A 49-year-old man (body weight: 86 kg, height: 169 cm) who was diagnosed as having hepatocellular carcinoma with liver cirrhosis due to hepatitis B virus (Child-Pugh grade C, MELD score 14) presented for LDLT. His past medical history revealed diabetes mellitus that was well controlled with an oral hypoglycemic agent. The abnormal results of preoperative laboratory testing were as follows: aspartate transminase (AST): 59 U/L, total bilirubin: 3.1 mg/dl, albumin: 2.4 g/dl, platelets: 91,000/ml. The ECG and chest X-ray were within normal limits. Preoperative transthoracic echocardiography (TTE) showed diastolic dysfunction grade 2 with left atrial enlargement. No left ventricular hypertrophy and LVOTO were identified (Fig. 1).

Anesthesia was induced with thiopental sodium 5 mg/kg and vecuronium 0.1 mg/kg and it was maintained with desflurane in air and oxygen. The routine vascular access consisted of a 9 Fr introducer (Advanced Venous Access®) on the right internal jugular vein, a pulmonary artery catheter and two arterial catheters on the radial artery and femoral artery respectively, and a femoral venous catheter. After anesthetic induction, the vital signs were stable and the intraoperative course was unremarkable during the preanhepatic phase (Table 1).

The anhepatic phase began 3 hours 30 minutes after anesthetic induction, and hemodynamic instability abruptly developed. The hemodynamic instability improved with a large volume of fluids and blood products (crystalloid solution 10,500 ml, colloid solution 1,200 ml, Cell Saver autotransfusion 978 ml and leukocyte-depleted red blood cells (LDRBCs) 2 units for 2 hours) and catecholamine infusion (dopamine 5 µg/kg/min and norepinephrine 0.1 µg/kg/min) (Table 1).

Reperfusion began 2 hours 20 minutes after portal vein clamping, and the hemodynamic instability further deteriorated. We administered a large volume of fluids and blood products (crystalloid solution 3,400 ml, Cell Saver autotransfusion 500 ml and fresh frozen plasma (FFP) 2 units in 1 hour) and increased the doses of catecholamines (norepinephrine 0.3 µg/kg/min) (Table 1). Two hours and 30 minutes after reperfusion, the endtidal CO₂ was suddenly decreased and the ST segment of the ECG was elevated; the hemodynamic instability did not improve despite intravascular volume replacement and increased doses of catecholamines (dopamine 10 µg/kg/min, norepinephrine 0.1 µg/kg/min and epinephrine 0.1 µg/kg/min) (Table 1). As there was preoperative diastolic dysfunction, we inserted a TEE to accurately evaluate the cardiac function at 3 hours and 30 minutes after reperfusion. The TEE evaluation revealed signs of dynamic LVOTO, including systolic anterior motion (SAM) and eccentric mild-to-moderate mitral regurgitation. Based on the TEE findings, the epinephrine infusion (0.1 µg/kg/min) was immediately discontinued, esmolol infusion (30 µg/kg/min) was initiated and several 100 µg boluses of phenyleprine and aggressive volume resuscitation were performed (crystalloid solution 1,400 ml, colloid solution 500 ml, Cell Saver autotransfusion 1,071 ml, FFP 2 units for 1 hour). Afterward, the vital signs gradually became stable and the operation ended uneventfully (Table 1). The total infused volume included crystalloid solution: 26,400 ml, colloid solution: 2,650 ml, Cell Saver autotransfusion: 4,071 ml, LDRBCs: 5 units, FFP: 8 units and cryoprecipitate: 9 units. Postoperatively, large volumes of fluids and blood products were necessary to stably maintain the hemodynamics and the decreasing the dose of vasopressors was not possible. On second postoperative day, the TTE revealed the SAM with dynamic LVOTO (Fig. 2A). The color doppler showed mild mitral regurgitation (Fig. 2B) and flow acceleration in the LVOT (Fig. 2C). The patient underwent reoperation for control of the surgical bleeding on postoperative day 3. He was discharged on postoperative day 42.

