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

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.

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]

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: