Drug management in emergent liver transplantation of mitochondrial disorder carriers: review of the literature.

BACKGROUND: Mitochondrial respiratory-chain disorders (MRCD) lead to progressive disabling of neurological and cellular conditions that involve muscles, brain, kidney, and liver dysfunction. Affected individuals may need surgery, including orthotopic liver transplantation (OLT). Surgery poses anesthesia challenges because of the prolonged use of anesthetic drugs and sedatives, which may inhibit oxidative phosphorylation, mimic mitochondrial cytopathic disorders, or unveil them ex novo. MATERIALS AND METHODS: We conducted a multilingual PubMed search of surgical and non-surgical anesthesia reports between the years 1992 and 2008, where anesthetic drugs were used in MRCD patients, especially for those undergoing urgent OLTs. RESULTS: There were 51 case reports of 210 anesthesia and critical care interventions in patients with MRCD, a large part of them were children. Data pertaining to the safe usage of anesthesia and perioperative drugs were limited and conflicting. We found no article that addressed the issue of perioperative handling of urgent OLT in MRCD patients. We therefore suggest our own - although limited - experience for such occasions. CONCLUSION: There are no randomized, controlled, trial-based indications regarding safe anesthetic drugs to be used perioperatively in MRCD carriers. Consultation among geneticists, anesthesiologists, intensivists, and surgeons is essential in patients with known/suspected metabolic syndrome for planning appropriate perioperative care.

Role of Transesophageal Echocardiography in the Diagnosis of Multi-chamber Intracardiac Thrombosis During Liver Transplantation: A Case Series.

Intra-cardiac thrombosis is one of the most devastating complications during liver transplantation. In the majority of cases, ICT, followed by massive pulmonary embolism, is commonly occurring shortly after liver graft reperfusion, but it has been reported to occur at any stage of the surgery. We present a series of 3 cases of intra-cardiac thrombosis during orthotopic liver transplantation surgery, including a case of four-chamber intra-cardiac clot formation during the pre-anhepatic stage. This article represents a single-centre 14 year-long experience. Intra-operative TEE is the gold standard to diagnose intra-cardiac thrombosis, monitoring its size, location and dynamics, as well as myocardial performance and the effects of resuscitation efforts.

Anesthetic implications of the new anticoagulant and antiplatelet drugs.

In this review, we discuss the anesthetic implications of the new anticoagulant and antiplatelet drugs, focusing our discussion mainly on neuroaxial/regional anesthesia and central catheter placement issues. We offer practical recommendations for their use.

Laryngeal mask anesthesia in children: a case report.

The laryngeal mask airway has become one of the major tools of modern anesthesia airway management. Despite the fact that no time limit has been recommended regarding its safe use in spontaneously breathing children, or adults, there is still reluctance to use the laryngeal mask airway in operations of long duration. We report the case of an uneventful 5-hour long laryngeal mask anesthesia in a spontaneously breathing 11-year-old boy undergoing lower limb surgery.

A randomized clinical trial testing the anti-inflammatory effects of preemptive inhaled nitric oxide in human liver transplantation.

Decreases in endothelial nitric oxide synthase derived nitric oxide (NO) production during liver transplantation promotes injury. We hypothesized that preemptive inhaled NO (iNO) would improve allograft function (primary) and reduce complications post-transplantation (secondary). Patients at two university centers (Center A and B) were randomized to receive placebo (n = 20/center) or iNO (80 ppm, n = 20/center) during the operative phase of liver transplantation. Data were analyzed at set intervals for up to 9-months post-transplantation and compared between groups. Patient characteristics and outcomes were examined with the Mann-Whitney U test, Student t-test, logistic regression, repeated measures ANOVA, and Cox proportional hazards models. Combined and site stratified analyses were performed. MELD scores were significantly higher at Center B (22.5 vs. 19.5, p<0.0001), surgical times were greater at Center B (7.7 vs. 4.5 hrs, p<0.001) and warm ischemia times were greater at Center B (95.4 vs. 69.7 min, p<0.0001). No adverse metabolic or hematologic effects from iNO occurred. iNO enhanced allograft function indexed by liver function tests (Center B, p<0.05; and p<0.03 for ALT with center data combined) and reduced complications at 9-months (Center A and B, p = 0.0062, OR = 0.15, 95% CI (0.04, 0.59)). ICU (p = 0.47) and hospital length of stay (p = 0.49) were not decreased. iNO increased concentrations of nitrate (p<0.001), nitrite (p<0.001) and nitrosylhemoglobin (p<0.001), with nitrite being postulated as a protective mechanism. Mean costs of iNO were $1,020 per transplant. iNO was safe and improved allograft function at one center and trended toward improving allograft function at the other. ClinicalTrials.gov with registry number 00582010 and the following URL:http://clinicaltrials.gov/show/NCT00582010.

Treatment of severe lactic acidosis during the pre-anhepatic stage of liver transplant surgery with intraoperative hemodialysis.

Severe uncompensated lactic acidosis manifesting during the pre-anhepatic stage of orthotopic liver transplant surgery is an uncommon event, but it poses serious concern because of the additional lactate production and impaired elimination by the liver that develops during the anhepatic and allograft reperfusion stages of the procedure. A man with end-stage liver disease secondary to hepatitis C and hemochromatosis and normal renal function, who developed severe lactic acidosis in the pre-anhepatic stage of liver transplantation, was treated successfully with intraoperative, continuous venovenous hemodialysis. Hemodialysis effectively corrected the patient's lactic acidosis and removed lactate, which contributed to hemodynamic stability during the anhepatic and graft reperfusion stages of his liver transplant surgery.

Intraoperative epoprostenol and nitric oxide for severe pulmonary hypertension during orthotopic liver transplantation: a case report and review of the literature.

BACKGROUND: The presence of pulmonary hypertension in patients scheduled for liver transplantation requires a comprehensive perioperative heart evaluation and treatment with epoprostenol (prostacycline) infusion until a liver donor becomes available. We contended that intraoperative attenuation of severe pulmonary hypertension could be achieved by epoprostenol infusion combined with nitric oxide inhalation. CASE REPORT: A 49 years old man with end stage liver disease secondary to hepatitis C and ethanol abuse presented for orthotopic liver transplantation. The case was complicated by severe pulmonary hypertension. Preoperative epoprostenol, at doses ranging from 6 to 26 ng.kg(-1).min(-1), was infused during the induction of anesthesia. Although lower than before (>70 mmHg), post-induction pulmonary pressure (by Swan-Ganz catheter) was 62/30 mmHg. Prior to surgical incision nitric oxide (NO) by inhalation was commenced, increasing the concentration from 10 to 40 ppm; pulmonary artery pressure (PAP) then declined to 55/25 mmHg. Before starting reperfusion of the transplanted liver, NO concentration was increased to 80 ppm: this allowed completion of the procedure with PAP at 32/16 mmHg. Real time transesophageal echocardiography indicated improvement in right heart function due to NO. Following surgery, NO was continued for 10 hs at a concentration of 40 ppm and the patient was then extubated. Epoprostenol infusion was continued for 2 months after the patient was discharged home; last PAP was measured 32/10 mmHg. CONCLUSIONS: Severe intraoperative pulmonary hypertension during liver transplantation was successfully treated using the combination of IV epoprostenol infusion and NO inhalation in medium and high concentrations.

Preoperative fasting after soft drink intake: 2 hours may be enough.

Sufficient preoperative fasting time is essential for safe induction of anesthesia to prevent aspiration of gastric contents. However, the time recommended for sufficient preoperative fasting varies greatly, depending on the nature of the oral intake, from 2 h for clear liquids to 6 h for solid foods. We report the case of a 30-year-old man who drank about 600 ml of the carbonated, glucose-rich soft drink, 7-UP, 2 h before surgery and absorbed nearly all of it within these 2 preoperative hours.

Adjuvant drugs for end-stage liver failure and transplantation.

End-stage liver failure is currently treatable both by dialysis and by liver transplantation, but this does not detract from its being a complex pathophysiological and pharmacological entity. More patients survive after transplant because of the impressive developments that enabled improved liver preservation, anesthesia and surgical techniques, as well as immunosuppressive drug therapy. Because of its multifaceted metabolism, liver failure can nevertheless cause a complex of pathophysiological conditions and, as such, poses a challenge for surgeons and anesthesiologists alike, not only before surgery but during transplantation as well. Obviating these problems frequently requires the use of adjunct drugs before, during and after liver transplantation, and these medications must be carefully balanced with each other while being alert to the avoidance of negative side effects. Hepatorenal syndrome and massive bleeding are the two main grave phenomena that characterize these patients before and during liver transplant, and this article will provide an overview of the adjunct drugs that are used to circumvent them perioperatively. Specifically, it details the drugs that are used to preserve and improve the already precarious hemodynamic conditions (e.g, by vasopressors, vasodilators, and beta blockers), as well as diuretics and hepato-protective drugs (e.g, furosemide, mannitol), antifibrinolytics (including the new recombinant activated Factor VII) and immunosuppressive drugs. Their doses and the ongoing debate on their concomitant use are reported as well.

Unexpected surgical difficulties leading to hemorrhage and gas embolus during laparoscopic donor nephrectomy: a case report.

PURPOSE: To report the case of a laparoscopic donor nephrectomy in which the preoperative evaluation of the patient gave no indication of the surgical difficulties that were encountered intraoperatively, resulting in substantial bleeding, a suspected gas embolism, and emergency conversion of the procedure from laparoscopic to open donor nephrectomy. CLINICAL FEATURES: A 59-yr-old man - height: 175 cm, weight: 85.5 kg, American Society of Anesthesiologists physical status I - presented as kidney donor for laparoscopic donor nephrectomy. He was healthy, on no medication, and had no previous abdominal surgery or diseases of the urinary tract. The preoperative computed tomography (CT) scan evaluation of his kidneys confirmed this by reporting a normal bilateral renal and renal vascular anatomy. In contradiction to the preoperative CT scan findings, the surgeon discovered abnormalities in the operative field. This included extensive scarring surrounding the left kidney, adenopathy near the right hilum, and a large branch lumbar vein entering the renal vein. The large branch lumbar vein was clipped but the clips dislodged, causing significant blood loss, and a suspected gas embolus. The procedure was converted to an emergency open donor nephrectomy. Postoperatively the patient made a full recovery. CONCLUSION: Laparoscopic donor nephrectomies, though usually performed on healthy individuals, have their pitfalls, and complications during this procedure can be sudden and serious. As shown in this case, although CT scan results are regarded as reliable, they can be misleading. As an anesthetic precaution for possible gas emboli during laparoscopic procedures, nitrous oxide should be avoided and the patient be ventilated with 100% oxygen.