System
Common complications
Cardiac
Right ventricular failure/infarction, congestive heart failure, dysrhythmias (atrial fibrillation, supraventricular tachycardia most common), infective endocarditis, cardiac syncope, sudden death
Respiratory
Hemoptysis, pulmonary hemorrhage, chronic pulmonary emboli, pulmonary infarction, hypoxia
Neurologic
Brain abscess, embolic strokes, intracerebral hemorrhage, TIA
Hematologic
Hyperviscosity syndrome, thrombosis, platelet dysfunction, clotting factor deficiencies (vWF deficiency in particular), hypofibrinogenemia
Hepatobiliary
Hyperbilirubinemia, pigment gallstones
Renal
Chronic renal disease with decreased glomerular filtration rate, hyperuricemia leading to gout, nephrolithiasis
Skeletal
Hypertrophic osteoarthropathy
What is the predicted perioperative mortality for patients with Eisenmenger’s syndrome undergoing noncardiac surgery?
There is a range in the reported literature, but generally perioperative mortality is high. 7–18% in one large study, but higher for emergent procedures. One recent study found a 25–30% perioperative mortality rate for patients with Eisenmenger’s syndrome undergoing major surgery or labor [2].
- 4.
What are the predictors of poor post-operative function in a patient with Eisenmenger’s syndrome?
Low baseline functional status
Clinical signs of heart failure
History of arrhythmia or current arrhythmia
Elevated right atrial pressure
Right ventricular hypertrophy or other repolarization abnormalities on EKG
Complex cardiac anatomy
- 5.
Describe the factors that will increase pulmonary vascular resistance
Hypoxia
Hypercarbia
Metabolic acidosis
Hypothermia
Agitation
Pain
Tracheal suctioning
- 6.
How do you calculate pulmonary vascular resistance (PVR)? What are the normal values? What can you expect PVR to be in this patient?
((mean pulmonary artery pressure − pulmonary capillary wedge pressure)*80)/Cardiac output (L) = PVR in dyne*s*cm−5
Normal values for PVR are approximately <250 dyne*s*cm−5, but can be >800 in patients with Eisenmenger’s syndrome.
- 7.
Discuss the major targeted therapies available for pulmonary hypertension (Table 7.2 )
Table 7.2
Major targeted therapies available for pulmonary hypertension
Medication class
Mechanism of action
Available drugs
Route of administration
Major side effects
Nitric oxide pathway
Inhibit PDE-5 to decrease
c-GMP breakdown
NO increases
c-GMP production to cause pulmonary vasodilation
Sildenafil
Taldalafil
iNO
PO
PO (once daily dosing)
Continuous inhaled
Flushing, headache, hypotension
Prostanoids
Augment endogenous prostacyclin to cause pulmonary vasodilation
Epoprostenol
Treprostinil
Iloprost
Cont IV/Inhaled
Cont SQ/IV, Int inhaled, PO
Int Inhaled
Flushing, headache, diarrhea, cough. Rebound pulmonary hypertensive crisis if stopped suddenly
Endothelin receptor antagonists
Inhibit endothelin-1, a potent pulmonary vasoconstrictor
Bosentan
Ambrisentan
Macitentan
PO
PO
PO
LFT elevations, headache, anemia, contraindicated in pregnancy
- 8.
What is the appropriate preoperative testing for a patient with Eisenmenger’s syndrome?
Recent echocardiogram to assess right ventricular (RV) function
Recent right heart catheterization for measurement of pulmonary and right ventricular pressures (in an emergent situation, estimation of pulmonary artery systolic pressures using the TR jet on echocardiogram would be reasonable instead of catheterization)
Pulmonary function tests, especially for thoracic surgical procedures
Labs including CBC (looking for baseline hematocrit, platelets), CMP (electrolyte abnormalities from diuretics, renal dysfunction, hyperbilirubinemia, hyperuricemia), coagulation studies including fibrinogen (clotting factor deficiencies, hypofibrinogenemia), BNP if this has been serially followed
Electrocardiogram looking for arrhythmias and repolarization abnormalities
Referral to a pulmonary hypertension specialist to optimize the perioperative pulmonary hypertension regimen
Assessment of functional status [1]
- 9.
Discuss the hemodynamic considerations for an anesthetic in a patient with Eisenmenger’s syndrome. What parameters are you particularly concerned about maintaining? What will happen if the patient does have a drop or rise in systemic vascular resistance (SVR) or cardiac output (CO)?
The principle anesthetic goals are to avoid increases in pulmonary vascular resistance and to avoid right ventricular failure. This can be done by maintaining systemic vascular resistance (SVR), avoiding decreases in cardiac output, and avoiding insults that increase PVR (see question #5).
A drop in SVR is dangerous for three reasons:
- 1.
A drop in SVR will increase the R -> L shunt, leading to worsening hypoxemia and subsequent decrease in the oxygen delivery to tissues (particularly the heart and brain).Related posts:
Stay updated, free articles. Join our Telegram channel
Full access? Get Clinical Tree
