Successful Hepatectomy Using Venovenous Bypass in a Patient With Carcinoid Heart Disease and Severe Tricuspid Regurgitation

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Successful Hepatectomy in a Patient with Carcinoid Heart Disease with Severe Tricuspid Regurgitation Using Venovenous Bypass: A Case Report Aphichat Suphathamwit MD, Achal Dhir MD, FRCA (UK), FRCPC, Wojciech Dobkowski MD, FRCPC, Douglas Quan MD, MSc, FRCSC, FACS

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S1053-0770(15)00345-6 http://dx.doi.org/10.1053/j.jvca.2015.05.061 YJCAN3292

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Journal of Cardiothoracic and Vascular Anesthesia

Cite this article as: Aphichat Suphathamwit MD, Achal Dhir MD, FRCA (UK), FRCPC, Wojciech Dobkowski MD, FRCPC, Douglas Quan MD, MSc, FRCSC, FACS, Successful Hepatectomy in a Patient with Carcinoid Heart Disease with Severe Tricuspid Regurgitation Using Venovenous Bypass: A Case Report, Journal of Cardiothoracic and Vascular Anesthesia, http://dx.doi.org/10.1053/j.jvca.2015.05.061 This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting galley proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.

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Successful Hepatectomy in a Patient with Carcinoid Heart Disease with Severe Tricuspid Regurgitation Using Venovenous Bypass: A Case Report

Aphichat Suphathamwit, MD,*a,c Achal Dhir, MD, FRCA (UK), FRCPC,a Wojciech Dobkowski, MD, FRCPC,a Douglas Quan, MD,MSc, FRCSC, FACSb

a

Department of Anesthesia and Perioperative Medicine and bDepartment of Surgery, Schulich

School of Medicine and Dentistry, Western University, University Hospital; 339 Windermere Road, London, Ontario N6A 5A5, Canada c

Department of Anesthesiology, Faculty of Medicine Siriraj Hospital, Mahidol University; 2

Prannok Road, Bangkoknoi, Bangkok 10700, Thailand

E-mail addresses: Achal Dhir: [email protected] Wojciech Dobkowski: [email protected] Douglas Quan: [email protected]

*Corresponding Author: Aphichat Suphathamwit, MD, Tel: +66-98-2786069; e-mail: [email protected]

Acknowledgments Conflicts of interest: None

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SUMMARY Functional gastrointestinal neuroendocrine tumor (GINET) can cause carcinoid syndrome and carcinoid heart disease. We report a 54-year-old female with GINET with hepatic metastasis and severe carcinoid heart disease. The surgical plan was to perform right hepatectomy with total vascular exclusion to reduce intraoperative hemorrhage from the congested liver, which was due to rightsided valvular disease. Invasive cardiovascular monitoring was applied using transesophageal echocardiography. Venovenous bypass (VVB) was used for hemodynamic support during cross-clamping of the inferior vena cava. Blood pressure dropped significantly after tumor resection, requiring vasopressor and octreotide infusions. The patient was transferred to the intensive care unit, where she required supportive treatment for right heart failure. She gradually recovered and was discharged home on postoperative day 15. She received tricuspid valve replacement 7 months later and has done well. We discuss the role of VVB in hepatic resection, octreotide infusion for intraoperative carcinoid crisis, and decision-making regarding the sequence of surgical intervention (heart vs. liver). Keywords carcinoid heart disease, hepatectomy, venovenous bypass

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INTRODUCTION Gastrointestinal neuroendocrine tumor (GINET) is an uncommon, slow-growing tumor arising from neuroendocrine cells. Its incidence, about 2.5–5 cases per 100,000, has been increasing up to 10% per year.1 Its ability to synthesize and secrete active peptides and other neuroamines can lead to carcinoid syndrome, which may manifest as cutaneous flushing, gastrointestinal hypermobility, and bronchospasm. Carcinoid heart disease is a process of endomyocardial fibroelastosis that usually affects the right heart valves (tricuspid and pulmonic) with involvement of sub-valvular apparatus. The degeneration of one or both right heart valves leads to dilation of the right heart chambers and right ventricular (RV) dysfunction, and the resulting increase in right-sided pressures and liver congestion can result in significant hemorrhage during liver resection. Here we present the successful management of right hepatectomy using venovenous bypass in a patient with severe tricuspid regurgitation and moderate pulmonic regurgitation due to carcinoid heart disease. We also present a review of literature regarding the appropriate sequence of surgeries in this unique situation.

