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Case Reports

Anesthesia Management of Orthotopic Liver Transplantation in a Patient with Mustard Repair of Transposition of Great Arteries and Superior Vena Caval Obstruction

Boucek, Charles M.D.*; Krishnamurthy, Prema M.D.†; Marsh, James Wallis M.D.‡; Lee, Jennifer M.D.§

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AS an increasing number of patients with surgically repaired congenital heart disease survive into adulthood, more of these patients may require complex, lifesaving surgical procedures. We report our experience with providing anesthesia for liver transplantation in a patient with surgically corrected D-transposition of the great arteries.
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Case Report

Our patient was born with D-transposition of the great arteries and underwent Mustard repair in infancy. Transfusion resulted in hepatitis B and subsequently progressive liver failure. By 29 yr of age, he was experiencing fatigue, encephalopathy, and refractory ascites and was referred to our center for liver transplantation. Aggressive diuretic therapy was limited by hyponatremia. His exercise tolerance was limited by fatigue after the climbing of one flight of stairs. Evaluation for possible liver transplantation included a cardiology consultation. Radionuclide angiography demonstrated normal biventricular function with a systemic ejection fraction of 51%. An echocardiogram was reported to show normal biventricular size and mild tricuspid regurgitation. The atrial baffle could not be clearly visualized. Cardiac catheterization was not recommended. After discussion of risks and alternatives, the patient was listed for transplantation (Child-Pugh score B). He returned to our medical center when an appropriate matching liver was available.
The patient had an allergy to iodinated contrast. Medications included lactulose, spironolactone, furosemide, carbamazepine, and levofloxacin. His weight was 66.7 kg, brachial blood pressure was 100/45 mmHg, and pulse was 80 beats per min. A grade 3/6 systolic murmur was noted over the left chest, and rhonchi were noted in both lung bases. Radial pulses were full. Femoral sites were scarred, and femoral pulses were not palpable. Serum sodium was 122 mEq/l, potassium 5.2 mEq/l, and hematocrit 26%.
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General anesthesia was induced with fentanyl, midazolam, and thiopental. Tracheal intubation was facilitated by rocuronium. Anesthesia was maintained with lorazepam, fentanyl, and low concentrations of isoflurane in oxygen/air. Vascular access included bilateral radial arterial catheters (Radial Artery Catheterization Set, REF product No. RA-04020, Arrow International, Reading, PA) and venous cannulae (MAC two-lumen venous access kit, product No. AK-11142 9 fr; Arrow International) in the right internal jugular and right subclavian veins. Attempts at placement of a pulmonary artery catheter were unsuccessful, but a single-lumen infusion catheter, REF product No. SC-14701, Arrow International) was advanced via the internal jugular vein and displayed a waveform with a, v, and c waves consistent with central venous pressure. Transesophageal echocardiography (TEE) demonstrated right and left atrial enlargement with an interatrial baffle, moderate mitral and tricuspid regurgitation, and biventricular systolic function (fig. 1). The right ventricle was hypertrophied. Saline contrast echocardiography confirmed the transit of systemic venous blood from the caval system to the left atrium, ventricle, and, ultimately, the pulmonary artery via the atrial baffle (fig. 2).
Removal of the native liver required 6 h and was performed by use of the piggyback technique. 1 Oxygen saturation remained between 98% and 100% throughout the operation, with Paco2 between 86 and 320 mmHg. Central venous pressure measured in the right internal jugular vein ranged from 12–23 mmHg but did not correlate with left ventricular filling viewed on four-chamber or transgastric short-axis views of the TEE. Packed erythrocytes, albumin, and crystalloid were administered to maintain ventricular filling as viewed by TEE. Transient systolic hypotension to 80 mmHg was treated with infusions of dopamine, epinephrine, norepinephrine, and vasopressin. Hydrocortisone and methylene blue were administered as boluses. Arterial blood gases, sodium, potassium, glucose, and hematocrit were measured hourly. Arterial pH remained above 7.28. Metabolic acidosis was treated with Tris(hydroxymethyl)aminomethane (Abbott Laboratories, Abbott Park, IL) and sodium bicarbonate to prevent rapid changes in serum sodium and osmolarity. Furosemide and insulin were given to reduce serum potassium in anticipation of graft reperfusion. Serum potassium declined from 5.2 mEq/l to 3.8 mEq/l immediately before reperfusion. Thirty seconds after reperfusion, it increased to 5.3 mEq/l. Reperfusion of the donor liver was accompanied by a brief episode of sinus bradycardia that responded to a small bolus of epinephrine. Hepatic arterial and duct-to-duct biliary reconstruction proceeded uneventfully. Four units of erythrocytes, 2 units of plasma, and 6 units of platelets were infused, resulting in a final hematocrit of 31%. Blood component therapy 2 was guided by serial thromboelastography (Hemoscope Corporation, Skokie, IL). At the end of surgery, the TEE probe was removed. Norepinephrine was discontinued in the early postoperative period, and the trachea was extubated on the second postoperative day. Lack of TEE made assessment of the adequacy of ventricular filling difficult in the intensive care unit. A pulmonary artery catheter was placed under fluoroscopic guidance via the right femoral vein. Mean pressure in the superior vena cava was 28 mmHg; the atrial pressure was 25 mmHg at end expiration and 0–5 mmHg on inspiration. Pulmonary artery systolic pressure was 45–50 mmHg. Computer tomography was obtained to evaluate the biliary system. This demonstrated that the superior vena cava was interrupted. Collateral flow through the azygous system carried blood to the atrial baffle and anatomic left ventricle. The patient subsequently returned to the operating room for a revision of the biliary anastomosis and was eventually discharged to home in stable condition.
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This patient with complex congenital heart disease developed cirrhosis secondary to transfusion-related viral hepatitis. Liver transplantation is a potentially lifesaving operation for patients with end-stage liver disease, but it is a complex procedure, made more so by residual cardiovascular abnormalities.
