2. Overview
Common congenital heart diseases seen in Adults
Long term consequences of congenital heart diseases and effect on Anesthesia management
i. Pulmonary Hypertension
ii. Bleeding and thrombosis risk
iii. Heart failure
iv. Dysrhythmias
Anaesthetic management
i. Preoperative evaluation
ii. Pre-medication
iii. Endocarditis Prophylaxis
iv. Intraoperative monitoring
v. Management strategies for specific defects
vi. Post operative management
3.
4. Cardiac Sequelae
Pulmonary hypertension
Ventricular dysfunction
Dysrhythmias and conduction
defects
Residual shunts
Valvular lesions-regurgitation or
stenosis
Hypertension
Aneurysms
Non-cardiac sequelae
Secondary erythrocytosis
Cholelithiasis
Nephrolithiasis
Developmental abnormalities
Seizure disorders from previous
thromboembolic events or
cerebrovascular accidents
Restrictive and obstructive lung
disease
Long term consequences of CHD
5. Pulmonary Hypertension (main etiology)
Presence of long standing
large and non-restrictive
defects
Increased pulmonary blood
flow and pressure to near
systemic levels
Irreversible vascular changes
– hypertrophy of the media,
cellular proliferation in intima,
smooth muscle cell migration
to sub endothelium,
progressive fibrosis
Obliteration of arterioles and
small arteries
Increased pulmonary
resistance and reversal of
shunt
EISENMENGER SYNDROME
6. Pulmonary Hypertension
Other causes of pulmonary venous hypertension-
a. Secondary to elevated ventricular end diastolic pressure
b. Elevated pulmonary venous atrial pressure
c. Pulmonary vein stenosis
8. Eisenmenger Syndrome
Refers to the development of pulmonary hypertension secondary to long standing left to right shunting.
Non cardiac surgery should only be performed if absolutely essential.
Perioperative mortality increases
Predictors of mortality-
Syncope
Age at presentation or development of symptoms
Poor functional class
Supraventricular dysrhythmias
Elevated right atrial pressures
Low oxygen saturation (<85%>
Renal insufficiency
Severe right ventricular dysfunction
Trisomy 21
9. Anaesthetic management in patients with
pulmonary hypertension
Minimize increase in pulmonary vascular resistance and decrease in
systemic vascular resistance.
Abrupt increase in PVR precipitate acute right ventricular failure
Decreased cardiac output and
oxygen desaturation
Bradycardia and
cardiac arrest
10.
11. Prevention
Hyperventilation (with fraction
of inspired oxygen 1.0)
Correction of acidosis
Avoidance of sympathetic
nervous system stimulation
Maintenance of normothermia
Minimization of intrathoracic
pressure
Use of inotropic support
Inhaled nitric oxide
Regional versus general
anaesthesia
Regional may produce
unacceptable decreases in SVR
and could exacerbate Right to
left shunt.
General anaesthesia allows for
optimal control of ventilation
and may be preferable in
patients with high risk surgery.
12. Bleeding and thrombosis risk
cyanosis
Chronic
hypoxia
Increased
erythropoietin
Increased red
cell mass
Increased
viscosity
13. Bleeding and thrombosis risk
Viscosity Sheer Stress Prostaglandins &
Nitric Oxide
Tissue
vascularity
and arteriolar
dilatation
BLEEDING
Flow in small
arterioles(exacerbated by iron
deficiency and dehydration)
THROMBOSIS
14. Bleeding and Thrombosis risk
PREVENTION-
Adequate hydration
Pre-operative phlebotomy if HCT>65%
Consider replacement of coagulation factors and platelets
Iron deficiency should be corrected pre-operatively
15. Heart failure
Adults with
corrected CHD
Increses ANP,
Renin, Aldosterone
, NE
Abnormal cardiac
autonomic nervous
system regulation
Altered
Hemodynamics
Heart Failure
16. Left sided failure
Should be Optimized in peri-
operative period with
Diuretics
Digoxin
ACE inhibitors
Beta blockers
Right sided failure
No evidence based guidelines
for management of heart failure
in patients with a systemic right
ventricle (congenitally corrected
TGA, Mustard or Senning repairs
of TGA and single ventricles)
Heart Failure
17. Dysrhythmias
CAUSE- Primary consequence of underlying congenital defect or secondary to surgical repair
MOST COMMON FORM- Intraatrial reentrant tachycardia originating from right atrium.
ATRIAL TACHYARRHYTHMIAS- often resistant to pharmacological treatment and results in rapid
hemodynamic deterioration.
VENTRICULAR DYSRHYTHMIAS- risk factors-
Decreased right or left ventricular function
Previous ventriculotomy
Older age at initial surgery
Patients who are repaired late exposed to longer periods of cyanosis, volume overload,
pressure overload Increased myocardial fibrosis and associated slowing of conduction
Increased risk of Arrhythmias
Acute hypoxemia can provoke ventricular dysrhythmias as subendocardial myocardial perfusion
is already impaired in hypertrophied myocardium
18. Pre-operative Evaluation
Multidisciplinary APPROACH- anesthesiologists, cardiologists,
intensivists, and surgeons
Look for echocardiogram and cardiac catheterisation to become
familiar with patient’s anatomy and physiology ANTICIPATE
intraoperative events
ASSESS perioperative risk- Increased with
Poor functional class
Pulmonary HTN
CHF
Cyanosis
Major surgery ( OLV,
changes in position-
prone,trendelenberg)
19. Pre-medication
Psychological preparation is important
ANXIOLYTICS and HYPNOTICS
Hypoventilation and
Hypercapnia
Cautious!!
