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ANESTHESIA FOR CHILDREN WITH CONGENITAL HEART DISEASE

George Nicolaou, MD FRCPC Department of Anesthesia & Perioperative Medicine University of Western Ontario

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

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

ETIOLOGY
10% associated with chromosomal abnormalities Two thirds of these occur with Trisomy 21 One third occur with karyotypic abnormalities such as Trisomy 13, Trisomy 18 & Turner Syndrome Remaining 90% are multifactorial in origin Interaction of several genes with or without external factors such as rubella, ethanol abuse, lithium and maternal diabetes mellitus

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

CARDIOPULMONARY CHANGES AT BIRTH

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

CARDIOPULMONARY CHANGES AT BIRTH

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

CARDIOPULMONARY CHANGES AT BIRTH

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

CARDIOPULMONARY CHANGES AT BIRTH


PVR high as SVR near or at term High PVR maintained by amount of smooth muscle in walls of pulmonary arterioles & alveolar hypoxia resulting from collapsed lungs Lung expansion alveolar oxygen tension PVR

CLASSIFICATION OF CHD
L R SHUNTS
Defects connecting arterial & venous circulation SVR > PVR PBF pulmonary blood flow pulmonary congestion CHF susceptibility to RTI Long standing L-R shunts PHT PVR > SVR R-L shunt Eisenmengers syndrome

CLASSIFICATION OF CHD
L - R SHUNTS INCLUDE :
ASD 7.5% of CHD VSD COMMONEST CHD 25% PDA 7.5% of CHD
Common in premature infants

ENDOCARDIAL CUSHION DEFECT - 3%


Often seen with trisomy 21

AORTOPULMONARY WINDOW

VENTRICULAR SEPTAL DEFECT

ATRIOVENTRICULAR CANAL DEFECT

L R SHUNTS
PERIOPERATIVE TREATMENT Indomethacin PDA closure Digoxin, diuretics, ACE inhibitors CHF Main PA band PVR L-R shunt Definitive open heart surgery POSTOPERATIVE PROBLEMS SVTs and conduction delays Valvular incompetence most common after canal defect repairs

CLASSIFICATION OF CHD
R L SHUNTS
Defect between R and L heart Resistance to pulmonary blood flow PBF hypoxemia and cyanosis

INCLUDE :
TOF 10% of CHD, commonest R-L shunt PULMONARY ATRESIA TRICUSPID ATRESIA EBSTEINS ANOMALY

R L SHUNTS
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

GLENN SHUNT

MODIFIED BLALOCK-TAUSSIG SHUNT

TETRALOGY OF FALLOT
10% of all CHD Most common R L shunt 4 anomalies:
RVOT obstruction ( infundibular, pulmonic or supravalvular stenosis ) Subaortic VSD Overriding aorta RVH

TETRALOGY OF FALLOT

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

TETRALOGY OF FALLOT
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)

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

TETRALOGY OF FALLOT
Halothane HR & -ve inotropy
Rapidly tuned on and off Careful in severe RVF

Thiopental -ve inotropy Squatting, abdominal compression SVR

EBSTEINS ANOMALY

CLASSIFICATION OF CHD
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

CLASSIFICATION OF CHD
COMPLEX SHUNTS INCLUDE :
TRUNCUS ARTERIOSUS TRANSPOSITION OF GREAT VESSELS 5% Arterial switch procedure > 95% survival TOTAL ANOMALOUS PV RETURN DOUBLE OUTLET RIGHT VENTRICLE HYPOPLASTIC LEFT HEART SYNDROME Most common CHD presenting 1st week of life Most common cause of death in 1st month of life

TOTAL ANOMALOUS PULMONARY VENOUS RETURN

TOTAL ANOMALOUS PULMONARY VENOUS RETURN

HYPOPLASTIC LEFT HEART SYNDROME

TRANSPOSITION OF GREAT VESSELS

TRUNCUS ARTERIOSUS

DOUBLE OUTLET RIGHT VENTRICLE

FONTAN PROCEDURE

NORWOOD PROCEDURE

JATENE PROCEDURE

CLASSIFICATION OF CHD
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

CLASSIFICATION OF CHD
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

COARCTATION OF AORTA

COARCTATION OF AORTA

INTERUPTION OF AORTIC ARCH

COR TRIATIATUM

CLASSIFICATION OF CHD

CLASSIFICATION OF CHD

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

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

Unpalliated Partially palliated Completely palliated


ASD and PDA only congenital lesions that can be truly corrected

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

HISTORY & PHYSICAL


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

HISTORY & PHYSICAL


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

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

LABORATORY EVALUATION
12 LEAD EKG
Chamber enlargement/hypertrophy Axis deviation Conduction defects Arrhythmias Myocardial ischemia

