Congen Heart Disease ASDA 2012 by Uso43b

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									Congenital Heart Disease
                Greg Gordon MD

     American Society of Dentist Anesthesiologists
            Baltimore, MD, May 3, 2012
            Training for Career in
         Pediatric Cardiac Anesthesia

        Specific Fellowship: Rare

     Suggested training (US & UK):

• Pediatric Anesthesia: 12 months
• Adult Cardiac Anesthesia: 6 months
• Pediatric Cardiac Anesthesia: 6 months
• Pediatric Critical Care: 6 months

 Baum V & De Souza DG. Pediatric Anesthesia 17:407, 2007
 White MC & Murphy TWG. Pediatric Anesthesia 17:421, 2007
Children & adults scheduled
 for dental or oral surgery
 and known to have CHD
Preop heart murmur:
 Is it CHD?
  Adults with CHD in US today




  1,500,000
           Growing 2% per year

Cahalan MK. Anesthetic Management of Patients with Heart Disease.
IARS 2003 Review Course Lectures
Andropolous, D. Anesthesia for the Patient with Congenital Heart Disease
For Noncardiac Surgery. ASA Refresher Course Lectures 2011
 3 y/o with TOF             s/p right BTS
  For dental restorations

•Turns blue with crying
•Scheduled to undergo cardiac repair
     in 3 months
•SpO2 93
•Systolic ejection murmur                   Tammy

•Slight clubbing of fingers
•Hct 52
  5 year-old for dental work

      Systolic murmur


                                    Victor
VSD


Needs surgical closure


Cardiologist recommended dental restorations first
11 y/o with tricuspid atresia
s/p Fontan procedure
For lengthy oral surgery
with possible large blood loss
 •Temporary BTS at age 3 weeks
 •Modified Fontan at age 3 years   Fran
 •Meds: digoxin, captopril
 •SpO2 88 on RA, 98 in O2
 •P 67, BP 99/42
 •First degree AV block
26 y/o with D-TGA

s/p Mustard in infancy

Dental restorations
                         Travis


Developmental delay

Pacemaker
4 y/o D-TGA

s/p Jatene in infancy

Dental restorations
                        Tracy
Very active

Keeps up with peers

Never any cyanosis
                      Objectives

Participants will be able to more intelligently discuss:


    • Newborn and infant heart and lungs

    • Initial evaluation the child’s heart

    • Pathophysiology of selected CHDs

    • Anesthetic implications of CHD
Pediatric Anesthesia
Congenital Heart Disease
      Lesson
      Presentation
      Quiz
greggordon.org
                    Fetal Circulation
Placenta (oxygenation) ->
Umbilical vein ->
Ductus venosus (liver bypass) –>
IVC ->
Foramen ovale (RV bypass) ->
Left atruim ->
Left ventricle –>
Ascending aorta (brain) ->
SVC ->
Right atrium ->
Right ventricle ->
Main pulmonary artery ->
Ductus arteriosus (lung bypass) ->
Descending aorta ->
Placenta
           The Newborn Heart
            Foramen Ovale

Functional closure first hours as LAP > RAP

Probe-patent

    50% of 5-year-olds

    25% of 20-year-olds

Paradoxical embolus
        The Newborn Heart
        Ventricular tissue

•Fewer myocytes
•Greater proportion of connective tissue
•Relative RVH
                So:

  •Decreased compliance
  •More sensitive to preload
The Newborn Heart




                    •Near peak of Starling curve
                    •Stroke volume relatively fixed
                           Normally near peak of Starling curve
                    •C.O. relatively heart rate dependent
                           Stroke volume relatively fixed
                           C.O. relatively heart rate dependent
           The Newborn Heart




                          ++
 Newborn myocardium derives relatively
 high fraction of activator Ca from the
 extracellular pool, so


Beware Ca channel blockers
             The Preterm Infant Heart

 More sensitive to depressant effects of inhaled agents
 Decreased response to catecholamines

           Relatively high PVR persists

Pulmonary vasculature more sensitive to vasoconstriction by:


             Hypoxia

             Acidosis

             Hypercarbia
       CHD Pearl




 murmur in newborn =


benign disease
    Initial evaluation of child’s heart

     History:
     To determine
Level of function
   1.Well compensated with
    no limitations
   2. Some limitations
   3. Poorly compensated with
    severe limitations
CHF and/or cyanosis
Initial evaluation of child’s heart

     History - cyanosis

•Turn blue?
•At rest?
•When crying?
•Passes out?
•Stops playing and squats
Initial evaluation of child’s heart

            History - CHF

                  Run around like crazy?
                  Like sibs?
                  Or tends to be quiet, slow?
                  Infant – feeding behavior:
                  Slow to finish bottle?
                  Sweats when nursing?
                  Eyes puffy in the morning?
Initial evaluation of child’s heart
     Physical exam
            •Listen to heart first when/if infant quiet
                   (warm stethoscope)
            •First concentrate on S1 and especially S2
                   Louder than normal?
                   Split normally?
            •Systolic murmur:
                   Starts after or obscures S1?
            •Diastolic murmur?
            •Widely radiating murmur?
            •Palpate liver
            •BP in arm and leg
            •Tongue - cyanosis
              CHD Pearl




Sudden CHF in ‘healthy’ 10-day-old =


complicated coarct
   General Approach to CHD Patient


1. Define cardiovascular pathology


2. Predict pathophysiology


3. Determine hemodynamic goals


4. Anticipate emergency treatments


        Cahalan MK. Anesthetic Management of Patients with Heart Disease.
        IARS 2003 Review Course Lectures
Recent Cardiologist Evaluation Needed?

Completely corrected,

Well compensated and stable:

Probably not

Complex and/or poorly compensated;

Cyanotic and/or single ventricle:

YES: Evaluation & ECHO within 3-6 mos
Don’t worry
Almost any anesthetic technic
may be used in any CHD patient

             if


the anesthesiologist understands
•the pathophysiology of the lesion and
•the pharmacology of the drugs employed.
                       Normal Neonate
                          1 week
  SVC                                             PV
             60
                                        99
 RA                                                    LA
 m=2          65                                       m=4




 RV                                                    LV
 30/3             65                                   80/5
                                        99



 MPA         65                              99   Ao
30/12 m=18                                         80/50
 Some basic definitions

     physiologic
     L to R shunt =
    lungs to lungs shunt
Blood that is returning to the heart
from the lungs is recirculated back
to the lungs without going out to the
rest of the body.
               Some basic definitions

   physiologic
   R to L shunt =
 body to body shunt
Blood that is returning to the heart
from the body is recirculated directly
back to the body without going to the
lungs to be oxygenated.
 Some basic definitions

     effective pulmonary
     blood flow=
     body to lungs flow
Blood that is returning to the heart
from the body that is actually directed
to the lungs to be oxygenated.
             Some basic definitions


      Nonrestrictive VSD
        VSD large enough that

pressure equalizes in the two ventricles

(no pressure gradient can be maintained)

      LV pressure = RV pressure
                  Premature
                   1 week old
  SVC             28 weeks EGA             PV

  RA                             96         LA
             65




  RV                                        LV
65/10                                       65/12

             65                  96

 MPA                 PDA                   Ao
65/30   80                                  65/25
                                      92
to R arm
& head     To L arm




                      MHMC PDA ligation
              CHD Pearl



           blue newborn +

   no airway or breathing problem +

            quiet heart =


decreased PBF lesion (TOF)
            Tetralogy Of Fallot


Most common cyanotic lesion
NB: cyanosis plus quiet heart
Diminished pulmonary blood flow
Ao ejection click
Hypercyanotic “tet” spells
                                        Tammy
  tachypnea, pallor, LOC, less murmur
 3 y/o with TOF s/p right BTS

1. Define cardiovascular pathology


2. Predict pathophysiology


3. Determine hemodynamic goals
                                     Tammy
4. Anticipate emergency treatments
          Tetralogy Of Fallot

Essentially a duality:
1. severe RVOT obstruction plus
2. nonrestrictive VSD

 With anatomic consequences:
 1. RVH
 2. Overriding aorta
                                     Tammy
And physiologic consequences
1. R to L shunt
2. Diminished pulmonary blood flow
                  Tetralogy of Fallot

  SVC
             40                         96
 RA                                                LA
 m=5                                               m=4




 RV                                                LV
 85/6                                              85/5
              40                        85



MPA                                          50   Ao
15/10   40
                                                  85/45
      Tetralogy Of Fallot



s/p   right BTS?

