bicarbonate, 896calcium, 894–895chloride, 895ECS fluid, plasma and ICS fluid

composition, 891–893Gamble gram, 893ionic balance, 892magnesium, 895phosphorus, 895potassium, 894sodium, 893–894

fluid and solute movement, 892fluid balance and distribution,

890–892Hybrid procedure, HLHS for

children, 667Hybrid suites, 403Hydralazine, 140Hydrochlorothiazide, 147Hydromorphone, 163Hypercarbia, 587Hypercyanosis, 485Hyperkalemic cardiac arrest, 975Hypertension, pulmonary, 464, 525, 562Hypertrophic cardiomyopathy

(HCM), 273Hypertrophy, vascular medial, 368Hypoglycemia, 861Hyponatremia, 121Hypoplasia

of aortic arch, 628–629of left heart structures, 628

Hypoplastic left heart syndrome (HLHS), 386, 526, 597

hybrid procedure, 667pathology, 664prenatal diagnosis, 664preoperative care, 665single ventricle reconstruction trial,

666–667stage I

hybrid procedure, 666modified Norwood procedure, 665Sano procedure, 665transplantation, 666

surgical approach, 667–668

Index

1010

Hypothalamic-pituitary-adrenal (HPA) axisimpaired HPA axis, 852with non-suppressed HPA axis, 852–853with uncertain status, 853

Hypothermia, 327, 435Hypovolemia, 388, 587Hypoxemia, chronic, 486Hypoxia, 587

IIAA. See Interrupted aortic arch (IAA)Ibutilide, 159ICD. See Implantable cardioverter-

defibrillators (ICD)Iloprost, 150Implantable cardioverter-defibrillators (ICD),

391, 412Impulse-generating cells, 70Infant mortality rate (IMR), 981Inferior mesenteric artery (IMA), 42Inferior vena cava (IVC), 377, 522

anomalies, 54–55during fetal circulation, 66formation, 47–48

Inflammatory conditionsacute rheumatic fever, 276Kawasaki disease, 275–276myocarditis, 276pericarditis, 276

Infundibular VSD. See Outlet type VSDInhalational therapies, 797Inhaled nitric oxide (iNO), 143, 149, 152

for pulmonary hypertension, 701, 703for right ventricle failure, 723

Inhibitor, cyclooxygenase, 408Inlet type VSD, 471Inoconstrictors

dopamine, 124–129, 132epinephrine, 122–124, 132norepinephrine, 130–132

Inodilatorsdobutamine, 138–139levosimendan, 139milrinone, 135–138

Inodilators, for right ventricle failure, 722–723INTEM test, 339Interleukin 18 (IL-18), for AKI, 913Internal jugular (IJV) vein, 231Interrupted aortic arch (IAA)

associated anomalies, 643classification, 642–643clinical findings, 643–644treatment

anesthetic management, 645intraoperative management, 646postoperative management, 646–647preoperative management, 645–646surgical, 644–645

Intersegmental branches, 43Interventricular septum (IVS), 469

creation of, 29membranous, 469muscular, 469

Intracardiac echocardiography (ICE), 214–215, 393

Intraoperative risk factors, for postoperative bleeding

cardiopulmonary bypass, 809–811mechanical circulation with ECMO, 809RACHS-1 score, 808

Intraoperative TEE, 452Intrauterine mortality, 381Intravenous anesthetic agents, 155, 164Intravenous sedative and anesthetic agent,

for pulmonary hypertension, 706–707

Intraventricular conduction defect, 256–259

Invasive cardiac monitoring, 229–230Iron Heart, 8Isoflurane, 165Isolated dextrocardia, 360, 370Isoproterenol, 132Isovolumic relaxation (phase 4), 95Isovolumic systole (phase 2), 95Isradipine, 146Istaroxime, 89IVS. See Interventricular septum (IVS)

JJacobi, Abraham, 4Jugular venous oxygen saturation (SjVO2),

305anatomic approach, 306–308catheters and technique, 306limitations, 308–309

Junctional ectopic tachycardia (JET), 498, 770–771

Juxta-arterial VSD. See Outlet type VSD

KKabuki syndrome, 630Kallikrein, 437Kaolin test, 340–341Kawasaki disease, 275–276, 733–734

Index

1011

Ketamine, 164, 496, 588, 610in cardiac catheterization, 208

Ketorolac, 163

LLabetalol, 148Ladd, William E., 5Laenne, Rene, 7Lansoprazole, 170Laplace’s law, 599Laryngeal nerves, 40–41Lateral branches development, 42–435-Lead electrocardiogram, 228–229Leadless pacemakers, 413Left anterior descending (LAD) coronary

artery, 501Left anterior hemiblock (LAHB), 257Left atrium, saturated and desaturated blood

mixture, 50Left bundle branch block (LBBB), 257–259Left heart structure, hypoplasia of, 628Left posterior hemiblock (LPHB), 258Left superior vena cava (LSVC), 55–56, 546Left-to-right shunt lesions

atrial septal defects, 377–378patent ductus arteriosus, 376–377ventricular septal defects, 378–379

Left ventricles (LV), fetal circulation, 66Left ventricular end-diastolic pressure

(LVEDP), 600Left ventricular function, mechanical support

of, 8–9Left ventricular hypertrophy (LVH), 253–254Left ventricular outflow tract (LVOT),

447, 476Levophed. See NorepinephrineLevosimendan, 133, 139

pharmacological properties, 136Levosimendan, for right ventricle failure, 723Lewis, Francis West, 4Lidocaine, 156

usage during CPR, 973–974Limb arteries formation, 44Lisinopril, 145Liver, saturated and desaturated blood mixture,