CASE REPORT

A 54-year-old female (163 cm, 79 kg) diagnosed with GINET and liver metastases as well as RV dysfunction from carcinoid heart disease was scheduled for right hepatectomy. One year earlier she had undergone an octreotide scan and cross-sectional imaging and received a diagnosis of carcinoid tumor originating at the ileocecal valve. The lesion was suspected to be in the terminal ileum, but the patient had not undergone excision of the primary lesion at that time. She now presented with occasional flushing and shortness of breath. She was an exsmoker with a 15-pack-year smoking history, having quit 15 years ago, and denied any drug 3

4 or food allergies. Transthoracic echocardiogram revealed a significantly dilated right ventricle, severe tricuspid regurgitation (TR), mild pulmonary stenosis (PS) and moderate pulmonary regurgitation (PR). The patient had undergone chemoembolization of the hepatic artery 3 months before surgery, but her symptoms had remained unchanged. Her medications were furosemide, slow-release potassium chloride, and octreotide acetate for injectable suspension (60 mg subcutaneously every 21 days). On physical examination, she was in no acute distress. She had stable vital signs, no peripheral edema, and a pan-systolic murmur best heard over the right parasternal area. Her airway assessment was normal. She had a normal complete blood count and electrolyte levels. Her international normalized ratio (INR) was 1.2. Partial thromboplastin time was 22 s. Liver and kidney function tests were unremarkable. Chest x-ray and electrocardiogram were within normal limits. Preoperative urinary 5-hydroxyindoleacetic acid (5-HIAA) ranged from 7.7 to 11.06 mg/24 h. Because the patient had significant cardiac involvement in the form of severe TR and RV dilatation, multidisciplinary team meetings involving cardiac surgery, hepatobiliary surgery, and anesthesiology were held. Discussions mainly concerned the appropriate sequence of the two required surgeries, hepatectomy and tricuspid valve replacement. The cardiac surgeons believed the carcinoid disease would affect the new bioprosthesis and were therefore reluctant to replace the tricuspid valve first. After much discussion, the team decided to proceed with hepatectomy first. It was decided that venovenous bypass (VVB) would be performed because of the planned total vascular exclusion to prevent excessive hepatic congestion and blood loss from high right-sided cardiac pressure. The risks and benefits of the surgery were discussed extensively with the patient, and she provided written informed consent.

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5 In the operating room, after attaching the standard American Society of Anesthesiologists (ASA)-recommended monitors (which are the same as those recommended by the Canadian Anesthesiologists’ Society) and prior to the induction of anesthesia, an arterial line was inserted. Anesthesia was induced with midazolam, fentanyl, propofol, and rocuronium. After uneventful induction and intubation, octreotide 1,600 mcg (20 mcg/kg) was injected subcutaneously. Anesthesia was maintained with fentanyl, sevoflurane, and rocuronium. Bispectral index, esophageal temperature probe, and neuromuscular monitoring were also used. An introducer sheath and a venous bypass cannula were inserted under ultrasound guidance through the right internal jugular vein under strict aseptic conditions. Intravenous heparin infusion was started, keeping the activated clotting time around 200 s to prevent clotting of the VVB cannula.37 A transesophageal echocardiography (TEE) probe was introduced without complication, and a comprehensive TEE examination, performed by a board-certified echocardiographer, confirmed the preoperative findings of severe RV dilatation (Fig. 1) with mild RV dysfunction, severe TR (Figs. 2&3), mild PS (Figs. 4&5), moderate PR (Fig. 4), and mild pulmonary hypertension (Figs. 1–5). The patient was stable during the first 5 hours, while the surgeons mobilized and identified the major vessels of the liver. Some difficulties were encountered because of adhesions from previous cholecystectomy. Baseline central venous pressure (CVP) was 28 mmHg and was maintained between 16 and 25 mmHg. Mean arterial pressure (MAP) was maintained at around 60–70 mmHg by administering crystalloid solution and a small amount of colloid (5% albumin). Hourly arterial blood gas analyses were performed to monitor blood electrolytes, lactate, and hemoglobin concentrations. A Pringle maneuver (clamping of hepatic artery and portal vein at the level of the hepatoduodenal ligament) was performed, followed by trial clamping of the inferior vena cava (IVC).Within a minute of IVC clamping, there was a precipitous drop in blood pressure to a MAP of 30 mmHg and a CVP of