Blalock-Taussig Shunt
                            Tammy
 Thomas-Blalock-Taussig Shunt




Vivien Thomas


                           Alfred Blalock


                                                       Helen Taussig




      Vivien Thomas, Partners of the Heart, 1998 and
  Something the Lord Made - Best Made-for-TV Movie, 2004
Thomas-Blalock-Tuassig
Dr. Blalock does the Blalock
       (Johns Hopkins)
Systemic to Pulmonary Shunts
        Tetralogy Of Fallot - Goals
Maintain adequate tissue oxygenation

1. Avoid increasing O2 demand
2.Maintain SVR, systemic BP
3.Minimize PVR

Maintain good hydration,
    especially if polycythemic
                                       Tammy
Oral premed/induction
midazolam + ketamine
(0.6 mg/kg + 6 mg/kg)
     Tetralogy Of Fallot - Goals
          Minimize PVR
Oxygen to FIO2 = 1
Mild hyperventilation
    PaCO2 low 30’s
    pH 7.45
Adequate anesthesia
Adequate analgesia                 Tammy
Normothermia, warm
Nitric oxide
   Tetralogy Of Fallot - Goals
        Maintain SVR
               Intravascular volume

                    Well hydrated

                    IV bolus prn

               Maintain BP
Tammy               ketamine

                    phenylephrine
Free written board answer:

Speed of induction:

R->L shunt
• Inhalational: slower
• IV: faster

L->R shunt
• Inhalational: maybe faster
• IV: slower
 But probably not clinically important

                 Tanner et al. Anesth Analg 64:101, 1985
        Beware:
        blunted chemoreceptor response to


            hypoxemia
Tammy
        Beware:
        VD:VT may be 0.6
        And increase with
        •start of mechanical ventilation
Tammy
        •too much PEEP
        •hypovolemia
          ETCO2 << PaCO2
         VD/VT = (PaCO2 – ETCO2)/PaCO2
      Tetralogy Of Fallot

 Minimize R->L Shunt

MAINTAIN      SVR
 •ketamine
 •phenylephrine             Tammy
        Tetralogy Of Fallot

Minimize RVOT obst & PVR
   •oxygen
   •beta blocker ready
    Maybe:
    •nitroglycerin
    •phentolamine             Tammy
    •tolazoline
    •prostaglandin E1
    •nitric oxide
       Tetralogy Of Fallot

And of course:

 •No Air in lines
    Maybe no N2O
       and

  infective
  endocarditis               Tammy

  prophylaxis
Infective Endocarditis Prophylaxis
Infective endocarditis prophylaxis

for dental procedures is reasonable

only for patients with underlying

cardiac conditions associated with

the highest risk of adverse outcome

from infective endocarditis.

     Wilson W, Taubert KA et al. AHA Guidelines. Prevention of
     Infective Endocarditis. Circulation 116:1736-54, 2007
Infective Endocarditis Prophylaxis
         Recommended

   Unrepaired cyanotic CHD,

   including palliative

   shunts and conduits.


                             Circulation 116:1736, 2007
Infective Endocarditis Prophylaxis
         Recommended

  CHD completely repaired with


  prosthetic material or device


  less than 6 months ago.


                             Circulation 116:1736, 2007
Infective Endocarditis Prophylaxis
         Recommended

  Repaired CHD with


  residual defect(s) at or near


  a prosthetic patch or device.


                             Circulation 116:1736, 2007
Infective Endocarditis Prophylaxis
         Recommended

  Prosthetic material in a valve.


  Previous infective endocarditis.


  Valvulopathy after transplant.


                             Circulation 116:1736, 2007
      Infective Endocarditis Prophylaxis
               Recommended

For patients with the above conditions,
prophylaxis is reasonable for
all dental procedures that involve
manipulation of gingival tissue or
the apical region of teeth or
perforation of the oral mucosa.