In patients with transposition of the great arteries, the right ventricle supplies blood to the aorta. The left ventricle supplies blood to the pulmonary artery. The systemic and pulmonary circuits coexist in parallel rather than intersecting pathways. Survival after delivery depends on intracardiac mixing of blood through atrial or ventricular septal defects or a patent ductus arteriosus. A variety of surgical techniques have been developed for the correction of this condition, all of which cross the pulmonary and systemic circulation at the level of the atria, the ventricles, or the great arteries themselves. The Mustard and Senning operations 3 are atrial repairs. The Rastelli procedure 4 is a ventricular repair using a ventricular septal defect and a valved conduit from the right ventricle to the aorta. The arterial switch procedure reimplants the aorta and pulmonary artery. Atrial repairs such as the Mustard operation retain the right ventricle as the systemic ventricle. With the Mustard operation, 5 the atrial septum is excised, creating a common atrium. An atrial baffle is created with pericardium. The baffle isolates blood returning from the venae cavae and redirects it through the atrium and into the left ventricle. Oxygenated blood returning from the pulmonary veins passes into the atrium on the other side of the baffle. The oxygenated blood then passes through the tricuspid valve into the right ventricle and from there into the aorta. The systemic circulation remains dependent on the right ventricle. Survival into adulthood is common after a successful repair, but patients continue to be at risk for premature death, heart failure, and dysrhythmias. 6 Conduction defects and attenuated augmentation of cardiac output with exercise because of flow restriction through the atrial baffle 7 are not uncommon in these patients. We are unaware of any previous reports of liver transplantation in patients with this condition.
Common problems encountered during liver transplantation include massive blood loss, vasodilatation, progressive acidosis, and coagulopathy. Because our patient’s femoral pulses were not palpable, a radial artery catheter was placed for blood pressure monitoring; a second radial arterial catheter was placed to permit frequent arterial sampling. Venous access for rapid infusion was established, but fluid management was complicated by the limitations of monitoring. It was anticipated that measurement of central venous pressure would reflect preload. The anesthesia team anticipated that navigating a pulmonary artery catheter through the atrial baffle would be difficult, although not necessarily impossible; pacemaker placement via this route has been performed in other patients. 8 Attempts at placement of a pulmonary artery catheter through the jugular and subclavian cannulae failed. Fluoroscopy was not used but would not have resulted in successful placement. Obstruction at the level of the superior vena cava, a recognized complication of the Mustard operation, was present but was not adequately appreciated preoperatively in our patient. The report of his cardiac catheterization, which had been performed 20 yr previously, was not available at the time of the operation. Subsequent computed tomography clarified the residual anatomy and confirmed that the superior vena cava was interrupted. Preoperative placement of a pulmonary artery catheter through the inferior vena cava was not attempted because liver transplantation requires surgical manipulation of both the hepatic vein and the inferior vena cava. A catheter in this location would have been at risk for surgical entrapment during the operation. A pulmonary artery catheter placed from the femoral route postoperatively demonstrated that the mean superior vena cava pressure exceeded atrial pressure.
Obstruction of the superior vena cava complicated the anesthetic management, not only by preventing the intraoperative use of a pulmonary artery catheter but also by making the central venous pressure unreliable, and it limited the options for venous return during removal of the native liver. In the absence of reliable conventional monitors of preload, observation of right and left ventricular filling by TEE was especially helpful. Examination by TEE before attempts at pulmonary artery catheter placement might have been useful in this case. Other commercially available methods to measure cardiac output, such as bioimpedance and Doppler interrogation of the aorta from the esophagus, may also be considered.
During liver transplantation, venovenous bypass 9,10 is sometimes used to decompress the splanchnic and systemic venous systems during native liver removal and graft insertion. Drainage cannulae are placed in the portal vein and inferior vena cava, and a pump returns blood to the superior vena cava through a cannula in an axillary or jugular vein. This preserves venous return during complete clamping of the inferior vena cava. Alternatively, the piggyback technique permits continuous flow through the inferior vena cava, which is only partially clamped. Venovenous bypass can be cumbersome, requires additional cannulations, and is associated with complications such as air embolism and injury to the brachial plexus and vessels. Had it been used in our patient, venous return might have exceeded the capacity of the azygous collaterals that, in our patient, carried blood from the superior vena cava to the atrium. Fortuitously, blood loss was relatively small. Both the subclavian and jugular venous lines led to the superior vena cava and dilated azygous collaterals. Rapid replacement of lost blood may have been limited by the capacity of this system.
We administered methylene blue, norepinephrine, and vasopressin 11 to help maintain systemic vascular resistance and contractility. Methylene blue may reduce hypotension and inotropic requirements during liver transplantation. 12 These agents, used in combination, maintained systemic arterial blood pressure without significant tachycardia during periods of relative hypovolemia.
Candidacy for noncardiac organ transplantation need not be denied to well-compensated patients who have undergone repair of congenital heart defects. Careful preoperative evaluation is especially important in these patients. TEE can be extremely helpful intraoperatively.
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