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Pathophysiology
Prognosis
History
Cardiovascular findings
Other findings
Lab investigations
Iron studies
Reduced resting PaCO2 due to resting tachypnea and reduced PaO2 due to right-toleft shunting
Mixed respiratory and metabolic acidosis
Chest radiograph
Electrocardiogram
Echocardiogram
Transthoracic echocardiogram
The structural cardiac defect responsible for the shunt can be
defined by the 2-dimensional imaging.
The location of cardiac shunt can be demonstrated by color
Doppler or venous agitated saline contrast imaging.
The pressure gradient across the defect can be estimated.
Estimated pulmonary artery systolic and diastolic pressures
Identification of coexistent structural abnormalities
Left and right ventricular size and function
Identification of surgical systemic-to-pulmonary shunts
The addition of supine bicycle ergometry can demonstrate
increased right-to-left shunting with exercise.
Transesophageal echocardiogram is useful for imaging posterior
structures, including the atria and pulmonary veins.
Cardiac catheterization
Severity of pulmonary vascular hypertension
Conduit patency and pressure gradient
Coexisting coronary artery anomalies (rare)
Degree of shunting
Medical Treatment
Fluid balance and climate control
Avoid sudden fluid shifts or dehydration, which may increase right-toleft shunting.
Avoid very hot or humid conditions, which may exacerbate
vasodilatation, causing syncope and increased right-to-left shunting.
Oxygen supplementation
Use is controversial
Oxygen therapy has been shown to have no impact on exercise
capacity and survival in adult patients with Eisenmenger syndrome
Sandoval etal Am J Respir Crit Care Med. 2001 Nov 1;164(9):1682-7
Medical Treatment
Medical Treatment
Pulmonary vasodilator therapy
Long-term prostacyclin therapy- Improvement in haemodynamics,
suturation & 6 minute walk test. Rosenzweig etal, Circulation 1999 Apr 13; 99(14): 1858-65
Fernandes etal Am J Cardiol 2003 Mar 1; 91(5): 632-5
Bosentan, an endothelin receptor antagonist Christensen,Am J Cardiol 2004 Jul 15; 94(2): 261-3
Schulze-Neick et al Am Heart J 2005 Oct; 150(4): 716
Surgical options
Heart lung transplant
Procedure of choice if repair of the underlying cardiac defect is not
possible.
Performed successfully for the first time in 1981.
Reported actuarial survival rates are 68% at 1 year, 43% at 5 years,
and 23% at 10 years.
The main complications are infection, rejection, and obliterative
bronchiolitis
Bilateral lung transplantation
Preferable procedure if the cardiac defect is simple (e.g.- ASD)
Repair of the underlying cardiac defect is required
Better than single-lung transplantation in terms of mortality, New
York Heart Association functional class, cardiac output, and
postoperative pulmonary edema.
Advantages over heart-lung grafting include no transplant coronary
artery disease or cardiac rejection.
Activity
Intense athletic activities carry the risk of sudden death.
Exercise prescription can be individualized based on
exercise testing that documents a level of activity that
meets the following 3 criteria:
Oxygen saturations remain greater than 80%.
No symptomatic arrhythmias.
No evidence of symptomatic ventricular dysfunction
Diet
Excessive sodium intake to be avoided
Anaesthetic
considerations
Preoperative assessment
Goals
Prevent further increase in Rt to Lt shunt
Maintain CO
Prevent arrhythmias
Avoid hypovolemia, PVR, SVR
Marked increase in SVR should also be
avoided as excessive systemic vasoconstriction
can precipitate acute LVH
What To Do?
General anaesthesia
Induction with high dose opioid (short acting) technique or
with ketamine, etomidate or low dose thiopentone
Cardiostable inhalational agent- isoflurane, sevoflurane,
xenon. Hofland J Br J Anaesth. 2001 Jun;86(6):882-6.
Muscle relaxation with atracurium, vecuronium
TIVA with propofol, remifentanil. Kopka A, Acta Anaesthesiol Scand. 2004 Jul;48(6):782-6
Some patient may not tolerate positive pressure ventilation
and PEEP well
Anaesthetic technique
100 % oxygen
Nitric oxide- 5 -20 ppm. Bouch DC etal, Anaesthesia. 2006 Oct;61(10):996-1000
Prostacycline- infusion or nebulization
Postoperative care
Eisenmenger and
pregnancy
In O.T.
G.A
Post op monitoring
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Thank
you!!