1. Lecturer Of Anesthesia
Anesthesia In Children With Congenital Heart Disease For Non-cardiac Surgery
Dr. Fady Adib
Lecturer Of Anesthesia
Ain-Shams University
Oct. 2012

2. The question facing anesthetists are:
1 . Should the patient be referred to specialist cardiologist before surgery?
2 . Should surgery be performed in a center specializing in congenital cardiology?
3 . How should anesthesia be conducted safely in the presence of congenital heart disease?

3. INTRODUCTION

4. The Incidence of CHD is about 1% of the newborn infants
- Surgery:
- Noncardiac conditions ( inguinal hernia, circumcision, or tonsillectomy).
- Associated noncardiac congenital anomalies (orthopedic or genitourinary).

5. Patient with congenital heart disease can be either:
A known cardiac lesion.
( Corrected or Uncorrected)
Accidentally discovered case.

6. - Due to advances in diagnosis, medical, critical and surgical care for CHD
- Therefore, it is common for patients with CHD to present for non-cardiac surgery, and even in patient with corrected CHD significant residual problems (arrhythmias, ventricular dysfunction, shunts, valvular stenosis, and PH) may be exist.

7. Anatomical & Physiological Differences

8. CVS: Anatomical Differences
- Myocardium is less compliant:
Cardiac Output is H.R. dependent.
- It can’t withstand a volume load.
- Decreased sympathetic innervation:
 catecholamine stores

9. Autonomic Development
- Beta receptors develop with age
- alpha receptors less developed
So V.C response to volume loss is decreased
- Parasympathetic response supervens
- Net result:
- Volume load heart failure
- Volume loss decrease vasoconstriction
- Hypotension

10. CLASSIFICATION OF CHD I- Acyanotic congenital heart disease: ASD VSD PDA II- Cyanotic congenital heart disease: Tetralogy of Fallot, with severe right ventricular outflow obstruction TGA Pulmonary atresia or severe stenosis Tricuspid atresia with pulmonary stenosis Truncus Arteriosus

11. Pathophysiological classification of congenital heart disease
- Shunt lesions
- Mixing lesions
- Obstructive lesions
- Regurgitation lesions

12. Shunt lesions - Direction & magnitude:
Intracadriac
e.g. ASD, VSD
Extracardiac:
e.g. PDA
- Direction & magnitude:
depends on size of shunt orifice
pressure on both sides of the shunt
- Lt.-to-Rt. Shunt ( e.g. VSD,PDA) VOLUME OVERLOAD
- Rt.-to-Lt. Shunt (e.g. Fallot 4) PRESSURE OVERLOAD

13. ..Shunt lesions.. (continued)
Lt-to-Rt shunt
e.g. ASD, VSD
Rt-to-Lt shunt
e.g. F4
- ↓ Pulmonary blood flow= hypoxemia
- Pressure overload of the Rt. ventricle
- Volume overload on
pulmonary circulation
- Increased cardiac work of Rt. Ventricle
- Excessive pulmonary
blood flow=++ PVR

14. Mixing lesions (large orifice)
- e.g. TGA, Univentricular heart
- Direction & Magnitude:
Depend on PRESSURE on both sides of the lesion
- Usually CYANOTIC
- With VOLUME & PRESSURE overload

15. Obstructive lesions - e.g. Aortic stenosis, Coarctation of Aorta
- Pressure overload ventricular hypertrophy
impaired coronary perfusion
systemic hypotension

16. Regurgitation lesions (uncommon)
- Volume overload ventricular dilatation

17. Once full details of the anatomy, surgical history and current pathophysiology are obtained patient can be divided into the following categories:
1 . Congenital heart disease, yet to be surgically treated.
2 . Surgically corrected, symptom free with no new development.
3 . Surgically corrected, symptomatic heart diseases
4 . Surgically palliated. Symptoms stable with no new development
5 . Surgically palliated with severe symptoms or no new development

18. Types of Cardiac Surgery
Biventricular
Univentricular
Usually complete repair
( Corrective )
Usually Palliative
e.g. Shunts, Banding
Problems encountered:
Debubbling & Antibiotic coverage,
As the lesion is still persistent e.g. VSD

19. - Corrective surgery: Correct anatomy
- Palliative surgery: The aim is to increase or decrease pulmonary blood flow

20. Corrective Surgery
Corrected ASD VSD PDA: Near normal patient. Only needs antibiotic prophylaxis.
Repaired TOF: Residual defects: VSD, outflow tract obstruction, pulmonary regurge, Heart block,
Corrected coarcitation usually require long term treatment of hypertension.