50Lorazepam, 164Losartan, 145Low cardiac output (LCO)

afterload due to vasoconstriction, 762–763contractility dysfunction, 763–764dysrhythmia ECG, 764factors, 761

paraclinical parameters, 761preload due to loss of intravascular

volume, 762residual defects, 762surgical-related factors, 762

Low molecular weight heparin (LMWH), postoperative bleeding, 808

L-TGA, 371L-TGV. See L-transposition of the great

vessels (L-TGV)L-transposition of the great vessels

(L-TGV), 370LVEDP. See Left ventricular end-diastolic

pressure (LVEDP)LVOT. See Left ventricular outflow tract

(LVOT)LVOT obstruction (LVOTO), 539, 646–647

obstruction, 372, 534–536neonates with critical, 601

Lymphatic ducts, 49Lymphatic system

anomalies, 56–57formation of, 48

Lymphedema, 56–57Lymph sacs, 48–49

MMacitentan, 151Magnesium sulfate, 161Magnetic resonance imaging (MRI)

for coronary artery anomalies, 737–738scanners, 200–201

Major aortopulmonary collateral arteries (MAPCA), 559, 563–564

Maple syrup urine disease (MSUD), 863Maternal systemic lupus erythematosus, 357Maximum clot firmness (MCF), 351MBTS. See Modified Blalock-Taussig shunt

(MBTS)M-clock (membrane clock) theory, 75Mechanical circulatory support

Berlin Heart, 751–752Cedars algorithm for single-ventricle

patients, 753–754ECMO, 749risks identifition, for death with VAD,

750–751survival to transplantation with VAD

support vs. status 1 patients, 750temporary VAD for single-ventricle

patients, centrifugal pump, 754waiting list survival, ERA VAD vs.

non-VAD survival, 751–752

Index

1012

Mechanical force production, 93–94Mechanical (contractile) function

thick filament, 82–84thin filament, 84–88

Mechanical ventilation, postoperative respiratory management

extubation failure, predictors of, 793goals, 787–788indications for utilization of, 787mixing lesions and cardiac shunts, 788perioperative care of single ventricle

physiology, 789prediction tests of extubation success, 791principle, 787right/left ventricular failure, 789strategies for ventilation and ventilator

mode, 790weaning ventilator support and

spontaneous breathing trials, 790–791

Melody valve, 395, 410Membranous IVS, 469Metabolic acidosis, in postoperative cardiac

surgery, 932–934Metabolic alkalosis, in postoperative cardiac

surgery, 934–935Metabolic disorders, perioperative

management for, 860–861fatty acid oxidation defects, 864homocystinuria, 864hypoglycemia, 861maple syrup urine disease, 863methylmalonic and propionic academia,

862–863mucopolysaccharidosis, 864–865phenylketonuria, 861urea cycle defects, 863–864

Metabolism, during CPB, 434–436Methadone (opioid), 162Methyldopa, 149Methylmalonic and propionic academia,

862–863Metolazone, 147Metoprolol, 148, 158Mexiletine, 156Midazolam, 164Middle cerebral artery (MCA), 298Mid-spectrum DORV, 679Milrinone, 133

administration routes, 137adverse effects, 137–138dosage, 137indications, 137mechanism of effect, 135

pharmacological properties, 136precautions, 137–138for right ventricle failure, 722

Milroy disease, 57Minimally invasive cardiac output, 235Mitral regurgitation, 589Mitral stenosis, congenital, 579–580Mitral valve, 476

Ebstein’s malformation of, 574embryology of, 573Hammock, 578orifice, 578parachute, 577–578stenosis, 587–588

Mixing lesions, 368–369M line, 88, 94Moderator band, 29Modified Blalock-Taussig shunt (MBTS), 487,

489, 504Modified Norwood procedure, HLHS for

children, 665Morphine (opioid), 162Motor evoked potential (MEP), 310M2 receptor activation, 91–92Mucopolysaccharidosis (MPS), 864–865Multifascicular block, 259Multiplate analyzer®, 345–346Multi-societal database committee for

pediatric and congenital heart disease classification, 912

Muscle relaxants, 529Muscular IVS, 469Muscular pulmonary atresia, 559Muscular VSDs, 472Mustard procedure, 526, 529Myocardial electromechanical function

action potential (see Action potential)excitation-contraction coupling, 77–80mechanical (contractile) function, 81–88

Myocardial filaments, 81thick filament, 82–84thin filament, 84–88

Myocardial function, 94Myocardial progenitor cells, 11Myocarditis, 276Myocardium, 14Myofibrils, 94Myosin, 82–83Myosin heavy chain (MHC), 82–83Myosin light chains (MLC), 82Myosin molecule (MM), 82Myosin strand (MS), 82Myotonic muscular dystrophy (MMD), 275Myxoma, electrocardiogram, 277

Index

1013

NNasal cannulas. See RAM cannulasNasal flaring, 319Nasal prongs. See RAM cannulasNATEM test, 339Native RVOT, 411–412Near-infrared spectroscopy (NIRS), 229, 638Near infrared spectroscopy (NIRS), CNS

monitoring byadvantages, 285cerebral hemodynamics, 285, 287consideration, 287oxygen supply and consumption, 285–286technology, 285

Necrosis, cystic medial, 621Nelson, Russell M., 8Neonatal circulation/circulation at birth,

66–69Neonatal continuous positive airway pressure

(nCPAP), 794–795Neonatal hematologic system, 329Neonatal hemostasis vs. adult hemostasis,

329–330Neonatal seizure, 836

diagnosis of, 837–838etiologies, 837risk factors for, 837treatment, 838

Neonatal stroke, 838Neonate

aortic stenosis of, 595–596, 600–604with critical left ventricular outflow tract

obstruction, 601Nesiritide, 133Neuraxial blocks, 876–877Neurodevelopmental issues, 416–417Neurologic deficits, 836–838Neuropathic pain, 872Neutrophil gelatinase-associated lipocalin

(NGAL), for AKI, 912–913Nicardipine, 144Nifedipine, 146Nikaidoh procedure, 534, 536Nitric oxide, 149, 152Nitroglycerin (NTG), 139–140, 143, 638Nitroprusside, 143, 638Nitrous oxide, 165Noncardiac anomalies, 531

of coarctation of the aorta, 630–631Noncardiac congenital anomaly, 503–504Noncommitted VSD, 676

with DOLV, clinical presentation of, 691Noninvasive cardiac monitoring, 227–228

blood pressure, 229

5-lead electrocardiogram, 228–229pulse oximetry, 228

Non-PDA-dependent lesions, 500–508Noonan’s syndrome, 556Norepinephrine, 132

dose and administration, 131indications, 130occurrence, 130precautions, 131for right ventricle failure, 723warnings and contraindications, 131