A 45-year-old man (body weight: 84 kg, height: 180 cm) was diagnosed with liver cirrhosis due to hepatitis C virus (Child-Pugh grade C, MELD score 40) and he presented for LDLT. He had oliguric acute renal failure caused by hepatorenal syndrome, and this required continuous renal replacement therapy. The abnormal results of the preoperative laboratory testing were as follows: AST: 56 U/L, alkaline phosphatase 35 U/L, GGT (Gamma(γ)-Glutamyl Transferase): 20 U/L, total bilirubin: 12.5 mg/dl, albumin: 2.6 g/dl, BUN (blood urea nitrogen): 31.7 mg/dl, creatinine: 3.75 mg/dl, platelets: 35,000/ml and the prothrombin time (INR): 2.71 sodium 129 mEq/L. The other laboratory testing and ECG were within the normal limits. However, the chest X-ray showed pulmonary edema on the bilateral lung fields. The preoperative TTE revealed asymmetric septal hypertrophy (the thickness of the interventricular septum and left ventricular posterior wall was 29 mm and 9 mm, respectively) with LVOTO. The mean pressure gradient of the LVOT was 80 mmHg. The color doppler showed eccentric moderate mitral regurgitation due to SAM. There was also grade 2 diastolic dysfunction. He experienced occasional hypotension (mean arterial pressure: 40 mmHg) accompanied by drowsy mentality, dizziness, blurred vision and chest discomfort caused by aggravation of the LVOTO due to volume depletion.

Anesthesia was induced with etomidate 0.2 mg/kg and atracurium 0.5 mg/kg and it was maintained with desflurane in air and oxygen. A 9 Fr introducer (Advanced Venous Access®) was placed on the right internal jugular vein, and a pulmonary artery catheter and two arterial catheters were placed on the radial artery and femoral artery, respectively, and a femoral venous catheter were placed for routine vascular access. The TEE probe was gently inserted and there was no variceal bleeding. There were no noticeable changes of the LVOTO and SAM compared to the preoperative findings. The vital signs were stable during induction, but the blood pressure began to decrease 20 minutes later. The favorable response to phenylephrine 100 µg bolus was confirmed, and then continuous infusion of norephinephrine was started at 0.05 µg/kg/min and this was adjusted in the range of 0.05-0.2 µg/kg/min along with maintaining the intravascular volume.

After bolus phenylephrine 100 µg and increasing the norephnephrine dose to 0.25 µg/kg/min, the anhepatic phase began and a portocaval shunt was created to mitigate the venous pooling. The patient's intermittent hypotension was treated with intravascular volume expansion and bolus phenylephrine, and the norephinephrine dose was increased to 0.3 µg/kg/min. The infused fluid and blood products were crystalloid solution 1,900 ml, half saline 5,600 ml, colloid solution 2,450 ml, Cell Saver autotransfusion 1,355 ml, LDRBCs 2 units, FFP 2 units and cryoprecipitate 6 units until 1 hour after the anhepatic phase.

Reperfusion began after a 100 µg bolus of phenylephrine. Milder reperfusion syndrome occurred and the patient spontaneously recovered. There were no noticeable changes of the LVOTO and SAM compared to the initial findings after induction. The rest of the operation was relatively uneventful with maintaining the intravascular volumeand continuous infusion of norepinephrine at 0.4-0.6 µg/kg/min. The total administered volume included crystalloid solution 7,700 ml, half saline 7,300 ml, colloid solution 2,970 ml, 5% dextrose water 200 ml, Cell Saver autotransfusion 3,701 ml, LDRBCs 5 units, FFP 6 units, leukocyte-depleted platelet concentrates 6 units and cryoprecipitate 6 units for 11 hours 30 minutes. On postoperative day 1, the TTE revealed moderate mitral regurgitation and dynamic LVOTO with SAM. An emergency operation was performed on postoperative day 2 due to persistent bleeding. The patient's postoperative course was complicated by septic shock due to the cellulitis of both thighs. Doppler ultrasonography showed sluggish flow of the hepatic vein, portal vein and hepatic artery. Eventually, the patient expired on postoperative day 69.