21. Palliative surgery Decrease PBF: Pulmonary artery banding (VSD)
Increase PBF: In Pulmonary atresia
BT shunt: Subclavian artery to Pulmonary artery.
Gllen: SVC to Pulmonary artery
Fontan after Gllen IVC to Pulmonary artery

26. Physiology of different types of circulation
- Normal or ‘series’ circulation.
- Parallel or ‘balanced’ circulation.
- Single-ventricle circulation

27. Risk classification increased risk of mortality and morbidity.
- factors associated with a high risk of
perioperative complications,
- {disease complexity, }
- {physiological status,}
young age, and Hospital Stay . - type of surgery,
Most Important

28. Complexity of heart disease
- single-ventricle physiology
- balanced circulation physiology
- cardiomyopathy
- aortic stenosis

29. Physiological status - Cardiac failure - Pulmonary hypertension
Physiological status can be divided into four major risk factors:
- Cardiac failure
- Pulmonary hypertension
- Arrhythmias
- Cyanosis

31. ANESTHETIC MANAGEMENT

32. - Perioperative management requires a team approach
- CHD is polymorphic and may clinically manifest across a broad clinical spectrum
- The plane of Anesthetic Management includes the following:
A - Preoperative Management
B - Intraoperative Management
C - Postoperative Management

33. Preoperative Anesthetic Considerations
1- Complete history and physical examin.
2- Review all investigations
3- Hydration should be maintained
4- All cardiac medication except possibly digitalis ,ACE and diuretics should be continued until surgery
5- Premedication should be give particularly to patients
at risk for right to left shunt
6- Antibiotic prophylaxis against endocarditis must be
given

34. Preoperative Anesthetic Management: A- History B- physical examination C- Investigations D- Premedications E- Fasting Guidelines

35. HISTORY & PHYSICAL EXAMINATION
- Vital signs
- Airway abnormality
- Associated extracardiac congenital anomalies
- Tachypnea, dyspnea, cyanosis
- Squatting
- Clubbing of digits
- Heart murmur (s)
- CHF:
- Jugular venous distention.
- Hepatomegally
- Ascitis
- Peripheral edema
- Assess functional status
- daily activities
- exercise tolerance
- ↓ cardiac reserve
- cyanosis
- respiratory distress during feeding
- Cyanosis
- Dyspnea
- Fainting attack
- Fatigue
- Palpitations
- chest pain
- Syncope
- Abdominal fullness
- Leg swelling
- Medications

36. Cardiac Catheterization
MRI
Laboratory Evaluation
12 Lead ECG
INVESTIGATIONS
chest X – Ray
Echocardiography
Cardiac Catheterization

37. Regarding investigations of CHD patients for non-cardiac surgery: A- Chest X – Ray has no rule B- Cardiac Catheterization is the first choice for diagnosis of CHD C- Echocardiography non invasive method for diagnosis of CHD D- MRI cannot give us idea about pulmonary veins IM Premedication for CHD patients presenting for non-cardiac suergery: A- Cooperative or unable to take orally B- Ketamine 1mg/kg C- Midazolam 5 mg/kg D -Glycopyrrolate or Atropine 0.02 mg/kg Procedural antibiotic prophylaxis is required in patients with A- Aortic valve replacement B- Mitral valve prolapse with regurge C- Previous history of infective endocarditis D- Ostium secundum ASD

38. AHA guidelines for bacterial endocarditis Prophylaxis in patients with cardiac conditions
Endocarditis prophylaxis not recommended
Endocarditis prophylaxis recommended
Negligible-risk category
- Physiologic, or functional heart murmurs
- Surgical repair without residua beyond
6 months : ASD, PDA,VSD
- Cardiac pacemaker or
- implanted defibrillator
- Isolated secundum atrial septal defect
- Mitral valve prolapse without reg.
- Previous coronary artery bypass surgery
- Previous rheumatic heart disease without valvular dysfunction
High-risk category
- Complex cyanotic congenital heart disease :
Transposition of the great vessels
Tetralogy of Fallot
- Surgically created systemic-to-pulmonary shuntsor conduits
- Prosthetic, Bioprosthetic, Homograft valves
- Previous bacterial endocarditis
Moderate-risk category
- Other congenital cardiac anomalies
- Acquired valvular dysfunction
- Hypertrophic cardiomyopathies
Regurg-- Mitral valve prolapse with valvar