Normal sinus rhythm (NSR), 247Norwood procedure, 526NPO intervals, perioperative fluid management

and, 389–390Nucleus solitarius, 101–102

OObstructive lesions, 369Oculocardiac reflex, 106Ohm’s law, 368Olanzapine, 169Omecamtiv mecarbil, 88Omeprazole, 170Open-chest defibrillation, 962–963Opiate medication, in cardiac catheterization,

207Orifice mitral valve, 578Ostium primum, 24Ostium secundum, 24–25Outflow tract, 16Outflow tract septation anomalies

great vessels transposition, 36tetralogy of Fallot, 37truncus arteriosus, 36

Outlet type VSD, 471Oxygenator, CPB, 431–432Oxygen dissociation curve (ODC), 435–436

PPAC. See Pulmonary artery catheter (PAC)Pacemaker, 391

devices, 414leadless, 413

Pacemaker potential. See Cardiac pacemaker cells, action potential in

Pain assessmentCHEOPS, 280–281COMFORT Scale, 281–282FLACC, 281PPPM, 282TPPPS, 282

Index

1014

Pain management, 415chronic postsurgical pain

definition, 871–872management of CPTP and post-

sternotomy pain, 875–876neuropathic pain, 872pain after sternotomy, 873–874pain after thoracotomy, 874–875

neuraxial blocks, 876–877peripheral block techniques, 877

paravertebral nerve blocks, 878–879pecs blocks/serratus plane block,

879–881transversus abdominis plane block,

881–883Palliation, transcatheter, 408Palpable pulsation, 359Palpation, 322, 359Pancuronium, 166Papillary muscles, 574Parachute-like asymmetrical valve, 578Parachute mitral valve, 577–578Parasympathetic receptors, 90Paravertebral nerve blocks (PVNB), 878–879Parental nutrition, 920Parents’ Postoperative Pain Measure (PPPM), 282Patent ductus arteriosus (PDA), 216, 366,

375–377, 486, 629Amplatzer Duct Occluder, 660asymptomatic treatment for, 659clinical conditions, 657clinical management, 660conical ductus, 658dependent lesions, 498–500, 502

non-PDA, 500–508diagnosis of, 657DOLV anatomy, 688electrocardiogram, 264–266elongated ductus, 660failure of, 659pathologies, 659in premature infants, 408–409prophylactic pharmacologic treatment for,

661saccular ductus, 658symptomatic treatment for, 659therapy options for, 658transthoracic echocardiography, 660tubular ductus, 658use of paracetamol, 659–660window ductus, 658

Patent foramen ovale (PFO), 377, 381Patent pulmonary outflow, surgical repair of

DOLV with, 692PCHA. See Pediatric Congenital Heart

Association (PCHA)

PCPs. See Primary care physicians (PCPs)PDA. See Patent ductus arteriosus (PDA)Peacock, Thomas, 7Pecs/serratus plane block, 879–881Pediatric anesthesiology, origins of, 5–6Pediatric assistance program, 985–986Pediatric cardiac and congenital surgery,

development of, 6–8Pediatric cardiovascular monitoring

arterial line, 230–231bioimpedance/bioreactance, 238–239central venous lines, 231–233invasive monitoring, 229–230minimally invasive cardiac output, 235near-infrared spectroscopy, 229noninvasive monitoring, 227–228

blood pressure, 2295-lead electrocardiogram, 228–229pulse oximetry, 228

pulmonary artery catheter, 233–235pulse contour analysis, 237ultrasound, 237

Pediatric Congenital Heart Association (PCHA), 400

Pediatric delirium, drugs for, 165, 167–169Pediatric heart patients

evaluation coordination, 399–400referral and evaluation of, 399and referral management, 397–399surgical and cardiologic interventional

issues, 401–403Pediatric heart surgeons (PHS) vs. adult heart

surgeon, 401, 403Pediatric hemostasis, 328Pediatric patients, 4Pediatric Perioperative Cardiac Arrest

Registry, 200, 209Pediatrics, birth of, 4–5Pediatric surgery

history of, 3surgical pioneers in, 5

PEEP. See Positive end-expiratory pressure (PEEP)

Percussion, 322Percutaneous coronary angioplasty

(PTCA), 346Pericarditis, 276Perimembranous VSD, 471, 484Perioperative cardiac arrest, 974–975Perioperative imaging

catheterization laboratoryintracardiac echocardiography,

214–215transthoracic echocardiography,

215–216epicardial echocardiography, 223–224

Index

1015

transesophageal echocardiographycontraindications to, 222–223operating room, 221safety and complications, 222–223

Perioperative managementchronic postsurgical pain

definition, 871–872management of CPTP and post-

sternotomy pain, 875–876neuropathic pain, 872pain after sternotomy, 873–874pain after thoracotomy, 874–875

in diabetic patients, 857–860neuraxial blocks, 876–877patients on steroids requiring surgery,

852–855in patients with metabolic disorder,

860–865peripheral block techniques, 877

paravertebral nerve blocks, 878–879pecs blocks/serratus plane block,

879–881transversus abdominis plane block,

881–883pituitary gland in, 855–857

Peripheral block techniques, 877paravertebral nerve blocks, 878–879pecs blocks/serratus plane block, 879–881transversus abdominis plane block,

881–883Periventricular leukomalacia (PVL), 838

clinical presentation of, 841definition, 839diagnosis, 841–842etiology, 839mechanism of injury, 840monitoring, 844postoperative delirium in pediatric patients

clinical assessment and monitoring of, 843

diagnostic features, 842treatment of, 843–844

prevalence, 840–841risk factors for, 839–840treatment for, 842

Persistent fetal circulation (PFC), 26Persistent foramen ovale (PFO), 26, 462Persistent truncus arteriosus, 36PgE. See Prostaglandin E (PgE)PHACE syndrome, 630Pharmacology. See Vasoactive agentsPharyngeal arch arteries, 38Phentolamine mesylate/regitine, 142,

