Eisenmenger Syndrome

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
aAdapted from [1], Copyright 2014 Springer Science+Business Media
 
  • 3.
    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].
      1. 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
         
      2. 5.
        Describe the factors that will increase pulmonary vascular resistance
        • Hypoxia
        • Hypercarbia
        • Metabolic acidosis
        • Hypothermia
        • Agitation
        • Pain
        • Tracheal suctioning
         
      3. 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.
      1. 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
        Reference [3, 6]
         
      2. 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]
         
      3. 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. 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).

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    1. Oct 9, 2017 | Posted by in Uncategorized | Comments Off on Eisenmenger Syndrome

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