39. AHA guidelines for antibiotic prophylaxis: dental, oral, Respiratory tract and esophageal procedures
Amoxicillin 1 h before procedure
-Children: 50 mg/kg p.o.
Adults: 2.0 g p.o.-
Standard prophylaxis
Ampicillin within 30 min before procedure
- Children: 50 mg/ kg i.m. or i.v.
Adults: 2.0 g i.m. or i.v. -
Unable to take oral medications
Clindamycin 1 h before procedure
Children: 20 mg/kg p.o.
Adults: 600 mg p.o.
OR Azithromycin or clarithromycin 1 h before procedure
-Children: 15 mg/kg p.o.
Adults: 500 mg p.o.-
Allergic to penicillin
Clindamycin within 30 min before procedur
-Children: 20 mg/ kg i.v
-Adult: 600 mg i.v..-
AND allergic to penicillin

40. AHA guidelines for antibiotic prophylaxis: genitourinary and gastrointestinal procedures
- within 30 min before procedure
- Children: Ampicillin 50 mg/ kg .and gentaicin 1.5 mg/kg i.m or i.v
- Adults: Ampicillin 2.0 g and gentamicin 1.5 mg/kg i.m or i.v
High risk patients
- Complete infusion 30 min before procedure
- Children: Vancomycin 20 mg/kg i.v over 1-2 hr
gentamicin 1.5 mg/kg i.m or i.v
-Adults: Vancomycin 1g/kg i.v over 1-2 hr
High risk patients Allergic to penicillin
Ampicillin within 30 min before procedure-
- Children: 50 mg/ kg i.m. or .iv
- Adults: 2.0 g i.m or i.v
Moderate risk patients
Complete infusion 30 min before procedure-
Moderate risk patients AND allergic to penicillin

41. Anesthetic Management
A - Preoperative Management
B - Intraoperative Management :
1- Monitoring
2- Choice of anesthetic agent
3- Maintenance of anesthesia
4- Emergence from anesthesia

42. Anesthetic Management Preoperative Assessment
- Associated congenital anomalies (difficult airway)
- Chest: signs of H.F.& chest infection…postpone
- Liver:
●enlarged in Rt. Sided failure
●shrunken in Lt. sided failure, diuretics, ↓feeding
- Cyanotic spells
- Acute hypertensive pulmonary crisis

43. Anesthetic Management Investigations
- Hematocrit:….thromboembolism
- Electrolytes:…..arrhythmias..(should be corrected)
- Blood gases:…cyanotic may be acidotic
- Echocardiography: satisfactory in simple cases
- Catheterization:
- Coagulation profile: cyanotic patients usually suffer from coagulopathies.

44. Premedication Fasting Guidelines - Oral Premedication:
- Midazolam mg/kg
- Ketamine mg/kg
- Atropine 0.02 mg/kg
IV Premedication:
- Midazolam mg/kg titrated in small increments
- Ketamine 1-2 mg/kg
- IM Premedication:
- Uncooperative or unable to take orally
- Ketamine 5 – 10 mg/kg
- Midazolam 0.2 mg/kg
- Glycopyrrolate or Atropine 0.02 mg/kg
Fasting Guidelines

45. Anesthetic Management Premedication
- Fasting: clear sugar fluid allowed till 4 hours
- ↓ 6 months: No premedication
- 6-9 months: optional e.g. to avoid spell or crisis
↑9 months:
-atraumatic (oral midazolam)
-IM: ketamine 2mg/kg
add Atropine 0.02mg/kg

46. Anesthetic Management O.R. preparation
- Temperature control: mattress & O.R. temp.
- Anesthetic machine: with O2, Air, N2O
- Infusion set…… free of air bubbles
- Drugs: Atropine, Bicarb., Epinephrine,
Phenylephrine
- Inotropic infusion should be premixed before induction in high risk patient (e.g.Dobutamine)

47. Anesthetic Management Monitoring & Lines
- ECG
- Pulse Oximetry: inaccurate in deep hypothermia
- Invasive B.P.: Lt. Radial, Rt. Radial, Femoral art.
- CV. Cannulation: Rt. & Lt. IJV, Femoral vein
- Temperature: central & peripheral
- Urine output:
- Capnography:
- Blood gases & electrolytes.

