ANESTHESIA FOR CHILDREN WITH CONGENITAL HEART DISEASE

Transcript

1. ANESTHESIA FOR CHILDREN WITH CONGENITAL HEART DISEASE George

Nicolaou, MD FRCPC Department of Anesthesia & Perioperative Medicine University of
Western Ontario http://www.childrensheartinstitute.org

2. INTRODUCTION
o Number of children reaching adulthood with CHD has increased over the last 5
decades
o D/T advances in diagnosis, medical, critical and surgical care
o Therefore, not uncommon for adult patients with CHD to present for non-cardiac
surgery

3. INCIDENCE
o 7 to 10 per 1000 live births
o Premature infants 2-3X higher incidence
o Most common form of congenital disease
o Accounts for 30% of total incidence of all congenital diseases
o 10% -15% have associated congenital anomalies of skeletal, RT, GUT or GIT
o Only 15% survive to adulthood without treatment

4. ETIOLOGY
o 10% associated with chromosomal abnormalities
o Two thirds of these occur with Trisomy 21
o One third occur with karyotypic abnormalities such as Trisomy 13, Trisomy 18 &
Turner Syndrome

o Remaining 90% are multifactorial in origin

o Interaction of several genes with or without external factors such as rubella,
ethanol abuse, lithium and maternal diabetes mellitus

5. FETAL CIRCULATION
o There are 4 shunts in fetal circulation: placenta, ductus venosus, foramen ovale,
and ductus arteriosus
o In adult, gas exchange occurs in lungs. In fetus, the placenta provides the
exchange of gases and nutrients

6. CARDIOPULMONARY CHANGES AT BIRTH

o Removal of placenta results in following:
o SVR (because the placenta has lowest vascular resistance in the fetus)
o Cessation of blood flow in the umbilical vein resulting in closure of the ductus
venosus

7. CARDIOPULMONARY CHANGES AT BIRTH

o Lung expansion -> reduction of the pulmonary vascular resistance (PVR), an
increase in pulmonary blood flow, & a fall in PA pressure

8. CARDIOPULMONARY CHANGES AT BIRTH

o LUNG EXPANSION:

Functional closure of the foramen ovale as a result LAP in excess RAP

The LAP increases as a result of the PBF and pulmonary venous return
to the LA

RAP pressure falls as a result of closure of the ductus venosus

PDA closure D/T arterial oxygen saturation

9. CARDIOPULMONARY CHANGES AT BIRTH

o PVR high as SVR near or at term

o High PVR maintained by amount of smooth muscle in walls of pulmonary
arterioles & alveolar hypoxia resulting from collapsed lungs
o Lung expansion -> alveolar oxygen tension -> PVR

10. CLASSIFICATION OF CHD

o L R SHUNTS

Defects connecting arterial & venous circulation

SVR > PVR -> PBF

p ulmonary blood flow -> pulmonary congestion -> CHF ->

susceptibility to RTI

Long standing L-R shunts -> PHT

PVR > SVR -> R-L shunt -> Eisenmengers syndrome

11. CLASSIFICATION OF CHD

o L - R SHUNTS INCLUDE : 5

ASD -> 7.5% of CHD

VSD -> COMMONEST CHD 25%

PDA -> 7.5% of CHD

ENDOCARDIAL CUSHION DEFECT - 3%

Common in premature infants

Often seen with trisomy 21( Common atrioventricular canal,

Atrioventricular septal defect)[single Av annulus that drains boths
atria] 3 components [Ostium primum+interventricular
communication+abnormal AV valves}

AORTOPULMONARY WINDOW [vascular communication between the

ascending aorta and main pulm artery]