144–145, 147, 149–185Phenylephrine, 134, 143, 639

adverse effects, 119

cautions, 120dosing, 119mechanism of action, 119

Phenylketonuria (PKU), 861Phenytoin, 157Phosphodiesterase (PDE) III inhibition, by

milrinone, 135Phosphodiesterase inhibitors, 171Phosphodiesterase 3 (PDE-3) inhibitors

for pulmonary hypertension, 702, 705for right ventricle failure, 722

Phosphodiesterase 5 (PDE-5) inhibitorsendothelin receptor blockers

(ET blockers), 153for pulmonary hypertension, 702, 704for right ventricle failure, 723sildenafil, 152–153

Phospholamban (PLB), 78, 92Physiology

autonomic control of heartANS functions, 90β1-and M2 receptor activation, 91–92sympathetic and parasympathetic

receptors, 90cardiac cycle, 94–95cardiac reflexes

bainbridge reflex, 102–103baroreceptor reflex/carotid sinus reflex,

101–102Bezold-Jarisch reflex, 103–104chemoreceptor reflex, 104–105Cushing reflex, 106oculocardiac reflex, 106valsalva maneuver, 105

cardiac workcardiac output, 97ejection fraction, 97–100stroke volume, 96–97

fetal cardiac contractile systemCa2+ homeostasis, 92–93cardiac action potential, 93mechanical force production and

contractile function, 93–94myocardial function, 94

fetal circulation, 65–66Frank-Starling relationship, 97–100myocardial electromechanical function

action potential (see Action potential)excitation-contraction coupling,

77–80mechanical (contractile) function,

81–88neonatal circulation/circulation at birth,

66–69therapeutic agents, against heart failure,

88–89

Index

1016

Pituitary glands, in perioperative managementcentral adrenal insufficiency, 855–856central diabetes insipidus, 856–857central hypothyroidism, 856

Platelet, 181desensitization of, 437function testing, 344–347inhibition, 346–347

Platelet Mapping assay, Thrombelastograph (TEG®), 341, 347–348

Platelet ROTEM® delta system, 347PLE. See Protein-losing enteropathy (PLE)Point of Care (POC) testing, in postoperative

bleeding, 812–813Poiseuille’s law, 367–368, 504Polycythemia, 327, 486Polysplenia, 370Positive end-expiratory pressure

(PEEP), 389Postoperative acid-base balance

pathophysiology in postoperative cardiac surgery

mechanisms, 931–932metabolic acidosis, 932–934metabolic alkalosis, 934–935respiratory acidosis, 935–936respiratory alkalosis, 936

physiology, 896–897practical approach to management of,

938–942Postoperative bleeding

case vignette, 823–824intraoperative risk factors

cardiopulmonary bypass, 809–811mechanical circulation with ECMO,

809RACHS-1 score, 808

outcome, 823pathophysiology of, 811–812point of care testing and algorithms in,

812–813preoperative risk factors

age, 806antiplatelet agent and anticoagulants,

807coagulation anomalies, 807low molecular weight heparin, 808platelet aggregation, 807–808unfractioned heparin, 808vitamin-K antagonists, 808

prophylaxis, 813–817risk factors for, 806transfusion complications, 822–823treatment of

abnormal clot generation, 817–818abnormal clot stability, 819clot firmness, 819–822estimated blood volume by age, 817

Postoperative carefor cardiac arrhythmias (see Arrhythmias,

postoperative care for)central nervous system management

classification of deficits, 834impact of outcome, in pediatric cardiac

surgery, 829intraoperative risk factors for injuries,

830, 833–834neurocognitive and neurodevelopmental

disorders, 838–839neurologic injuries, 836–838postoperative risk factors for

injuries, 834–836preoperative risk factors for injuries,

830–832white matter injuries with

periventricular leukomalacia, 839–844

delirium in pediatric patientsclinical assessment and monitoring

of, 843diagnostic features, 842treatment of, 843–844

hemodynamic complicationslow cardiac output, 761–764postoperative bleeding and cardiac

tamponade, 764–765pulmonary hypertension after cardiac

surgery, 765–766monitoring techniques

arterial oxygen saturation, 760central venous catheter, 760left atrial catheter, 761near-infrared spectroscopy, 760pulmonary artery catheter, 760–761

patient transfer to ICU, 759Postoperative cognitive impairments

(POCI), 280Postoperative fluid management

balance and distribution, physiology, 890–892

fluid overload, pharmacologic management of

dopamine, 921fenoldopam, 923loop diuretics, 921nesiritide, 922

practical approach to management of, 936–938

Index

1017

and solute movement, physiology, 892treatment course

daily fluid balance, monitoring of, 920fluid intake calculation, 914fluid overload, 914goal-directed fluid therapy, 914–916maintenance fluid therapy, in paediatric

patients, 919–920parental nutrition, 920type of fluid solution, 916–919

Postoperative renal managementphysiology, 897

glomerular filtration rate, 898–903renal blood flow (RBF), 898urine concentration and sodium

excretion fraction, 904practical approach to, 938treatment for( (see Renal replacement

therapy (RRT))Postoperative respiratory management

chest physiotherapy, 797chronic respiratory failure and

tracheostomy, 797–798extracorporeal CO2 removal, 796high-frequency ventilation, 796–797inhalational routes of drug delivery, 797mechanical ventilation

goals, 787–788indications for utilization of, 787mixing lesions and cardiac

shunts, 788perioperative care of single ventricle

physiology, 789prediction tests of extubation success,

791predictors of extubation failure, 793principle, 787right/left ventricular failure, 789strategies for ventilation and ventilator

mode, 790weaning ventilator support and

spontaneous breathing trials, 790–791

noninvasive ventilationHFNC, 795–796nCPAP, 794–795RAM cannulas, 794

pediatric vs. adult patientsanatomy, 785–786physiology, 786

Post-traumatic stress disorder (PTSD), 417Potts procedure, 380Potts shunt, 487Pourcelot resistance index, 298

PPH. See Primary pulmonary hypertension (PPH)

Prasugrel, 179Prazosin, 148Preload, ESV, 96Premature infant, PDA in, 408–409Preoperative risk factors, for postoperative

bleedingage, 806antiplatelet agent and anticoagulants, 807coagulation anomalies, 807low molecular weight heparin, 808platelet aggregation, 807–808unfractioned heparin, 808vitamin-K antagonists, 808

pRIFLE classification system, 911Primary care physicians (PCPs), 398Primary heart field (PHF), 12Primary pulmonary hypertension (PPH),

