The patient was a 25-year-old obese man (height, 159.8 cm; body weight, 92.5 kg). At birth, he was diagnosed with double-outlet right ventricle, pulmonary atresia, and atrioventricular septal defect after cyanosis and heart murmur were evident. He underwent a right-modified Blalock-Taussig shunt at 34 days of age to increase the pulmonary blood flow. Cardiac catheterization indicated that the left ventricle was hypoplastic (left ventricular end-diastolic volume, 58% of normal), and pediatric cardiologists clarified the impossibility of biventricular repair. Therefore, he underwent the Glenn procedure at the age of 1 year and a fenestrated total cavopulmonary connection at the age of 3 years.
In this patient, the systemic ventricle was a morphologic right ventricle with a low ejection fraction, and the central venous pressure (CVP) was high at 16 mmHg; therefore, he was at a greater risk of hemodynamic instability.
At the age of 20 years, abdominal ultrasonography detected a large (>6 cm) tumor in segment 6 of the liver. Surgical treatment in Fontan-palliated patients is deemed unpredictable; therefore, he was treated non-surgically. However, at 25 years, protein induced by vitamin K antagonist-II level increased to 336 mAU/ml (cutoff value, 40 mAU/ml). Hepatocellular carcinoma was suspected; therefore, he was admitted to our hospital for surgical liver resection. On admission, the Child-Pugh class was determined as A. Blood hemoglobin was 17.3 mg/dl, platelet count was 21.3 × 104/μl, and prothrombin time was 82.3%. The liver function was well preserved. Preoperative echocardiography revealed that the right-ventricular ejection fraction was 34% with a limited atrioventricular valvular regurgitation; CVP was 16 mmHg, saturation of peripheral oxygen (SpO2) was 94% on room air at rest, and PVR was low (1.0 WUm2).
It is difficult to determine whether laparoscopic surgery or open laparotomy procedure is more advantageous for a patient with Fontan physiology. Considerable preoperative discussions were conducted among the medical team members. In patients with Fontan physiology, adequate intravascular volume (preload) and low PVR should be maintained during the surgical procedure. During laparoscopic surgery, hypercarbia due to CO2 insufflation and high airway pressure caused by obesity could increase PVR. However, postoperative pain after open surgery could also increase PVR; therefore, we selected the less-invasive laparoscopic surgery for this Fontan-palliated patient. During pneumoperitoneum, this patient was scheduled to inhale nitric oxide, which dilates the pulmonary blood vessels selectively. Moreover, CO was measured continuously by transpulmonary thermodilution, such as a pulse contour cardiac output catheter (PiCCO; PULSION Medical Systems, Munich, Germany), in addition to transesophageal echography.
We planned to maintain low abdominal air pressure during pneumoperitoneum and to shift promptly to open abdominal surgery if hemodynamic instability became apparent because of the accumulated partial pressure of carbon dioxide (PaCO2) or postural change.
In the operating room, the patient’s electrocardiogram, SpO2, and noninvasive blood pressure (NIBP) were monitored. At baseline, NIBP was 132/88 mmHg, heart rate was 91/min, and SpO2 was 93% under room air conditions. After insertion of epidural catheter, general anesthesia was induced by propofol (90 mg), remifentanil (0.2 μg/kg/min), and rocuronium (100 mg). After orotracheal intubation, anesthesia was maintained by desflurane inhalation and fentanyl and remifentanil infusion. He received volume-controlled ventilation with fraction of inspired oxygen (FIO2) of 0.7, positive end-expiratory pressure of 6 cmH2O, peak inspiratory pressure of less than or equal to 22 cmH2O, tidal volume of 450 ml, and respiratory rate of 15–18 breaths/min. Minute ventilation was adjusted to maintain end-tidal CO2 (EtCO2) at 30–35 mmHg.
With ultrasound guidance, CO and stroke volume variant were monitored via the PiCCO catheter. Then, a central venous catheter (PreSep CatheterTM; Edwards Lifesciences, Irvine, CA, USA) was placed via the right internal jugular vein to monitor CVP and central venous saturation (ScvO2) continuously. The tip of the central venous catheter was located just cephalad to the connection between the superior vena cava and the right pulmonary artery. Figure 1 shows the trend of the vital signs, including CO, cardiac index (CI), CVP, ScvO2, and EtCO2 during anesthesia. Time 0 was defined as the start of general anesthesia. At baseline, FIO2 was 0.7, CI was 1.75 l/min/m2, CVP was 20 mmHg, and ScvO2 was 74%.
After initiating pneumoperitoneum, arterial blood pressure (ABP) increased slightly, but CO and ScvO2 were invariant. After postural change to the reverse Trendelenburg position, CVP decreased from 17 to 13 mmHg, but CO and ScvO2 remained unchanged. The insufflation pressure was maintained at 8–10 cmH2O. We administered an appropriate muscle relaxant for optimum surgical conditions. During pneumoperitoneum, the patient inhaled nitric oxide to decrease PVR. The Pringle maneuver during the liver resection did not affect CVP adversely. After withdrawal of the reverse Trendelenburg position, the CVP increased rapidly from 14 to 24 mmHg, but decreased subsequently to 20 mmHg. Nevertheless, CO and ScvO2 remained stable. CI and CO were stable at low levels, and laparoscopic partial hepatectomy was concluded successfully with hemodynamic stability. Transesophageal echography revealed no remarkable changes. Blood loss and urine output were 295 ml and 158 ml, respectively, which were replaced with 2200 ml of crystalloid.
After reversal of neuromuscular blockade, the patient was extubated and admitted to the intensive care unit. Postoperative analgesia using levobupivacaine and fentanyl was successfully administered via an epidural catheter. He remained hemodynamically stable and was shifted to the general ward on the 1st postoperative day. He was discharged on the 8th postoperative day with no postoperative complications. The resected liver mass was pathologically diagnosed as focal nodular hyperplasia with no malignancy.