48. Monitoring Non-invasive Invasive - Clinical observation
- ECG
- NIBP
- Pulse oximetry
- Precordial stethoscope
- Continuous airway manometry
- Multiple site temperature measurement
- Volumetric urine collection
- Art. catheterization
- CVP
- PAC
- TEE

49. Inraoperative management

50. The goals of Intraoperative management
- Prophylaxis against Subacute bacterial endocarditis.
- Prophylaxis against Air bubble embolism.
- Hemodynamic management.
PVR
SVR
Contractility

51. Air bubble precautions
- Check and remove all air bubbles from IV tubing, injection ports, and stopcocks
- Connect the IV tubing to the venous cannula while there is a free flowing IV fluid and blood.
- Before IV injection into the cannula, small amount if fluid is injected into the hub of the cannula.

52. Air bubble precautions
Aspiration before injection to clear any air.
Hold the syringe upright to keep the bubbles away.
Do not inject the last milliliters from the syringe.
Do not leave the central line open to air.
N2O is better avoided.

53. Hemodynamic management

54. Left to right shunts: ( pulmonary blood flow)
- The aim is to prevent:
in SVR
in PVR
contractility
Avoid
vasodilators
high FiO2
hypocapnea
and alkalosis

55. Right to left shunts: ( pulmonary blood flow)
- The aim is to prevent:
in SVR
in PVR
contractility
Avoid
Sympathetic
stimulation
low FiO2
hypercapnea
acidosis.
Avoid N2O

56. Anesthetic Management Induction
- Aim: to preserve SVR & PVR
- Method:
● Inhalational: Sevoflurane
● I.V.: ketamine 2 mg/kg + fentanyl 2-3μg/kg+
Pancuronium 0.1 mg/kg
- Antibiotic Prophylaxis:
- Intubation: Oral/ Nasal (postoperative)
- Corticosteroids: decrease systemic inflammatory response

57. The effect of shunt on the speed of induction of anesthesia:
- In patients with a right-to-left shunt:
Inhalation induction is prolonged.
Intravenous induction is more rapid.
- In patients with left-to-right shunting, the speed of inhalation or intravenous induction is not changed.

58. Factors Affecting PVR Factors Increasing: Factors Decreasing: - PEEP
- High airway pressure
Atelectasis,hypoxia,hypercarbia
- Acidosis
- Catecholamine
-High hematocrite
Factors Decreasing:
- No PEEP
- Low airway pressure
- High FiO2, hypocarbia
- Alkalosis
- Vasodilators
- Low hematocrite
- Nitric oxide

59. Anesthetic Management Maintenance
- Patient with Poor Myocardium:
- Narcotic based....Extubation not advisable
- Patient with Good Myocardium:
- Inhalational (isoflurane, sevoflurane)
- Most Stressful Situations:
- Skin incision
- Sternotomy
- Major vessels cannulation (Aorta, SVC,IVC)

60. Choice of anesthetic Regimen
● Development of anesthetic regimen is based on various factors such as presence and direction of shunts , HF, arrhythmia , pulmonary circulation, and lowering or maintenance of PVR

61. Choice of Anesthetic Agent
Intravenous anesthetics
Volatile anesthetics
Muscle relaxants -
Ketamine : No change in PVR in children when airway maintained & ventilation supported
Sympathomimetic effects help maintain HR, SVR, MAP and contractility
Greater hemodynamic stability in hypovolemic patients
Copious secretions (laryngospasm)
Etomidate : Induction dose of 0.3mg/kg  no change in mean pulmonary artery pressure and PVR, pulmonary blood flow, PHT or  myocardial function
Propofol : decrease in SBP and SVR, and increase in SBF in all patients, whereas HR ,PAP, PBF remained unchanged
OPIOD: Excellent induction agents in very sick children No cardiodepressant effects if bradycardia avoided Fentanyl µg/kg IV , Sufentanil 5-20 µg/kg IV
Barbiturates : Not recommended in patients with severe cardiac reserve