LABORATORY EVALUATION
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

LABORATORY EVALUATION
ECHOCARDIOGRAPHY
Anatomic defects/shunts Ventricular function Valve function Doppler & color flow imaging direction of flow through defect/valves, velocities and pressure gradients

LABORATORY EVALUATION
CARDIAC CATHERIZATION
Size & location of defects Degree of stenosis & shunt Pressure gradients & O2 saturation in each chamber and great vessel Mixed venous O2 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

LABORATORY EVALUATION
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

PREMEDICATION
a) Omit for infants < six months of age b) Administer under direct supervision of Anesthesiologist in preoperative facility c) Oxygen, ventilation bag, mask and pulse oximetry immediately available d) Oral Premedication
Midazolam 0.25 -1.0 mg/kg Ketamine 2 - 4 mg/kg Atropine 0.02 mg/kg

PREMEDICATION
e) IV Premedication
Midazolam 0.02 - 0.05 mg/kg titrated in small increments

f) 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

MONITORING
Routine CAS monitoring Precordial or esophageal stethoscope Continuous airway manometry Multiple - site temperature measurement Volumetric urine collection Pulse oximetry on two different limbs TEE

MONITORING
PDA
Pulse oximetry right hand to measure pre-ductal oxygenation 2nd probe on toe to measure post-ductal oxygenation

COARCTATION OF AORTA
Pulse oximeter on right upper limb Pre and post - coarctation blood pressure cuffs should be placed

ANESTHETIC AGENTS
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

INHALATIONAL ANESTHETICS
HALOTHANE
Depresses myocardial function, alters sinus node function, sensitizes myocardium to catecholamines MAP + HR CI + EF

Relax infundibular spasm in TOF Agent of choice for HCOM

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

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

INHALATIONAL ANESTHETICS
DESFLURANE Pungent not good for induction; highest incidence of laryngospasm SNS activation with fentanyl HR + SVR Less myocardial depression than Halothane

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

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

IM & IV ANESTHETICS
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 atropine or glycopyrrolate

IM & IV ANESTHETICS
KETAMINE 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

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

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

IM & IV ANESTHETICS
OPIOIDS
Excellent induction agents in very sick children No cardiodepressant effects if bradycardia avoided If used with N2O - negative inotropic effects of N2O may appear Fentanyl 25-100 g/kg IV Sufentanil 5-20 g/kg IV Pancuronium 0.05 - 0.1 mg/kg IV offset vagotonic effects of high dose opioids

IM & IV ANESTHETICS
ETOMIDATE
CV stability 0.3 mg/kg IV

THIOPENTAL & PROPOFOL


Not recommended in patients with severe cardiac defects In moderate cardiac defects:
Thiopental 1-2 mg/kg IV or Propofol 1-1.5 mg/kg IV Patient euvolemic

ANESTHETIC MANAGEMENT
GENERAL PRINCIPLES
Q P R

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

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

ANESTHETIC MANAGEMENT
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

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

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

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

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

ANESTHETIC MANAGEMENT
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

ANESTHETIC MANAGEMENT
L-R SHUNTS :
GOAL = SVR and PVR L-R shunt
PPV & PEEP increases PVR Ketamine increases SVR Inhalation agents decrease SVR

ANESTHETIC MANAGEMENT
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

ANESTHETIC MANAGEMENT

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

ANESTHETIC MANAGEMENT
R L SHUNTS :
Continue PE1 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

ANESTHETIC MANAGEMENT

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

ANESTHETIC MANAGEMENT
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

ANESTHETIC MANAGEMENT

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

REGIONAL ANESTHESIA &ANALGESIA CONSIDERATIONS


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

REGIONAL ANESTHESIA &ANALGESIA


Central axis blockade may cause vasodilation which can:
i. Be hazardous in patients with significant AS or left-sided obstructive lesions ii. Cause oxyhemoglobin saturation in R-L shunts iii. Improve microcirculation flow and venous thrombosis in patients with polycythemia

Children with chronic cyanosis are at risk for coagulation abnormalities

POSTOPERATIVE MANAGEMENT
Children with CHD are very susceptible to:
i. Deleterious effects of hypoventilation ii. Mild decreases in oxyhemoglobin saturation

Therefore, give supplemental O2 and maintain patent airway In patients with single ventricle titrate SaO2 to 85%. Higher oxygen saturations can PVR PBF systemic blood flow

POSTOPERATIVE MANAGEMENT

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