12. VENTRICULAR SEPTAL DEFECT

13. ATRIOVENTRICULAR CANAL DEFECT

14. L R SHUNTS
o PERIOPERATIVE TREATMENT

Indomethacin -> PDA closure

Digoxin, diuretics, ACE inhibitors -> CHF

Main PA band -> PVR -> L-R shunt

Definitive open heart surgery

o POSTOPERATIVE PROBLEMS

SVTs and conduction delays

Valvular incompetence -> most common after canal defect repairs

15.
o A subset of associated cardiac anomaliesso-called ductal-dependent lesions
depend on flow through the PDA to maintain systemic blood flow.
Premature closure of the ductus without concurrent repair of the following
defects is contraindicated and may be fatal:
o Pulmonary artery hypoplasia
o Pulmonary atresia
o Tricuspid atresia
o Transposition of the great arteries
o Aortic valve atresia
o Mitral valve atresia with hypoplastic left ventricle
o Severe coarctation of the aorta/Interrupted aortic arch

16. CLASSIFICATION OF CHD

o R L SHUNTS

Resistance to pulmonary blood flow -> PBF -> hypoxemia and cyanosis

Defect between R and L heart

o INCLUDE :

TOF 10% of CHD, commonest R-L shunt

PULMONARY ATRESIA

TRICUSPID ATRESIA

EBSTEINS ANOMALY ( atrialized Rt Ventricle, proximal inlet portion

of RV is above the displaced Tricuspid valve, also is stenosed
usually.Associated with ASD, PS, or pulm atresia ,PDA and corrected
TGA. Inc incidence of prexcitation syndrome[WPW]

17. R L SHUNTS
o GOAL -> PBF to improve oxygenation

Neonatal PGE1 (0.03 0.10mcg/kg/min) maintains PDA -> PBF

PGE1 complications -> vasodilatation, hypotension, bradycardia,

arrhythmias, apnea or hypoventilation, seizures, hyperthermia

Palliative shunts -> PBF, improve hypoxemia and stimulate growth in

PA -> aids technical feasibility of future repair

18. GLENN SHUNT

19. MODIFIED BLALOCK-TAUSSIG SHUNT

20. TETRALOGY OF FALLOT

o 10% of all CHD
o Most common R L shunt
o 4 anomalies:

RVOT obstruction ( infundibular, pulmonic or supravalvular stenosis )

Subaortic VSD

Overriding aorta

RVH

21. TETRALOGY OF FALLOT

22. TETRALOGY OF FALLOT

o Hypercyanotic ( tet ) spells occur D/T infundibular spasm, low pH or low PaO2
o In awake patient manifests as acute cyanosis & hyperventilation
o May occur with feeding, crying, defecation or stress
o During anesthesia D/T acute dynamic infundibular spasm

23. TETRALOGY OF FALLOT

o Treatment of Hypercyanotic Spells

High FiO2 -> pulmonary vasodilator -> PVR

Hydration (fluid bolus) -> opens RVOT

Morphine (0.1mg/kg/dose) -> sedation, PVR

Ketamine -> SVR, sedation, analgesia -> PBF

Phenylephrine (1mcg/kg/dose) -> SVR

-blockers (Esmolol 100-200mcg/kg/min)

o -> HR,-ve inotropy -> improves flow across obstructed valve & infundibular
spasm

24. TETRALOGY OF FALLOT

o Halothane -> HR & -ve inotropy

Rapidly tuned on and off

Careful in severe RVF

o Thiopental -> -ve inotropy

o Squatting, abdominal compression -> SVR

25. EBSTEINS ANOMALY

26. CLASSIFICATION OF CHD

o COMPLEX SHUNTS (MIXING LESIONS)

Continuous mixing of venous and arterial blood blood saturation 70% 80%

May or may not be obstruction to flow

Produce both cyanosis and CHF

Overzealous improvement in PBF steals circulation from aorta -> systemic

hypotension -> coronary ischemia

27. CLASSIFICATION OF CHD

o COMPLEX SHUNTS INCLUDE :

T RUNCUS ARTERIOSUS(single arterial trunk arising from both

ventricles,failure of the truncus to divide into aorta nd pulmonary
artery)Always VSD

T RANSPOSITION OF GREAT VESSELS 5%

Arterial switch procedure > 95% survival (d-type)

L-type where both the 2 ventricles and the 2 great vessels are
swaped(corrected TGA)

T OTAL ANOMALOUS PV RETURN(all pulm viens drain into sys circ.

Rather than lt atrium. Vertical vien then SVC, Supracardiac ,intracardiac in
rt atrium ,or infracardiac in portal vien or IVC)

D OUBLE OUTLET RIGHT VENTRICLE(4 Types depending on the

relation of the conotruncal VSD with the great vessels.

H YPOPLASTIC LEFT HEART SYNDROME

Most common CHD presenting 1 st week of life

Most common cause of death in 1 st month of life

28.
o 4 types of DORV:
1. Subaortic VSD with or without pulmonary stenosis(like Fallots)
o 2.Subpulmonary VSD with or without subaortic stenosis and or arch obstruction
(like TGA
o 3.Doubly comitted VSD
o 4.Remote VSD

29. TOTAL ANOMALOUS PULMONARY VENOUS RETURN

30. TOTAL ANOMALOUS PULMONARY VENOUS RETURN

31. HYPOPLASTIC LEFT HEART SYNDROME

32. TRANSPOSITION OF GREAT VESSELS

33. TRUNCUS ARTERIOSUS

34. DOUBLE OUTLET RIGHT VENTRICLE

35. FONTAN PROCEDURE

36. NORWOOD PROCEDURE

37. JATENE PROCEDURE

38. CLASSIFICATION OF CHD

o OBSTRUCTIVE LESIONS

Either valvular stenosis or vascular bands

perfusion & pressure overload of corresponding ventricle

CHF common

Right sided obstructions PBF hypoxemia and cyanosis

Left sided obstructions systemic blood flow tissue hypoperfusion,

metabolic acidosis and shock

39. CLASSIFICATION OF CHD

o OBSTRUCTIVE LESIONS INCLUDE :

AORTIC STENOSIS

MITRAL STENOSIS

PULMONIC STENOSIS

COARCTATION OF AORTA 8% of CHD

80% have bicuspid aortic valve

COR TRIATRIATUM

INTERRUPTED AORTIC ARCH

40. COARCTATION OF AORTA

41. COARCTATION OF AORTA

42. INTERUPTION OF AORTIC ARCH

43. COR TRIATIATUM

44. CLASSIFICATION OF CHD

45. CLASSIFICATION OF CHD

46. ANESTHETIC MANAGEMENT

o Perioperative management requires a team approach
o Most important consideration is necessity for individualized care
o CHD is polymorphic and may clinically manifest across a broad clinical spectrum

47. ANESTHETIC MANAGEMENT

o Unpalliated
o Partially palliated
o Completely palliated

ASD and PDA only congenital lesions that can be truly corrected

Anesthesiologists will encounter children with CHD for elective non-cardiac surgery at
one of three stages:

48. ANESTHETIC MANAGEMENT

o 50% Dx by 1 st week of life; rest by 5 years
o Childs diagnosis & current medical condition will determine preoperative
evaluation
o Understand the anatomic and hemodynamic function of childs heart
o Discuss case with pediatrician and cardiologist
o Review diagnostic & therapeutic interventions
o Above will estimate disease severity and help formulate anesthetic plan

49. HISTORY & PHYSICAL

o Assess functional status daily activities & exercise tolerance
o Infants - cardiac reserve -> cyanosis, diaphoresis & respiratory distress during
feeding
o Palpitations, syncope, chest pain
o Heart murmur (s)
o Congestive heart failure
o Hypertension

50. HISTORY & PHYSICAL

o Tachypnea, dyspnea, cyanosis
o Squatting
o Clubbing of digits
o FTT d/t limited cardiac output and increased oxygen consumption
o Medications diuretics, afterload reduction agents, antiplatelet, anticoagulants
o Immunosuppressants heart transplant