374–376Primary pulmonary vascular disease, 374–376Primary ventricular septum/fold, 29Primitive atrial septum (PAS), 24–25Primitive atrium, 16, 21–22Primitive ventricle, 16PR interval, 248–249Procainamide, 156Progenitor cells development, 11–12Proinflammatory cytokines, 437Propafenone, 157Prophylaxis

antifibrinolytics, 815–817general measures, 814–815preoperative optimization, 813–814

Propofol, 164in cardiac catheterization, 206–207

Propranolol, 148, 158Prostacyclin

for pulmonary hypertension, 702–703for right ventricle failure, 723

Prostaglandin E (PgE), 498, 531Prostaglandin E1, 144

for right ventricle failure, 723Protease-activated receptor-1 (PAR-1)

antagonists, 178Protein kinase A (PKA), 92

action by milrinone, 135Protein-losing enteropathy (PLE), 386Prothrombin complex, 182Prothrombin complex concentrates

(PCCs), 818PS. See Pulmonic stenosis (PS)Psychological assessment, 417Psychological stressors, 417

Index

1018

PTCA. See Percutaneous coronary angioplasty (PTCA)

PTSD. See Post-traumatic stress disorder (PTSD)

Pulmonary artery (PA), 3703D reconstruction of, 393

Pulmonary artery band (PAB), 541Pulmonary artery catheter (PAC),

233–235, 372, 376Pulmonary atresia, 498–500

muscular, 559Pulmonary atresia with intact ventricular

septum (PA/IVS), 558anatomy, 558–560anesthetic considerations, 562presentation, 560–561treatment, 561–562

Pulmonary atresia with VSD (PA/VSD), 559, 563

anatomy, 563anesthetic considerations, 566presentation, 563–564treatment, 564–566

Pulmonary blood flow (PBF) regulation. See Pulmonary vascular resistance (PVR)

Pulmonary channel, 30Pulmonary congestion, 579Pulmonary edema, 360, 562Pulmonary hypertension (PH),

366, 464, 525, 562anesthesia for patients with congenital

heart disease, 705alleviating and aggravating factors, 706intravenous sedative and anesthetic

agent, 706–707volatile anesthetics, 706, 708

cardiac MR and multi-slice CT scan, 700chest X-ray, 699classification, 697–698clinical features, 698–699definition, 697drugs used in, 147, 150–153echocardiography, 700electrocardiography, 699hemodynamic complications after cardiac

surgery, 765–766management, 700paraclinical studie, 699pharmacological treatment

endothelin receptor antagonists, 701–702, 704

inhaled nitric oxide, 701, 703PDE-3 inhibitors, 702, 705

PDE-5 inhibitors, 702, 704prostacyclin system analogues,

702–703pulmonary vascular system drugs and

anesthetic drugs, 700reversible and irreversible stages, 701soluble guanylate cyclase stimulator,

702, 705radioisotope ventilation/perfusion scan,

700right heart catheterization and pulmonary

angiography, 700Pulmonary outflow tract obstruction (POTO),

subaortic VSD with DOLV, 689–690

Pulmonary regurgitation/stenosis, 380Pulmonary shunting

anesthesia issues of systemic, 505–508physics and physiology of systemic,

503–505Pulmonary stenosis, 498–500Pulmonary trunk, 50Pulmonary valve atresia, electrocardiogram,

268Pulmonary valve insufficiency (PI), 269Pulmonary valve (PV) replacement, 493Pulmonary valve stenosis

electrocardiogram, 267–268Pulmonary vascular disease, 366Pulmonary vascularity, 360Pulmonary vascular occlusive disease, 378Pulmonary vascular resistance (PVR),

51, 67, 69, 368, 519–520, 713–714diagnosis of cardiac arrest, 955epidemiology of cardiopulmonary arrest,

955heart disease and cerebral function, 954outcome of cardiopulmonary arrest, 955pharmacology, 955prevention of cardiopulmonary

arrest in, 955Pulmonary vascular system and anesthetic

drugs, for pulmonary hypertension, 700

Pulmonary vasodilators, for right ventricle failure, 723–724

Pulmonic stenosis (PS), 374Pulmonic valve stenosis, congenital.

See Congenital pulmonic valve stenosis

Pulsatility index (PI), 298Pulse contour analysis, 237Pulseless ventricular tachycardia.

See Ventricular fibrillation

Index

1019

Pulse oximetry, 228, 324Pure vasodilators

alprostadil, 140–141hydralazine, 140nitroglycerin, 139–140phentolamine mesylate/regitine, 142, 145,

147, 149–185sodium nitroprusside, 141–142

Pure vasopressors/vasoconstrictorsphenylephrine, 119–120, 134vasopressin, 120–121, 134

Purkinje fiber, 34cell, 71

PVR. See Pulmonary vascular resistance (PVR)P wave, 247, 250

QQRS

axis, 247–248complex, 250–251

QT interval, 249–250Quetiapine, 169Quinidine, 156

RRadiation issues, 416Radiography, chest, 360Rainbow Flex Procedural Bed, 408RAM cannulas, 794Ramipril, 145Ramsay Sedation Scale (Ramsay),

155, 283Ranitidine, 170Rapid shallow breathing index (RSBI), 791Rapid TEG, 341Rashkind septostomy, 525Rastelli palliation, 533Rastelli procedure, 534–535Rastelli repair, VSD baffle in, 535Recession, 319Red packed cells, 181Reflexes, cardiac. See Cardiac reflexesRefractory period, 74–75Regitine. See Phentolamine mesylate/regitineRegulatory proteins, 81Regurgitant lesions, 369Relative refractory period, 74Remifentanil (opioid), 162Remote VSD

anatomy of DORV with/without PS, 676clinical presentation, 677–678with DOLV, clinical presentation of, 691