                                   Circulation 116:1736, 2007
     Infectious Endocarditis Prophylaxis

        NOT Recommended

Any form of CHD not listed above

Local injection -> noninfected tissue

Shedding deciduous teeth

Bleeding/trauma to lips, oral mucosa

                                   Circulation 116:1736, 2007
     Tetralogy Of Fallot
infective endocarditis prophylaxis

            and
    maintain


SVR                                  Tammy
                   Tetralogy Of Fallot
             Treatment of Tet Spell
• 100% O2
• knee-chest position
• morphine 0.05-0.1 mg/kg
• crystalloid 15-30 ml/kg
• phenylephrine to increase systolic BP 20-40 mmHg
• beta blockade: propranolol 0.1 mg/kg or
       esmolol 0.5 mg/kg and 50-300 mcg/kg/min
• ABG: NaHCO3 if necessary
• ECMO/surgery

   DiNardo JA et al. in Davis PJ et al. Smith’s Anesthesia for Infants and Children, 8th ed. 2011
Schedule case early in the day
•Less fasting dehydration
•Less time of stress
•More time to monitor postop
•More support available
•Less team turnover
Schedule case WHERE?
•Well-compensated, no limitations,
not-complex:
Ambulatory center may be OK
•Not well-compensated, complex:
Center with CHD expertise &
backup available
 5 year-old for dental work

      Systolic murmur


                                    Victor
VSD


Needs surgical closure


Cardiologist recommended dental restorations first
            Newborn VSD
Most common lesion

2/3rds close spontaneously

Small VSD

      Definite murmur

      Will probably close

Large VSD

      No murmur

      No problems

      Home with Mom
      CHF symptoms by 4-8 weeks
                     VSD
                  nonrestrictive
  SVC
                                        98
         60

 RA                                96          LA
m=6                                            m=12




RV           80                                LV
90/8                               94         90/10



        88                              94    Ao
 MPA
90/35                                        90/60
          Nonrestrictive VSD

             L->R shunt

Pulmonary to System Flow Ratio


          SaO2 – SvO2
          __________             Victor
QP:QS =
          SpvO2 – SpaO2

            94 - 60
           _______
      =
            98 - 88

      =     3.4:1
      Nonrestrictive VSD - Goals

Maintain PVR
     Normal ventilation
         (paCO2 = 40’s)
     FIO2 < 1                      Victor


Lower SVR better
      Major inhalational agents

      Propofol, thiopental
Proper management of the physiologic

abnormalities is more important

than the choice of specific anesthetic

and pharmacologic approaches.
   Nonrestrictive VSD - Goals

     Of course:


•No Air in lines
     Maybe no N2O

                                            Victor
infective
endocarditis
prophylaxis?

NO longer recommended

        Wilson W, Taubert KA et al. AHA Guidelines. Prevention of
        Infective Endocarditis. Circulation 116:1736-54, 2007
Unrepaired nonrestrictive VSD ->

1. PVOD developing
2. Less L->R shunt
3. Less CHF
4. Less murmur
5. PVOD irreversible         Victor

6. R-L shunt
7. Less PBF
8. More cyanosis

Eisenmenger syndrome
11 y/o with tricuspid atresia
s/p Fontan procedure
Oral surgery, big blood loss?
 •Temporary BTS at age 3 weeks

 •Modified Fontan at age 3 years
                                   Fran
 •Meds: digoxin, captopril

 •SpO2 88 on RA, 98 in O2

 •P 67, BP 99/42

 •First degree AV block
     Tricuspid Atresia
3rd most common cyanotic CHD
1. TOF
2. TGA
Type IB most common
•Small VSD (and RV)
•PS
                                 Fran
20% extracardiac abnormalities
•GI
•Musculoskeletal

Cyanosis
•Mixing in LA
•Decreased PBF
•Spells
       Fontan procedure

Indicated to palliate:

Tricuspid atresia

Hypoplastic left heart syndrome

Double outlet right ventricle

Double inlet left ventricle

Unbalanced AV septal defect
        Fontan physiology


Two defining features:
1. Single systemic ventricle

2. Pulmonary blood flow:


without pump!
           Fontan procedure

Three main versions
Atriopulmonary connection (the original)
Total cavopulmonary connection (TCPC):
     Intracardiac (lateral tunnel)
     Extracardiac

Two stages:
Bidirectional Glenn shunt
Fontan completion
Modified
Bidirectional
Modified
Collaborate with cardiologist
Clarify
   History
   Pathophysiology
   Risks
Status best possible?
Explain recent studies
                Age 5 years




        16/10




16/12
                  88/6
11 y/o with tricuspid atresia s/p Fontan procedure
Potential problems during surgery

          Hypoxemia
          1. Hypovolemia
          2. Low PBF
          CHF
          1. Volume shifts
          2. Anemia                             Fran
          3. Hypertension
          Paradoxical embolus
          Thrombosis
            Vena cavae
            RA
            Pulmonary arteries
11 y/o with tricuspid atresia s/p Fontan procedure

Goals during surgery
  Monitor RA pressure
  •RA catheter
  •Maintain starting pressure

  Maintain systemic BP near baseline

  Minimize myocardial depressants               Fran
  NO AIR IN LINES
  No N2O

  Relatively high FIO2

  Normal Hct

  IE prophylaxis
11 y/o with tricuspid atresia s/p Fontan procedure

MAJOR GOAL
  Maintain cardiac output and
  transpulmonary gradient (TPG):
  Adequate preload
  Low PVR
  Low intrathoracic pressure                    Fran
  Normal ventilation
  Unobstructed PV return
  Regular sinus rhythm
  Low ventricular afterload
  Normal ventricular funtion
                Monitor RA Pressure
                Right IJ?




        16/10


                                      Fran


16/12
                        88/6
           CHD Pearl


        blue newborn +

no airway or breathing problem +

      hyperactive heart =




         TGA
26 y/o with D-TGA

s/p Mustard in infancy

Dental restorations
                         Travis


Developmental delay

Pacemaker
TGA s/p Mustard
 D-TGA, Transposition of the Great Arteries


Newborn: 75% no VSD

PGE1 to keep PDA

BAS prior to surgery
                                      Travis

Older: Mustard or Senning

Younger: Jatene ASO
           D-TGA

SVC


            BAS
RA                      LA
                   99




RV                      LV
                   99




 Ao   65    PDA         MPA
                D-TGA
           Mustard Procedure
SVC                            PV




RV                              LV




      95
Ao                             MPA
        D-TGA + Mustard

RV systemic ventricle

RV failure
Tricuspid regurgitation
Ventricular arrhythmias
                          Travis
Sudden death

Atrial injury/scars
Atrial flutter/fib
Sick sinus syndrome
26 y/o TGA s/p Mustard, pacemaker


Poor RV function
Consider inotrope
Arrhythmias
                                    Travis

Pacemaker
CIED practice advisory
                CIED practice advisory
    Preop:
    What type CIED?
    Pacer dependent?
    Check function: interrogate device
    EMI (e.g. Bovie) during procedure?
    Reprogram to asynchronous mode?
    Have backup pacing &
        defibrillation equipment
        immediately available
Practice Advisory for the Perioperative Management of Patients with Cardiac
Implantable Electronic Devices: Pacemakers and Implantable Cardioverter-
Defibrillators. Anesthesiology 114:247-61, 2011
4 y/o D-TGA

s/p Jatene in infancy

For dental restorations

                          Tracy
Very active

Keeps up with peers

Never any cyanosis
       D-TGA

 SVC


RA              LA




                LV
RV




 Ao            MPA
       D-TGA

 SVC


RA              LA




                LV
RV




 MPA           Ao
   4 y/o D-TGA s/p Jatene

Treat as normal, healthy child!




                            Tracy




         Be happy!
     For more cool stuff about CHD
   check out the lesson and fun Quiz at


http://greggordon.org/edu/ped/chd1.htm
Now we can more intelligently discuss:

  •Newborn and infant heart and lungs

  • Initial evaluation the child’s heart

  • Pathophysiology of selected CHD

  • Anesthetic implications of CHD

								
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