51. LABORATORY EVALUATION

BLOODWORK

Electrolyte disturbances 2 to chronic diuretic therapy or renal

dysfunction

Hemoglobin level best indicator of R-L shunting magnitude &

chronicity

Hematocrit to evaluate severity of polycythemia or iron deficiency

anemia

Screening coagulation tests

Baseline ABG & pulse oximetry

Calcium & glucose - newborns, critically ill children

52. LABORATORY EVALUATION

o 12 LEAD EKG

Chamber enlargement/hypertrophy

Axis deviation

Conduction defects

Arrhythmias

Myocardial ischemia

53. LABORATORY EVALUATION

o CHEST X - RAY

Heart size and shape

Prominence of pulmonary vascularity

Lateral film if previous cardiac surgery for position of major vessels in

relation to sternum

54. LABORATORY EVALUATION

o ECHOCARDIOGRAPHY

Anatomic defects/shunts

Ventricular function

Valve function

Doppler & color flow imaging direction of flow through defect/valves,

velocities and pressure gradients

55. LABORATORY EVALUATION

o CARDIAC CATHERIZATION

Size & location of defects

Degree of stenosis & shunt

Pressure gradients & O 2 saturation in each chamber and great vessel

Mixed venous O 2 saturation obtained in SVC or proximal to area where

shunt occurs

Low saturations in LA and LV = R L shunt

High saturations in RA & RV = L R shunt

56. LABORATORY EVALUATION

o CARDIAC CATHERIZATION

Determine shunt direction: ratio of pulmonary to systemic blood flow : Qp

/ Qs

Qp / Qs ratio < 1= R L shunt

Qp / Qs ratio > 1= L R shunt

57. PREMEDICATION
o Omit for infants < six months of age
o Administer under direct supervision of Anesthesiologist in preoperative facility
o Oxygen, ventilation bag, mask and pulse oximetry immediately available
o Oral Premedication

Midazolam 0.25 -1.0 mg/kg

Ketamine 2 - 4 mg/kg

Atropine 0.02 mg/kg

58. PREMEDICATION
o IV Premedication

Midazolam 0.02 - 0.05 mg/kg titrated in small increments

o IM Premedication

Uncooperative or unable to take orally

Ketamine 1-2 mg/kg

Midazolam 0.2 mg/kg

Glycopyrrolate or Atropine 0.02 mg/kg

59. MONITORING
o Routine CAS monitoring

o Precordial or esophageal stethoscope

o Continuous airway manometry
o Multiple - site temperature measurement
o Volumetric urine collection
o Pulse oximetry on two different limbs
o TEE

60. MONITORING
o PDA

Pulse oximetry right hand to measure pre-ductal oxygenation

2 nd probe on toe to measure post-ductal oxygenation

o COARCTATION OF AORTA

Pulse oximeter on right upper limb

Pre and post - coarctation blood pressure cuffs should be placed

61. ANESTHETIC AGENTS

o INHALATIONAL AGENTS

Safe in children with minor cardiac defects

Most common agents used are halothane and sevoflurane in oxygen

Monitor EKG for changes in P wave retrograde P wave or junctional

rhythm may indicate too deep anesthesia

62. INHALATIONAL ANESTHETICS

o HALOTHANE

Depresses myocardial function, alters sinus node function, sensitizes

myocardium to catecholamines

MAP + HR

CI + EF

o Relax infundibular spasm in TOF

o Agent of choice for HCOM

63. INHALATIONAL ANESTHETICS

o SEVOFLURANE
o No HR
o Less myocardial depression than Halothane
o Mild SVR -> improves systemic flow in L-R shunts
o Can produce diastolic dysfunction

64. INHALATIONAL ANESTHETICS

o ISOFLURANE
o Pungent not good for induction
o Incidence of laryngospasm > 20%
o Less myocardial depression than Halothane
o Vasodilatation leads to SVR -> MAP
o HR which can lead to CI

65. INHALATIONAL ANESTHETICS

o DESFLURANE
o Pungent not good for induction; highest incidence of laryngospasm
o SNS activation -> with fentanyl

o HR + SVR
o Less myocardial depression than Halothane

66. INHALATIONAL ANESTHETICS

o NITROUS OXIDE
o Enlarge intravascular air emboli
o May cause microbubbles and macrobubbles to expand obstruction to blood
flow in arteries and capillaries
o In shunts, potential for bubbles to be shunted into systemic circulation

67. INHALATIONAL ANESTHETICS

o NITROUS OXIDE
o At 50% concentration does not affect PVR and PAP in children
o Mildly CO at 50% concentration
o Avoid in children with limited pulmonary blood flow, PHT or myocardial
function

68. IM & IV ANESTHETICS

o KETAMINE
o No change in PVR in children when airway maintained & ventilation supported
o Sympathomimetic effects help maintain HR, SVR, MAP and contractility
o Greater hemodynamic stability in hypovolemic patients
o Copious secretions -> laryngospasm -> atropine or glycopyrrolate

69. IM & IV ANESTHETICS

o KETAMINE
o Relative contraindications may be coronary insufficiency caused by:

anomalous coronary artery

severe critical AS

hypoplastic left heart syndrome with aortic atresia

hypoplasia of the ascending aorta

o Above patients prone to VF d/t coronary insufficiency d/t catecholamine release

from ketamine

70. IM & IV ANESTHETICS

o IM Induction with Ketamine:
o Ketamine 5 mg/kg
o Succinylcholine 5 mg/kg or Rocuronium 1.5 2.0 mg/kg
o Atropine or Glycopyrrolate 0.02 mg/kg
o IV Induction with Ketamine:
o Ketamine 1-2 mg/kg
o Succinylcholine 1-2 mg/kg or Rocuronium 0.6-1.2 mg/kg
o Atropine or Glycopyrrolate 0.01 mg/kg

71. IM & IV ANESTHETICS

o OPIOIDS
o Excellent induction agents in very sick children
o No cardiodepressant effects if bradycardia avoided
o If used with N 2 O - negative inotropic effects of N 2 O may appear
o Fentanyl 25-100 g/kg IV
o Sufentanil 5-20 g/kg IV
o Pancuronium 0.05 - 0.1 mg/kg IV offset vagotonic effects of high dose opioids

72. IM & IV ANESTHETICS

o ETOMIDATE
o CV stability
o 0.3 mg/kg IV
o THIOPENTAL & PROPOFOL
o Not recommended in patients with severe cardiac defects
o In moderate cardiac defects:

Thiopental 1-2 mg/kg IV or Propofol 1-1.5 mg/kg IV

Patient euvolemic

73. ANESTHETIC MANAGEMENT

o GENERAL PRINCIPLES
o Where:
o Q = Blood flow (CO)
o P = Pressure within a chamber or vessel
o R = Vascular resistance of pulmonary or systemic vasculature
o Ability to alter above relationship is the basic tenet of anesthetic management in
children with CHD

74. ANESTHETIC MANAGEMENT

o P manipulate with positive or negative inotropic agents
o Q hydration + preload and inotropes
o However, the anesthesiologists principal focus is an attempt to manipulate
resistance, by dilators and constrictors

75. ANESTHETIC MANAGEMENT

o GENERAL CONSIDERATIONS

De-air intravenous lines air bubble in a R-L shunt can cross into systemic
circulation and cause a stroke

L-R shunt air bubbles pass into lungs and are absorbed

Endocarditis prophylaxis

Tracheal narrowing d/t subglottic stenosis or associated vascular

malformations

76. ANESTHETIC MANAGEMENT

Tracheal shortening or stenosis esp. in children with trisomy 21

Strokes from embolic phenomena in R-L shunts and polycythemia

Chronic hypoxemia compensated by polycythemia -> O2 carrying

capacity

HCT 65% -> blood viscosity -> tissue hypoxia & SVR & PVR ->
venous thrombosis -> strokes & cardiac ischemia

77. ANESTHETIC MANAGEMENT

Normal or low HCT D/T iron deficiency -> less deformable RBCs ->
blood viscosity

Therefore adequate hydration & decrease RBC mass if HCT > 65%

Diuretics -> hypochloremic, hypokalemic metabolic alkalosis

78. ANESTHETIC MANAGEMENT

o ANESTHESIA INDUCTION
o Myocardial function preserved IV or inhalational techniques suitable
o Severe cardiac defects IV induction
o Modify dosages in patients with severe failure

