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A 30-year-old female with an unrepaired patent ductus arteriosus and Eisenmenger syndrome presents for laparoscopic ovarian cystectomy.

  • Electrocardiogram: Normal sinus rhythm at 75 bpm, right ventricular hypertrophy (RVH).

  • Echocardiogram: Moderately decreased right ventricular function, moderate RVH, bowing interventricular septum.

  • Physical examination: 65-kg female with 92% O2 saturation on room air.


Eisenmenger syndrome occurs when a congenital cardiac defect with left-to-right shunting reverses direction as a result of marked elevations in pulmonary vascular resistance to a level equal to or exceeding systemic vascular resistance. The shunt becomes either bidirectional or right to left. Exposure of the pulmonary vasculature to increased blood flow and pressure, as seen frequently in intracardiac shunts, results in pulmonary obstructive disease. In the early stage, this is confined to the arteriolar musculature and is still amenable to pharmacological treatment or surgical correction. However, if the cardiac defect remains uncorrected, the pulmonary vasculature changes will become fixed, and surgical correction at this point carries a very high mortality rate. Shunt reversal occurs in 50% of unrepaired ventricular septal defects and 10% of unrepaired atrial septal defects.

Signs and symptoms include cyanosis, decreased exercise tolerance, palpitations from atrial fibrillation or atrial flutter, and arterial hypoxemia leading to erythrocytosis and polycythemia with visual disturb-ances, headache, dizziness, and paresthesias. Patients can present with hemoptysis from pulmonary infarction or rupture of dilated pulmonary vessels. There is an increased risk of cerebral vascular accidents or brain abscesses from paradoxical emboli. Both coagulopathy and thrombotic events can occur. The cardiac output becomes fixed as a result of fixed elevated pulmonary vascular resistance, and patients become preload dependent to maintain cardiac output. Syncopal episodes can occur when cardiac output is inadequate, and sudden death is possible.

Treatment with intravenous epoprostenol may be beneficial, but no treatment has been proven effective. Correction of the cardiac defect with concurrent lung transplantation or combined lung-heart transplantation is possible only for a select few. Symptoms of hyperviscosity may be relieved with phlebotomy with isovolemic replacement.

Patients with fixed pulmonary vascular resistance, by definition, will not respond to pharmacologic intervention, but some patients’ pulmonary circulation may retain some degree of reactivity to pulmonary vasodilators such as nitric oxide or inhaled prostacyclin.


  • Maintain the preoperative level of systemic vascular resistance and avoid vasodilating drugs that can increase right-to-left shunting. Norepinephrine infusion can help maintain systemic vascular resistance. Epinephrine and vasopressin infusions may also be used.

  • Maintain preload because cardiac output is fixed. If the patient is taking nothing by mouth for extended periods of time prior to the procedure, consider giving a fluid bolus before anesthetic induction.

  • Remove all bubbles in IV lines.

  • Consider prophylactic phlebotomy with isovolemic replacement if hematocrit is higher than 60%.

  • Consider preinduction arterial monitoring and IV induction.

  • Have nitric oxide in the operating ...

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