Anesthesia for Adult Patient with
Congenital Heart Disease
Mohamed Saleh, MD
Department of Anesthesia and Intensive Care,
Ain-Shams University
Case Scenario
• A 17 years-old patient, was presented for
emergency appendectomy. He had a history
of repaired TOF at the age of 2 years. 12 years
later, he started to complain of palpitations,
exertional dyspnea, and was diagnosed as
pulmonary regurgitation and right ventricular
dysfunction.
• How to manage this patient in the perioperative period?
Introduction
Complete
surgical repair
Partial or
palliative surgery
Journal of Cardiothoracic and Vascular Anesthesia, Vol 20, No 3 (June), 2006: pp 414-437
Non-operated
Arrhythmias
Hematologic
dysfunction
Decreased
Pulmonary Blood
Flow
Increased
Pulmonary blood
Flow
Hypoxemia
Pulmonary HTN
Hemostatic
Dysfunction
Renal
dysfunction
Neurologic
disease
Pressure and
/or Volume
over load
Ventricular
dysfunction
Arrhythmias
Pulmonary hypertension
Ventricular dysfunction
• Volume Overload
• Pressure Overload
Hematologic dysfunction
• Erythrocytosis
• Hyper-viscosity
• Iron deficiency anemia
Hemostatic Dysfunction
•
•
•
•
Thrombocytopenia
Platelet function abnormalities
Disseminated intravascular coagulation
Decreased production of coagulation
factors
– Impaired liver function
– Vitamin K deficiency
• Primary fibrinolysis
Renal dysfunction
•
•
•
•
•
Hypercellular glomeruli
Basement membrane thickening
Focal interstitial fibrosis
Tubular atrophy
Hyalinization of afferent and
efferent arterioles.
Neurologic disease
• Cerebral abscess
• Cerebral emboli /
thrombosis
Common congenital heart diseases
Atrial Septal Defect
Anatomy Physiology
Potential Issues
Specific Anesthetic
Management
L-R shunt
• Small to moderate size
defects well tolerated
• Atrial fibrillation (increased
risk if repaired after age 40)
• Risk of paradoxical emboli
• Large defects lead to
arrhythmias, exercise
intolerance, and rarely PHT
(occurs in less than 5% of
patients)
De-air intravenous lines
Ventricular Septal Defect
Anatomy Physiology
Potential Issues
Specific Anesthetic
Management
L-R shunt
Unrepaired:
• Large defect risk of PHT (50% by
age 2)
• Small to moderate size defects,
risk for endocarditis, subpulmonic obstruction, subaortic
obstruction, and aortic
regurgitation
• Right ventricular failure
Repaired:
• Complete heart block in some
patients (rare)
• Persistent PHT
• Dysrhythmia
Manage L-R shunt
May be
associated
with
other defects
Maintain pulmonary blood
flow if R-L shunt present
Increased risk of
postoperative
pulmonary infection
Manage pacemaker
Coarctation of the aorta
Anatomy Physiology
Potential Issues
Specific Anesthetic
Management
LV pressure
overload and
Hypertrophy
• Blood pressure gradient between
upper and lower limbs
• Risk of bleeding if thoracic
surgery
• LV hypertrophy and LV diastolic
dysfunction
• Systemic hypertension
• Aneurysms of ascending aorta
and descending aorta
• Premature coronary artery
disease
• Intracranial aneurysms (10%)
Inaccurate blood pressure
(left arm) if previous
subclavian angioplasty
Aortic branch
collaterals
Associated
with bicuspid
aortic valve
(50-80%)
Endothelial
dysfunction
(diffuse
aortopathy)
Postoperative
hypertension
Avoid tachycardia,
Hypotension
Aortic Stenosis
Anatomy Physiology
Potential Issues
Specific Anesthetic
Management
LV pressure
overload and
Hypertrophy
Unrepaired:
• Pulmonary edema
• PHT
• Myocardial ischemia
• Syncope
• Post stenotic dilatation
Repaired:
• Aortic regurgitation
• LV diastolic dysfunction
Avoid tachycardia,
hypotension
Avoid factors that increase
myocardial oxygen
Consumption
Tetralogy of Fallot
Anatomy Physiology
Potential Issues
Specific Anesthetic
Management
Pulmonic
stenosis
Unrepaired: Rare, mean age of death 25 yr,
R-L shunt, Cyanosis
Palliated: Blalock-Taussig shunt
• Chronic left ventricular volume overload
• Cyanosis if shunt is too small
• Pulmonary hypertension
Repaired:
• Sinus and AV node dysfunction
• Dysrhythmia: atrial and ventricular
• Ascending aortic aneurysm
• Residual pulmonary regurgitation or
stenosis
• Residual VSD
• Left ventricular dysfunction
• Persistent pulmonary hypertension from
previous shunts
• RV failure from chronic pulmonary
insufficiency
Avoid tachycardia,
hypovolemia, increased
contractility
Maintain pulmonary blood
flow
Maintain sytemic blood
pressure
Detect and manage
dysrhythmias
Manage pacemaker
External pacing available
RV
hypertrophy
Overriding
aortic root
VSD
Cyanosis
Transposition of Great Arteries
Anatomy Physiology
Potential Issues
Specific Anesthetic
Management
PA arises from LV
Unrepaired: Associated with VSD or
ASD or PDA
Repaired: Senning or Mustard
• Atrial dysrhythmia
• Sinus node dysfunction (by age
40, 50% have pacemaker)
• Systemic ventricle dysfunction
• Residual atrial or ventricular level
shunts
Repaired: Arterial switch
• Myocardial ischemia (narrowed,
occluded coronary arteries,
endothelial dysfunction)
• Ascending aortic aneurysm
Maintain pulmonary blood
flow
Manage dysrhythmia
Manage heart failure
Detailed preoperative
evaluation, both functional
and coronary imaging
study
Aorta arises from
RV
Possible
associated
lesions:
VSD, ASD, PDA,
Pulmonary
stenosis,
coarctation of the
aorta
Abnormal
coronary
artery anatomy
Univentricular Heart
Anatomy Physiology
Potential Issues
Specific Anesthetic
Management
Double inlet
atrioventricular
Connections
Unrepaired: Rare
• Dysrhythmias
• Congestive heart failure
• Bidirectional shunting
• Cyanosis
• PHT
Repaired: Blalock-Taussig shunt,
Glenn shunt, or Fontan
• Dysrhythmias
• Heart failure
• Hepatic dysfunction
• Thromboemboli
• Restrictive lung disease
Manage dysrhythmias
Maintain pulmonary blood
flow
Manage dysrhythmias
Maintain low pulmonary
vascular resistance
Maintain adequate
preload
Replace coagulation
factors
Absence of one
atrioventricular
Connection
Single welldeveloped
ventricle
Strategy for management of ACHD
patient through non-cardiac surgery
anesthetist
surgeons
cardiologist
Adult Congenital Heart Center
intensivist
Classification of ACHD according to complexity
1- Define the Condition
– Primary lesion
– Previous palliative and corrective surgeries
– Residual and sequelae
2- Assess the Surgical Risk
– Predictors and risks of the proposed surgical
procedure itself
– Specific ACHD risk factors
– Other co-morbid conditions
3- Develop a Management Plan
– Understanding of anatomy and physiology
– Hemodynamic goals
– Perioperative management
Promote Oxygen
Delivery to
tissues
Maintain
balanced Pulm.
& Systemic Blood
Flow
Optimize
Hematocrit
HISTORY & PHYSICAL EXAMINATION
•
Assess functional status
•
Symptoms, signs of right sided failure
•
Symptoms, signs of left sided failure
•
Symptoms of low cardiac output
•
Arrhythmias
•
Cyanosis & its sequelae
chest X – Ray
Laboratory
Evaluation
12 Lead ECG
Preoperative
investigation
Cardiac
Catheterization
Echocardiography
De-airing
• All intravenous lines must be meticulously deaired.
• Patent foramen ovale is present in
approximately 25% of adults
Indications for Prophylaxis against IEC in CHD*
– Unrepaired cyanotic CHD, including palliative shunts and
conduits
– Completely repaired congenital heart defect with prosthetic
material or device, whether placed by surgery or by catheter
intervention, during the first 6 months after the procedure**
– Repaired CHD with residual defects at the site or adjacent to the
site of a prosthetic patch or prosthetic device (which inhibit
endothelialization)
– Previous IE
•
*Except for the conditions listed above, antibiotic prophylaxis is no longer recommended for any other form of CHD.
•
**Prophylaxis is reasonable because endothelialization of prosthetic material occurs within 6 months after the procedure.
Premedication
• Premedication with
anxiolytics and hypnotics
must be undertaken very
cautiously.
Monitoring
Non-invasive
•
•
•
•
ECG
NIBP
Pulse oximetry
End tidal capnogram
Invasive
- Art. catheterization
- CVP
- PAC
- TEE
Choice of anesthetics
• There are no evidence-based
recommendations to guide the anesthetic
management of patients with CHD undergoing
non-cardiac surgery.
Intravenous anesthetics
Barbiturates
Propofol
Benzodiazepines
Ketamine
Etomidate
Inhalational anesthetics
Halothane
Enflurane
Isoflurane
Sevoflurane
Desflurane
Regional anesthesia
Spinal anesthesia
Epidural anesthesia
Fluid management
• Minimizing the NPO interval
• Preoperative hydration
• Maintenance of adequate intravascular volume
• Adjustment of the transfusion threshold.
Positioning
Lateral
Anti-Trendelenberg position
Trendelenburg position
Prone
Positive Pressure Ventilation
• Control pulmonary vascular resistance
• Reduces systemic venous return, in patients
with single ventricle physiology
Postoperative Management
PACU vs. ICU
Major risk factors during the postoperative period:
• Bleeding.
• Dysrhythmias.
• Thromboembolic events.
Postoperative Management
• Post-operative analgesia
• Fluid Management
Thank you