62. Choice of Anesthetic Agent (Cont.)
- Desflurane  Pungent , PAP and  PVR, Less myocardial depression than Halothane  HR , SVR
- Halothane   PBF not affecting PVR, Depresses myocardial function, alters sinus node function,
sensitizes myocardium to catecholamines
- Isoflurane  Pungent,  PAP not affecting PVR, Less myocardial depression than Vasodilatation leads to  SVR →  MAP ,  HR which can lead to  CI
- Sevoflurane  Less myocardial depression than Halothane, more  in PAP compared with isoflurane, No  HR, Mild  SVR, Can produce diastolic dysfunction
- Nitrous oxide  At 50% concentration does not affect PVR and PAP in children
Avoid in children with limited pulmonary blood flow, PHT or  myocardial function

63. Neuromuscular Blocking Drugs
Nondepolarizing
Depolarizing
- Succinylcholine in pediatric is controversial
- If used should be with atropine, to avoid associated brady cardia or sinus arrest
- also if used with potent narcotic atropine should be used to avoid severe bradycardia in children with  CR
- Atracuruim and vecronium: have few cardiovascular side effects in children when given in recommended doses.
- Pancuronuim if given slowly doesn't produce HR or BP changes. if given as bolus doses it can produce tachycardia , ↑BP (through sympathomimetic effect )
-Cisatracuruim and Rocuroinuim: safe

64. Anestheia of Fallot patient:
- The aim is to prevent intraoperative cyanotic spells.
- Avoid prolonged fasting
- Heavy sedative premedication.
- Intravenous induction.
- Ketamine, Fentanyl, Pancronium, Halothane.
- Adequate intravascular volume.
- Avoid systemic vasodilatation.
- Adequate anesthetic depth to avoid sympathetic stimulation.

65. Management of intra operative cyanotic spells in Fallot patient
- Direct abdominal or aortic compression
- IV vasoconstricror as ephedrine, phenylephrine, or dopamine
- IV fluid.
- Deep level of anesthesia.
- Beta adrenergic blockers as osmolol or propranolol

66. REGIONAL ANESTHESIA &ANALGESIA
Considerations :
- Coarctation of aorta considerations
- Childern with chronic cyanosis  risk of
coagulation abnormality
- VD : which can:
1- be hazardous in patients with significant
AS or left-sided obstructive lesions
2- Cause  oxyhemoglobin saturation in R-L shunts

67. Postoperative Anesthetic Management
- Supplemental O2 and maintain patent airway.
- In patients with single ventricle titrate SaO2 to 85%. Higher oxygen sat. can  PVR  PBF   SBF
 Pain  catech. which can affect VR and shunt direction
 Pain  infundibular spasm in TOF  RVOT obstruction  cyanosis, hypoxia, syncope, seizures, acidosis and death
 Anticipate conduction disturbances in septal defects

68. Remember Management of - Acute hypertensive pulmonary crisis:
- Ventilatory manipulation: reduce PVR
(PaO2, PaCO2, PH, Lung volumes)
- Drugs: Milrinone, Isopril, PgE2

69. Remember Management of - Intraoperative Cyanotic Spells
-Increase S.V.R.: by direct aortic compression ± vasopressor (phenylephrine, ephedrine…..)
-Reduce infundibular obstruction by ß-Blockers (esmolol, propranolol…), OR Halothane
-Deepen the level of anesthesia.
-Adequate hydration (ample fluid and decrease viscosity)

70. SUMMARY
- Familiarity with the CHD pathophysiology, adequate preoperative preparation, choice of monitors, induction, maintenance , emergence from anesthesia, and plans for the postoperative period to avoid major problems in anesthetic management
- A wide variety of anesthetic regimens is used for patients with congenital heart disease (CHD) undergoing cardiac or non-cardiac surgery, or other diagnostic or therapeutic procedures. The goal of all of these regimens is to produce anesthesia or adequate sedation, while preserving systemic cardiac output and oxygen delivery

71. Thank You