Renal bleeding, 123Renal blood flow (RBF), 898Renal management

physiology, 897glomerular filtration rate, 898–903renal blood flow (RBF), 898urine concentration and sodium

excretion fraction, 904practical approach to, 938treatment for (see Renal replacement

therapy (RRT))Renal replacement therapy (RRT)

blood urea nitrogen level, 924continuous renal replacement therapy.,

926–928conventional criteria for, initiation CRRT,

923–924factors, 923peritoneal dialysis, 925–926

Renin-angiotensin-aldosterone system (RAAS), 900–901

Resistance index (RI), 298Respiratory acidosis, in postoperative cardiac

surgery, 935–936Respiratory alkalosis, in postoperative cardiac

surgery, 936Respiratory monitoring

accessory muscle use, 319adventitious/abnormal sounds,

321–322auscultation, 320bronchial sounds, 321capnography and capnometry, 324–325cardiopulmonary interaction, 322–323clinical assessment, 317inspection, 318nasal flaring, 319palpation, 322percussion, 322pulse oximetry, 324recession, 319respiratory monitoring adjuncts, 323respiratory sounds, 321skin color, 320work of breathing, 318

Respiratory sounds, 321Resting membrane potential, 72Restrictive cardiomyopathy, 274–275Retromandibular window, 302–303Rhabdomyoma, electrocardiogram, 277Rib notching, 625Richmond Agitation Sedation Scale (RASS),

155, 283–284RIFLE classification system, 909, 911

Index

1020

Right-and left-sided obstructive lesions, anatomy of DOLV, 688

Right atrium (RA), 521saturated and desaturated blood mixture,

50Right bundle branch block (RBBB), 257–258,

379, 381Right ventricle-dependent coronary circulation

(RVDCC), 559Right ventricle (RV) failure

anatomy, 712definition, 714diagnosis, 718–720echocardiographic evaluation of,

719–720etiology

altered contractility, 715pressure overload, 715–717volume overload, 715

vs. left ventricle, 712–714management, 721pathophysiology, 716, 718in pediatric population, 724physiology, 712–714preload optimization, 721–722

inodilators, 722–723pulmonary vasodilators, 723–724vasoconstrictor, 723

prevention and treatment, 720–721Right ventricle (RV), fetal circulation, 66Right ventricular dysfunction, clinical

presentation of DOLV, 691Right ventricular hypertrophy (RVH),

252–253Right ventricular outflow tract (RVOT),

411–412, 476obstruction, 379–380, 484–485, 559

Riociguat, 151Risk adjustment for congenital heart surgery

model, 986–987Risperidone, 167–168Rivaroxaban, 173Robson, Charles H., 6Rocuronium, 166Roger, Henri, 7Ross procedure, 597, 604ROTEM algorithms, in postoperative bleeding,

812–813Rubella syndrome, 374RVDCC. See Right ventricle-dependent

coronary circulation (RVDCC)RVOT. See Right ventricular outflow tract

(RVOT)

SSandifort, Edwardo, 7Sano procedure, HLHS for children, 665Sarco-/endoplasmic reticulum Ca2+ transport

ATPase (SERCA), 78Sarcolipin (SLN), 79Sarcomeres, filaments attachment, 88Sarcoplasmic reticulum (SR), 77–79Saturated and desaturated blood mixture, 50Secondary heart field (SHF), 13, 31Sedation Agitation Scale (SAS), 155, 284Sedation assessment, CNS monitoring,

282–284, 310Sedative drugs, 155, 164Semilunar valve

formation, 31stenosis of, 35

Senning procedure, 526, 530Septation, 22. See also Septum developmentSeptomarginal trabecula, 29Septostomy, balloon atrial, 362Septum development

atrioventricular canal, 26–27interventricular septum, 29primitive atrial septum, 24–25truncus arteriosus and conus cordis, 30–31

Septum primum, 24–25Septum secundum, 25Septum spurium, 19Sevoflurane, 165Shone syndrome, 587, 630Short-circuiting the liver, 49Shunt lesions, 367–368Sildenafil, 151–153, 587Single-ventricle palliation, surgical repair of

DOLV, 692Single ventricle physiology, perioperative care

of, 789Single ventricle reconstruction trial, HLHS for

children, 666–667Sinoatrial (SA) node

development of, 32electrical activity, 71

Sinus node dysfunction (SND), 776Sinus venarum, 21Sinus venosus, 12

atrial septal defect, 26formation of, 18–19

Sinus venosus type ASD (SVASD), 461SIRS. See Systemic inflammatory reaction

syndrome (SIRS)Skeletal anomalies, 630–631Skin color assessment, 320

Index

1021

Skodaic hypothesis, 620Smooth muscle cells (SMC), 620Society of Thoracic Surgeons (STS), 403Sodium bicarbonate, usage during CPR,

969–970Sodium nitroprusside (SNP), 141–142Soluble guanylate cyclase stimulator (sGC

stimulator), 702, 705Somatosensory evoked potential (SSEP), 310Sotalol, 159Spectral Doppler imaging, 583–584Spinal cord protection strategy, 639Spironolactone, 146Spontaneous breathing trials (SBTs),

790–791Standard Electrode System, 292–293Stenosis. See also specific types of stenosis

of semilunar valve, 35Stenosis/atresia of the left main coronary

artery, 733Stenotic lesions, 369Stenson, Niels, 7Steroids, perioperative management for

patients onacute physical/emotional stresses, 852dosing glucocorticoids, 854with non-suppressed HPA axis, 852–853surgical procedures, 854–855time period for impaired HPA axis, 852with uncertain status of HPA axis, 853using inhaled steroids, 853using intra-articular or spinal

glucocorticoid injections, 854using topical steroids, 853

Stressor, psychological, 417Stress ulcer, prevention and treatment,

169–170Stroke volume (SV), 96–97ST segment, 251–252Subaortic VSD

anatomy of DORV, 674–675DOLV with/without POTO, clinical

presentation of, 689–690and PS with DORV, 675surgical repair of DOLV with, 691

Subarterial VSD. See Outlet type VSDSubclavian arteries, 41Suboccipital window, 302Subpulmonary VSD. See Outlet type VSDSubpulmonic VSD, DOLV with/without

AOTO, 690Succinylcholine, 166Superior mesenteric artery (SMA), 42

Superior vena cava (SVC), 50, 521anomalies, 55–56during fetal circulation, 66

Supracristal VSDs with DOLV, clinical presentation of, 690

Supravalvar pulmonary stenosis (SVPS), 608Supravalvular aortic stenosis (SVAS), 607

anatomy and pathophysiology, 608–609anesthetic management, 610–611anesthetic risk assessment, 609treatment, 609–610