Increased
Pulmonary Vascular
Resistance
( particularly in
patients with
underlying
pulmonary HTN or
Systemic to
Pulmonary shunts)
20. Endocarditis Prophylaxis
AHA has recently published updated guidelines for the prevention of IE.
Only patients with cardiac conditions associated with the highest risk of
adverse outcomes should continue antibiotic prophylaxis before surgery-
Previous endocarditis
Unrepaired CCHD
Completely repaired congenital heart defects during the first 6 months
after the procedure
Repaired CHD with residual defects at the site (which inhibit
endothelialisation)
22. Signify increase in Pulmonary
vascular resistance
Increase Right to left shunt
Decrease pulmonary blood flow
through shunts
Increase in left to right shunt
may not be detected by pulse
oximetry and oxygen saturation
may be maintained even if
systemic cardiac output is
severely compromised.
Pulse oximetry
23. • EtCO2 underestimates PaCO2 in the case of Right to
Left shunting
• Knowledge of the Anatomy and Physiology of
specific palliative repairs is important for
choosing appropriate monitoring
• Congenital defects associated with inadequate PBF
are palliated with systemic to pulmonary shunts
Intraoperative monitoring
24. Classic Blalock Taussig
shunt
• End to side anastomosis of
the subclavian and
pulmonary arteries
• Significance- Arterial
pressure and SpO2 cannot
be measured on the
ipsilateral side
26. Fontan shunt
• Total cavopulmonary
connection
• Pulmonary and systemic
circulations are totally
separated by diverting all
the systemic venous return
to the PA, usu. Without
interposition of a sub-
pulmonic ventricle.
27. Monitoring
Alterations in intracardiac anatomy complicate the placement of central
venous catheters in palliated adults
CVP values interpretation will be different.
Eg. In Fontan; CVP ~ mean PAP
In Intraatrial Buffle(Mustard or Senning procedure) ; PAC placement is
difficult or impossible.
Vascular access --- may be difficult --- previous catheterization
Invasive arterial pressure monitoring – Essential – sensitive to sudden
changes in preload, SVR and PVR
TEE – might be useful – monitor intravascular volume status and
ventricular function
28. Major objective- Promote tissue O2 delivery
Prevent arterial
desaturation
Maintain a
balance
between
pulmonary and
systemic flows
Optimize HCT
33. Management strategies for specific defects
UNIVENTRICULAR HEART- eg. Fontan – bypasses right ventricle, leaving passive non pulsatile flow from
both SVC and IVC to PA
• Any factor that increases PVR – decreases PBF – Arterial desaturation.
• Fontan patients frequently present with complications – supraventricular dysrhythmias, restrictive lung
disease, thromboembolic complications and hepatic dysfunction.
• Both procoagulant and anticoagulant effects are observed with Fontan as a result of liver dysfunction,
factor loss in patients with protein losing enteropathy – Increased Risk of Bleeding
• Patients with a Fontan circulation should maintain an arterial saturation above 90%. If <90%, further
evaluation for venovenous collaterals, AVM or residual shunt.
34. Anesthetic agent of choice
• Maintain hemodynamic stability
• PreferredETOMIDATE
• Increase PVR, maintain SVR and ventricular performance (not used in adults)
• Beneficial effects seen in children undergoing sevoflurane anesthesiaKETAMINE
• Most depress myocardial contractility and decrease SVR– Adverse effect on
tissue oxygen delivery during induction of anesthesia
• Choice of a specific volatile or IV agent should be based on patient’s
physiology and overall goal of balancing pulmonary and systemic blood flow
OTHERS
35. Specific intraoperative considerations with
shunts
All IV lines must be meticulously DEAIRED to decrease the risk of systemic air
embolization
Hemodynamic effects of ventilation strategies, positioning, pharmacological
agents, and blood loss must all appropriately BALANCE pulmonary and systemic
blood flow.
Ventilation with High Airway Pressure can COMPROMISE venous return, increase
PVR and exacerbate Right to Left shunt.
Inadequate anesthesia and sympathetic nervous system STIMULATION might
increase SVR and exacerbate Left to Right shunting, reducing systemic cardiac
output.
TRENDELENBURG position– Increase CVP– Cerebral hypoperfusion in a patient
with Glenn or Fontan.
Systemic HYPOTENSION– decrease PBF– Arterial Desaturation, in a patient with a
systemic to pulmonary artery shunt.
36. Post op management
Managed in ICU experienced with caring for adults with CHD
Major RISKS include :-
Bleeding
Dysrhythmias
Thromboembolic events
In case of pulmonary HTN, oral PULMONARY VASODILATORS such as
sildenafil and inhaled nitric oxide may be beneficial.