79. ANESTHESIC MANAGEMENT

o ANESTHESIA MAINTENANCE
o Depends on preoperative status
o Response to induction & tolerance of individual patient
o Midazolam 0.15-0.2 mg/IV for amnesia

80. ANESTHETIC MANAGEMENT

o L - R SHUNTS :

Continuous dilution in pulmonary circulation may onset time of IV

agents

Speed of induction with inhalation agents not affected unless CO is

significantly reduced

Degree of RV overload and/or failure underappreciated careful induction

81. ANESTHETIC MANAGEMENT

o L-R SHUNTS :

GOAL = SVR and PVR -> L-R shunt

PPV & PEEP increases PVR

Ketamine increases SVR

Inhalation agents decrease SVR

82. ANESTHETIC MANAGEMENT

o R-L SHUNTS :

GOAL : PBF by SVR and PVR

PVR & SVR -> PBF

Hypoxemia/atelectasis/PEEP

Acidosis/hypercapnia

HCT

Sympathetic stimulation & surgical stimulation

Vasodilators & inhalation agents -> SVR

83. ANESTHETIC MANAGEMENT

o PVR & SVR -> PBF

Hyperoxia/Normal FRC

Alkalosis/hypocapnia

Low HCT

Low mean airway pressure

Blunted stress response

Nitric oxide/ pulmonary vasodilators

Vasoconstrictors & direct manipulation -> SVR

84. ANESTHETIC MANAGEMENT

o R L SHUNTS :

Continue PE 1 infusions

Adequate hydration esp. if HCT > 50%

Inhalation induction prolonged by limited pulmonary blood flow

IV induction times are more rapid d/t bypassing pulmonary circulation

dilution

PEEP and PPV increase PVR

85. ANESTHETIC MANAGEMENT

o COMPLEX SHUNTS :

Manipulating PVR or SVR to PBF will:

Not improve oxygenation

Worsen biventricular failure

Steal circulation from aorta and cause coronary ischemia

Maintain status quo with high dose opioids that do not significantly
affect heart rate, contractibility, or resistance is recommended

86. ANESTHETIC MANAGEMENT

o COMPLEX SHUNTS :

Short procedures slow gradual induction with low dose Halothane least
effect on +ve chronotropy & SVR

Nitrous Oxide limits FiO2 & helps prevent coronary steal & Halothane
requirements

87. ANESTHETIC MANAGEMENT

o OBSTRUCTIVE LESIONS

Lesions with > 50 mmHg pressure gradient + CHF opioid technique

Optimize preload improves flow beyond lesion

Avoid tachycardia myocardial demand & flow beyond obstruction

Inhalation agents -ve inotropy & decrease SVR worsens gradient &
flow past obstruction

88. REGIONAL ANESTHESIA &ANALGESIA

o CONSIDERATIONS

89.

Coarctation of aorta dilated tortuous intercostal collateral arteries

risk for arterial puncture and absorption of local anesthetic during
intercostal blockade

Lungs may absorb up to 80% of local anesthetic on first passage.

Therefore risk of local anesthetic toxicity in R-L shunts

o Central axis blockade may cause vasodilation which can:

Be hazardous in patients with significant AS or left-sided

obstructive lesions

Cause oxyhemoglobin saturation in R-L shunts

Improve microcirculation flow and venous thrombosis in

patients with polycythemia

REGIONAL ANESTHESIA &ANALGESIA

o Children with chronic cyanosis are at risk for coagulation abnormalities

90. POSTOPERATIVE MANAGEMENT

o Children with CHD are very susceptible to:

Deleterious effects of hypoventilation

Mild decreases in oxyhemoglobin saturation

o Therefore, give supplemental O 2 and maintain patent airway

o In patients with single ventricle titrate SaO 2 to 85%. Higher oxygen saturations
can PVR PBF systemic blood flow

91.
o Pain catecholamines which can affect vascular resistance and shunt direction
o Anticipate conduction disturbances in septal defects
o Pain infundibular spasm in TOF RVOT obstruction cyanosis, hypoxia,
syncope, seizures, acidosis and death
POSTOPERATIVE MANAGEMENT