Supraventricular tachycardiaatrial tachycardia, 771–774AV reciprocating and nodal reentrant

tachycardia, 773–774cause of, 770definition, 770junctional ectopic tachycardia, 770–771

Supraventricular tachycardias (SVT), electrocardiogram, 260

Surgery, pediatrics. See Pediatric surgerySurgical pioneers, in pediatric surgery, 5Sustainable congenital heart center

developmentmodels of assistance, 983–984

education abroad, 984experienced physician returns to lead

program, 984one-year program, 985visiting team of specialists, 984–985

pediatric assistance program, 985–986risk adjustment for congenital heart

surgery model, 986–987SVAS. See Supravalvular aortic stenosis

(SVAS)SVASD. See Sinus venosus type ASD

(SVASD)SVC. See Superior vena cava (SVC)SVPS. See Supravalvar pulmonary stenosis

(SVPS)SVR. See Systemic vascular resistance (SVR)Sympathetic receptors, 90Systemic inflammation, during CPB, 436–437Systemic inflammatory reaction syndrome

(SIRS), 436Systemic pulmonary shunting

anesthesia issues of, 505–508physics and physiology of, 504–505

Systemic vascular resistance (SVR), 51, 67, 368, 376, 610

Systemic venous blood (SVB), 521–522Systole, 95Systolic ejection (phase 3), 95

Index

1022

TTachycardia, 587

cause of, 770supraventricular tachycardia, 770–773supraventricular tachycardias, 260ventricular tachycardia, 773, 775ventricular tachycardias, 260–262

Tadalafil, 151Taussig-Bing anomaly, conal septal deviation,

537–538Taussig, Helen, 7TAVR. See Transcatheter aortic valve

replacement (TAVR)TEE. See Transesophageal echocardiography

(TEE)TEG®. See Thromboelastography (TEG®)Terazosin, 148Terlipressin, 121Tetralogy, 37Tetralogy of Fallot (TOF), 7–8, 37, 379–381,

392–395, 481–482, 551–552absent pulmonary valve syndrome,

500–501anatomic variants of, 498anatomy, 552anesthesia issues of the patient with BTS,

508anesthetic considerations, 554–555anesthetic management of clinical cases,

680anomalies requiring palliative shunting

prior to complete repair, 502case presentation, 482clinical presentation, 677–678congenital cardiac anomaly, 502conotruncal spiraling, 483coronary artery anomalies, 501–502DORV with subaortic VSD and PS,

anatomy of, 675electrocardiogram, 269embryology and anatomy, 483–484features of, 484noncardiac congenital anomaly, 503–504non-PDA-dependent lesions, 500–508PDA-dependent lesions, 498–500physics and physiology of systemic

pulmonary shunting, 504–505presentation, 552–553pulmonary atresia, 498–500pulmonary stenosis, 498–500repair, 487–488, 491

transventricular and transatrial, 491surgical correction, postoperative

arrhythmias, 778–779

systemic pulmonary shuntinganesthesia issues of, 505–508physics and physiology of, 504–505

treatment, 553–554uncomplicated

anesthesia considerations, 495–498comorbidities, 494–495contemporary surgical management

and outcomes, 490historical therapeutic approaches and

outcomes, 486–489natural history of operative correction,

493–494presentation and anatomic correlation,

484–486surgical technique, 490–492timing of surgery, 490

TGA. See Transposition of the great arteries (TGA)

Thiazide, 145, 147Thiazide-like diuretics, 145, 147Thick filament, 82–84Thin filament, 84–88Thoracic duct development, 493D echocardiography, congenital mitral

regurgitation, 584Thrombin, 437

activation, 346Thromboelastogram-directed

transfusion, 415Thromboelastography (TEG®), 333

graph analysis and parameters, 336–338history and nomenclature, 334–335indications and limitations, 342–344operation principles, 335–336platelet function testing, 344–347platelet mapping assay,.347–348result interpretation, 342types of testing, 338–341

Thromboelastometry (ROTEM®), 333graph analysis and parameters, 336–338history and nomenclature, 334–335indications and limitations, 342–344operation principles, 335–336platelet function testing, 344–347result interpretation, 342types of testing, 338–341

Thrombolytic agents, 181Thromboxane inhibitors, 178Tirofiban, 180Titin, 83–84, 88Toddler Preschool Postoperative Pain Scale

(TPPPS), 282TOF. See Tetralogy of Fallot (TOF)

Index

1023

Total anomalous pulmonary venous connection (TAPVC), 267

Tranexamic acid, 183Transcatheter

palliation, 408PDA, 409valves, 409–412

Transcatheter aortic valve replacement (TAVR), 372

Transcatheter occlusion of PDA, 408–409Transcatheter pulmonary valve replacement,

392–395Transcatheter valve replacement, 215Transcranial Doppler (TCD),

CNS monitoringindications, 299–300limitations, 303principles, 297–299signal acquisition of, 300–303

Transcutaneous cardiac pacing (TCP), 963Transesophageal echocardiography

(TEE), 361, 374, 376, 378, 387, 393, 395, 452

congenital mitral regurgitation, 584congenital mitral stenosis, 582for coronary artery anomalies, 735, 737perioperative imaging

atrial anatomy, 217–219benefits, 221contraindications to, 222–223operating room, 221probe sizes, 217safety and complications, 222–223thoracic imaging, 212

Transfusion-related acute lung injury (TRALI), 822–823

Transorbital window, 302Transposition of the great arteries

(TGA), 482addendum on invasive monitoring catheter

insertion, 542anatomy of DORV with subpulmonary

VSD, 675–676anesthetic considerations, 531–532with aortic coarctation, 532–533arterial and venous mixing in, 519clinical presentation, 520, 677comorbidities, 530complex, 532conal septal deviation