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6 6 mmHg, requiring release of the clamps. We then introduced a 19-French venous cannula from the left femoral vein into the IVC. VVB was initiated and flow rate was > 2 L/min. Even with VVB, MAP dropped to 40–50 mmHg after total vascular exclusion but was manageable with intravenous infusions of norepinephrine at 3–5 mcg/min and octreotide 100 mcg/hr. Hemoglobin concentration was 8.2 g/dL; however, in view of the low BP, blood loss of about 1,000 mL, and more blood loss expected, packed red blood cells were transfused . Approximately 90% of the entire tumor mass was successfully resected (Fig. 6). Despite total vascular exclusion, there was still significant blood loss during hepatic parenchymal transection (about 3,000 mL in 1 hour), so transfusion of packed red blood cells was continued. Because point-of-care tests of coagulation such as thromboelastography and rotational thromboelastometry were not available, we decided to transfuse fresh frozen plasma based on an INR of 1.5. Immediately after the extended right hepatectomy (segments IV–VIII), the patient’s blood pressure dropped to 50 mmHg systolic in the absence of further significant blood loss. Release of the vascular clamp increased the CVP to 15 mmHg but did not improve the BP. TEE did not reveal any new abnormal changes at this stage, and carcinoid crisis was suspected. Epinephrine infusion at 2 mcg/min was then started along with two intravenous boluses of octreotide 100 mcg each and another 100 mcg injected subcutaneously. About 20 min after hepatectomy, the BP started to increase and remained stable thereafter. We were able to discontinue epinephrine infusion, but infusions of norepinephrine 5 mcg/min and octreotide 100 mcg/hr were continued for 48 hours in the ICU. Total operative duration was 9 hours, with a VVB time of 30 minutes. Estimated blood loss was about 5 L. In total, our patient received 8 L of crystalloid, 500 mL of albumin, 6 units of packed red blood cells, and 1,000 mL of fresh frozen plasma. Her platelet count

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7 dropped from 170 × 103/mL to 92 × 103/ mL after transfusion of crystalloid, 4 units of packed red blood cells, and fresh frozen plasma, but we decided not to transfuse platelets. During the postoperative period, the patient developed a pleural effusion and cardiac failure requiring chest-tube drainage and diuretic therapy. As expected, she developed hepatic dysfunction that peaked on postoperative day 2, manifesting as elevation of liver enzymes to an ALT of 164 u/L and an AST 220 u/L and elevation of INR to 2. However, these parameters trended down and became normal by postoperative day 10. The patient was discharged home on postoperative day 15. Her laboratory values at different time points are presented in Table 1. The patient was followed by a team of surgeons, oncologists, and cardiologists. Two months later, her urinary 5-HIAA had dropped to 1.25 mg/24 hours. Another TTE was performed 7 months after hepatic resection and before tricuspid valve replacement. Interestingly, it showed nonsignificant PR with trivial PS, severe TR, and improved RV function. Computed tomography revealed evidence of residual hepatic carcinoid disease. She underwent an uneventful tricuspid valve replacement and was doing well 18 months after cardiac surgery.

DISCUSSION GINET is relatively uncommon, with an incidence of only 0.49% of all malignancies.2 More than 60% of carcinoid tumors originate from the gastrointestinal tract. The liver is the most common site of distant metastasis, occurring there in more than 50% of patients.4 Vasoactive peptides and various mediators, mainly serotonin and histamine from the tumor cells, are the major cause of carcinoid syndrome. The classic manifestations are cutaneous flushing and gastrointestinal hypermotility. Other symptoms, such as nausea, 7

8 vomiting, diarrhea, hypertension, and hypotension, are also seen in more than 80% of patients.5 Carcinoid heart disease is a late finding of carcinoid syndrome, manifesting in up to 70% of patients with carcinoid syndrome.6 It results from vasoactive compounds bypassing the detoxification process of the liver and ending up in the heart. The deposition of fibrous tissues on the endocardium and right heart valves results in endomyocardial fibroelastosis. This leads to valvular stenosis and/or regurgitation, ultimately affecting right heart function. The left side of the heart is rarely affected (< 10% of cases) because of inactivation of the vasoactive mediators in the lungs.7,8 Anesthetic management of a patient with carcinoid syndrome and carcinoid heart disease have been thoroughly investigated9,10,11,12 and include careful preoperative assessment of disease severity, degree of cardiac involvement, and current treatment; avoidance of drugs causing histamine release; invasive hemodynamic monitoring; and somatostatin-analog (octreotide) therapy. Severe TR results in poor right heart function and increased right heart pressure. These lead to pulsatile liver, which prohibits safe hepatic resection. In the present case, we planned total hepatic vascular exclusion to reduce blood loss from the congested liver. The patient did not tolerate the dramatic reduction of venous return resulting from IVC occlusion, and we could not administer excess fluids before clamping, as we do for liver transplant surgery, because of her right heart dysfunction. Hence, VVB was used to improve cardiac preload. VVB was widely used in the early period of conventional liver transplantation. Many authors reported a number of benefits, such as improved hemodynamic stability, better perfusion of the kidneys and intestine, and improved short-term survival rates.13-15 Currently, with the introduction of piggyback technique and some recent studies finding no distinct