Taussig-Bing anomaly, 537–538TGA/VSD and LVOT obstruction,

534–536with conal septal deviation, 533

contemporary management and interventions prior to surgery, 524–526

contemporary surgical management and outcomes, 526–530

corrected, 538–542embryology, 516–517historical therapeutic approaches and

outcomes, 521–524with LVOTO, 534simple, 518–520surgical correction, postoperative

arrhythmias, 780surgical repair of

DORV with PS, 679with subpulmonic VSD and without

PS, 679terminology and anatomy, 517–518ultrasound for CVL placement and arterial

cannulation, 543–546Transtemporal window, 301Transthoracic echocardiography (TTE),

215–216, 452, 626congenital mitral regurgitation,

583–584for coronary artery anomalies,

735–736for patent ductus arteriosus, 658

Transversus abdominis plane (TAP) blockanesthesia, 883complications, 883nerve distribution in, 881surface anatomy, 881–882ultrasound probe position for in-line

approach, 881, 883Treprostinil, 150Tricuspid atresia, electrocardiogram, 267Tricuspid valve (TV), 559Trifascicular block, 259Triflusal, 171Trigeminal cardiac reflex (TCR), 107Tropomyosin (TM), 84–85Troponin C (TnC), 85–86Troponin complex (Tn), 85Troponin I (TnI), 86Troponin T (TnT), 86Truncus arteriosus, 16, 30–31, 36

electrocardiogram, 267TTE. See Transthoracic echocardiography

(TTE)Turner syndrome, 57, 372, 617, 630, 635T wave, 251Two-dimensional (2D) echocardiography,

553, 583

Index

1024

UUltrasound, 237

for CVL placement and arterial cannulation, 543–546

Umbilical arteries, 42Umbilical veins, 46–47

inflow by, 14Uncoupling proteins (UCPs), 80Unfractionated heparin (UFH), 175, 177

for postoperative bleeding, 808Univentricular approach, 561Univentricular heart, surgical repair of,

779–780Urogenital system, CoA, 630–631

VVACTERL, 388Vacuum-assisted venous drainage (VAVD),

428Valsalva maneuver, 105Valsartan, 145Valve sparring approach, 554Valvular aortic stenosis, 597–598

anatomy, 598–599categorizing severity of, 605child/adolescent with, 605–606pathophysiology, 599–600

Valvular sinuses, 31Valvuloplasty, 221

balloon, 371–372, 557Vancomycin, 185Vascular access and approach,

202–203Vascular access, CPB, 426–429Vascular formation, 38Vascular medial hypertrophy, 368Vascular occlusive disease,

pulmonary, 378Vasculogenesis, 38Vasoactive agents

classification, 117–118inoconstrictors

dopamine, 124–129, 132epinephrine, 122–124, 132norepinephrine, 130–132

inodilatorsdobutamine, 138–139levosimendan, 139milrinone, 135–138

pure vasodilatorsalprostadil, 140–141hydralazine, 140nitroglycerin, 139–140

phentolamine mesylate/regitine, 142, 145, 147, 149–185

sodium nitroprusside, 141–142pure vasopressors/vasoconstrictors

phenylephrine, 119–120, 134vasopressin, 120–121, 134

receptor types targeted by, 118Vasoactive drugs, 638–639Vasoconstrictor

alpha-mediated, 497for right ventricle failure, 723

Vasodilators, 147, 149. See also Pure vasodilators

Vasopressin, 134, 143indications, 120–121mechanism of action, 120for right ventricle failure, 723usage during CPR, 968

VAVD. See Vacuum-assisted venous drainage (VAVD)

Vecuronium, 166Vena cava anomalies

inferior vena cava, 54–55superior vena cava, 55–56

Vena cava system, 47Venarum sinus, 21Venous return, 96Venous system formation

cardinal veins, 47inferior vena cava, 47–48umbilical veins, 46–47vitelline veins, 46

Ventral intersegmental arteries, 43Ventricular assist devices (VADs), 184Ventricular fibrillation

automated external defibrillation, 963defibrillation in children, practical aspects

of, 961–962electric countershock, 960–961open-chest defibrillation, 962–963transcutaneous cardiac pacing, 963

Ventricular septal defect (VSD), 371, 375, 378–379, 468–469, 526, 629

anatomy of double-outlet left ventricle, 687–688

anesthetic management, 477AVSD (see Atrioventricular septal defect

(AVSD))baffle in Rastelli repair, 535cardiac catheterization, 474cardiovascular findings, 473–474chest X-ray, 474classification, 471–472clinical findings, 473

Index

1025

diagnosis, 473–476DORV

anesthetic management of clinical cases, 680–682

clinical presentation, 677surgical repair, 678

echocardiography, 474–476electrocardiogram, 264electrocardiography, 474embryology and classification, 469–470general findings, 473inlet type, 471muscular, 472outcome, 477outlet type, 471pathophysiology, 471–473perimembranous, 471, 484pulmonary atresia with, 563

anatomy, 563anesthetic considerations, 566presentation, 563–564treatment, 564–566

remote VSD with DORV, with/without PS, 676

respiratory findings, 473subaortic (see Subaortic VSD)subpulmonic VSD with DOLV, with/

without AOTO, 690supracristal VSD with DOLV, clinical

presentation of, 690surgical procedures, postoperative

arrhythmias, 777treatment, 476–477

Ventricular syncytium, 70Ventricular tachycardia (VT), 773, 775

electrocardiogram, 260–262Verapamil, 160VerifyNow® System, 345Vesicular sounds, 321

Visceral pericardium, 14Viscoelastic testing, 343–344Visible pulsation, 359Visual evoked potential (VEP), 310Vitamin-K antagonists, for postoperative

bleeding, 808Vitelline arteries, 42Vitelline veins, 46

inflow by, 14Volatile anesthetic agents, in cardiac

catheterization, 206Volatile anesthetics, 165

for pulmonary hypertension, 706, 708Volumetric capnography, 791von Willebrand disease, 343von Willebrand factor (vWF), 329, 347VSD. See Ventricular septal defect (VSD)

WWarfarin, 172Waterston procedure, 380Waterston shunt, 487Watson, Donald, 5Weaning ventilator support, 790–791White matter injuries (WMI), 839–844Williams syndrome, 374, 606–607, 610, 642Wolff-Parkinson-White (WPW) syndrome,

382Work of breathing (WOB), 318

XXenon, 165

ZZ band, 94Z discs, 87–88

Index