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9 advantage of VVB,38 it has become less popular for liver transplantation surgery. However, in some centers VVB is still performed routinely and has proven useful under specific conditions; in particular, in the presence of impaired cardiac function.16-18 VVB is also considered helpful in major liver resections in which total vascular exclusion is required, such as hepatic tumor involving the hepatic vein19 or IVC,20,21 as well as in patients with cirrhosis.22 It could be argued that by using a heparin-bonded circuit, we could have avoided heparin infusion and possibly the use of fresh frozen plasma. Because of the unavailability of either a heparin-bonded circuit or point-of-care coagulation tests, we chose to transfuse fresh frozen plasma because of an INR of 1.5 and the possibility of dilutional coagulopathy. Severe hypotension occurred in our patient despite the use of VVB. Possible explanations are hypovolemia, embolism, right-sided cardiac failure, or carcinoid crisis. A pulmonary artery catheter may have provided additional information, such as pulmonary artery pressure and cardiac output. However, a pulmonary artery catheter was not floated because of pulmonary valve lesions, and we believe that in the presence of continuous TEE monitoring, a pulmonary artery catheter would not have helped to guide hemodynamic management. Fluid status was guided by TEE, which also ruled out any embolic phenomena and/or significant cardiac dysfunction. We concluded that the hypotension was likely secondary to carcinoid crisis. In addition to the octreotide infusion, norepinephrine was first used to counter carcinoid crisis-related vasodilation. Epinephrine was also infused in low doses to help improve cardiovascular performance, as we did not want to rely solely on high doses of norepinephrine. Some reports have suggested that beta-agonist drugs can indirectly promote the release of vasoactive peptides, further worsening the hypotensive episode.10,24 However, many recent studies have demonstrate the effectiveness of vasopressors along with octreotide during hypotension caused by carcinoid crisis.1,3,9,11

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10 Octreotide, a somatostatin analog, is now the drug of choice for patients with carcinoid syndrome and carcinoid crisis.24,25 It not only inhibits the release of vasoactive peptides from carcinoid tumor cells,24 but also blocks the action of mediators already released into the circulation.3 Various recommendations for dosage, route, and timing of octreotide administration have been proposed, but no standard regimen is completely reliable (Table 2). A study by Seymour and Sawh revealed that octreotide doses ranging from 25 to 500 mcg intravenously followed by an infusion of 50–150 mcg/hr can effectively treat a hypotensive episode resulting from carcinoid crisis.27 A higher dose of octreotide rescue is expected for patients with carcinoid heart disease and those with poorly controlled carcinoid syndrome.27,28 At our center, we routinely administer 500 mcg of octreotide subcutaneously 2 h preoperatively for all patients undergoing liver resection for metastatic GINET. Another important issue thoroughly discussed by the multidisciplinary team was whether tricuspid valve replacement or hepatic resection should be performed first. If the heart is minimally affected, there may be no difference. However, such a decision is challenging if both organs are severely affected. The cardiac surgeons were reluctant to perform tricuspid valve replacement first because they believed hormone secretion from the hepatic metastases was quite significant. They argued that the bioprosthesis would be affected by the disease process before hepatic surgery could be safely performed. Bhattacharyya et al. observed a significant increase in median survival rate of surgically treated carcinoid heart disease from 11 to 72 months, with impressive improvement in New York Heart Association functional class.29 Tumor resection also increased the 5-year survival rate of carcinoid heart disease patients from 29% to 86%.30 However, the perioperative mortality rates of carcinoid heart disease intervention ranged from 7% to 16%,28,31-33 although a clear trend toward better surgical outcomes has been observed over time.34 In contrast, the mortality rate of patients who underwent

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11 abdominal surgery for metastatic carcinoid tumor is reported to be as high as 12%, and half had carcinoid heart disease.35 Both approaches have considerable risk. Several reports favor surgery for carcinoid heart disease before hepatic resection. A study by Lillegard et al. concluded that correction of the heart disease first would permit safe hepatic resection in approximately 65% to 75% of cases.2 Mokhles et al. found that progressive heart failure, rather than carcinoid syndrome itself, is the most common cause of death.36 They also found low perioperative mortality after heart surgery and dramatic improvements in cardiac functional capacity and clinical outcomes. Castillo et al. recommended that because RV failure may increase perioperative mortality, it might be prudent to perform early surgical intervention in patients with mildly symptomatic carcinoid heart disease.1 Another approach could be to perform both surgeries simultaneously, but there has been no such case report in the literature. Finally, there is no high-level evidence to support the best approach for this kind of case. The best management remains an individual decision and depends on the patient’s clinical condition, perioperative resources, and the involvement of an experienced multidisciplinary team. GINET is a rare, slow-growing tumor that can cause systemic effects known collectively as carcinoid syndrome. Carcinoid heart disease commonly affects the right heart chambers through the deposition of fibrous tissue, which causes valvular degeneration. Hepatic resection of the metastatic carcinoid tumor and valve repair improve clinical outcome and prolong survival, but the appropriate sequence of surgeries requires further study. If hepatic resection is performed first, total vascular exclusion with venovenous bypass provides substantial support and can be used in cases of significant untreated heart disease.

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18.

Beltran J, Taurá P, Grande L, et al: Venovenous bypass and liver transplantation.

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Hemming AW, Reed AI, Langham MR Jr, et al: Combined resection of the liver and

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Yamaoka Y, Ozawa K, Kumada K, et al: Total vascular exclusion for hepatic

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Hamid SK, Harris DNF: Hypotension following valve replacement surgery in

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Kvols LK, Martin JK, Marsh HM, et al: Rapid reversal of carcinoid crisis with a

somatostatin analogue. N Engl J Med 313:1229-1230, 1985 26.

Le BT, Bharadwaj S, Malinow AM: Carcinoid tumor and intravenous octreotide

infusion during labor and delivery. Int J Obstet Anesth 18:182-185, 2009 27.

Semour N, Sawh SC: Mega-dose intravenous octreotide for the treatment of carcinoid

crisis: a systematic review. Can J Anesth 60:492-499, 2013 28.

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patients with carcinoid heart disease having valvular surgery: a review of one hundred consecutive cases. Anesth Analg 105:1192-1199, 2007 29.

Bhattacharyya S, Davar J, Dreyfus G, et al: Carcinoid heart disease. Circulation

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12 cases. Gen Thorac Cardiovasc Surg 59:780-785, 2011 35.

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abdominal surgery for metastatic carcinoid tumors. Br J Anaesth 87:447-452, 2001

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Mokhles P, van Herwerden LA, de Jong PL, et al: Carcinoid heart disease: outcomes

after surgical valve replacement. Eur J Cardiothorac Surg 41:1278-1283, 2012 37.

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transplantation. HPB (Oxford) 10:196-203, 2008

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16 FIGURE LEGENDS Fig 1. Intraoperative transesophageal echocardiography showing severe right ventricle dilatation.

Fig 2. Intraoperative transesophageal echocardiography showing severe tricuspid uspid regurgitation.

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Fig 3. Intraoperative transesophageal echocardiography. Hepatic vein flow depicting severe tricuspid regurgitation (systolic reversal flow).

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Fig 4. Intraoperative transesophageal echocardiography showing mild pulmonary artery stenosis and moderate pulmonary regurgitation.

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Fig 5. Intraoperative transesophageal echocardiography. Pulmonary valve velocity time integral showing mild pulmonic stenosis.

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20 Fig 6. Intraoperative photograph of resected tumor.

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21 Table 1. Selected Laboratory Values Time point

Intraoperative

Postoperative

Variabl POD e

Preoperati

2 hr pre

30 min pre

1 hr post

ve

hepatecto

hepatecto

hepatecto

my

my

Immediat

2

PO

e

(peak

D5

my )

Hb 155

115

82

99

73

81

87

250

170

79

92

103

66

174

1.2

1.5

1.5

1.5

1.8

2

1.6

-

2.2

3.6

6.3

6.3

7.2

3.5

10

-

-

109

164

58

17

-

-

248

220

39

(g/L) Platelet s (*103 /mm3) INR Lactate s ALT (units/ L) AST (units/ L) ALT, alanine aminotransferase; AST, aspartate aminotransferase; Hb, hemoglobin; INR, international normalized ratio; POD, postoperative day.

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22 Table 2. Perioperative Octreotide Management AuthorsReference no. Modlin et al.3

Ellis et al.1

Preoperative

Intraoperative

50 mcg before skin

50-mcg/hr infusion

incision

(50-mcg boluses)

50–100 mcg/hr

50–100 mcg/hr

50–100 mcg/hr

overnight

50–100 mcg boluses

up to 48 hours

Le et al.26

25 mcg/hr

22

Postoperative