Orthotopic Liver Transplantation in a Patient with Acutely Decompensated Liver Disease and Personal History of Malignant Hyperthermia.

Introduction: Orthotopic liver transplants are characterized by sudden changes in hemodynamics, intraoperative hemorrhage, metabolic and electrolyte derangements, and arrhythmias. Many of these features are also hallmarks of malignant hyperthermia episodes and make differentiation difficult intraoperatively. Additionally, the treatment for malignant hyperthermia, dantrolene, can cause hepatotoxicity in already damaged native livers and newly reperfused organ allografts. Thus, it is imperative to avoid a triggering anesthetic in these patients. Here we report on a successful total intravenous anesthetic in a malignant hyperthermia susceptible individual undergoing an orthotopic liver transplant for acutely decompensated end-stage liver disease. Case Presentation. A 49-year-old male with a past medical history significant for malignant hyperthermia episodes as a child was admitted with decompensated alcoholic cirrhosis. He underwent uneventful total intravenous general anesthesia with propofol and sufentanil continuous infusions for an orthotopic liver transplant. He required minimal vasoactive agents to maintain a mean arterial blood pressure >65 mmHg and was extubated on postoperative day 1. Conclusions: Total intravenous anesthesia is necessary for patients with a personal history of malignant hyperthermia. However, this type of general anesthesia is difficult in the setting of fluctuating hemodynamics, hemorrhage, and changes in drug metabolism and clearance during the anhepatic and reperfusion phases of an orthotopic liver transplant. Propofol and sufentanil continuous infusions provided stable hemodynamics and an excellent plane of anesthesia throughout the case and should be considered in other individuals undergoing this procedure who require a total intravenous anesthetic.

Cardiac arrhythmia from epinephrine overdose in epidural test dose.

Medication shortages are a clinical reality that force changes in practice patterns leading to unintended consequences. Potential solutions to any drug shortage require a thoughtful, multidisciplinary and often creative approach. Here, we report a case of unintentional epinephrine overdose leading to an unstable cardiac arrhythmia and our subsequent development of a visual cue system to prevent future errors. A 56-year-old man with a history of rectal adenocarcinoma presented for low anterior resection and creation of diverting loop ileostomy. Epidural placement was requested by the surgical team, and following administration of a second test dose (created by the physician), the patient experienced supraventricular tachycardia with heart rates of 200-210 BPM for approximately 2 minutes. This rhythm then converted to atrial fibrillation with rapid ventricular response with heart rate of 150-170 BPM. The patient was stabilized after cardioversion. Later evaluation of medication administration revealed that the second epidural test dose inadvertently contained 100 mcg epinephrine instead of the intended 10 mcg dose. The test dose had to be created because the original ampule with the kit had been utilized. Since this time, our kits have no test dose, and this shortage is concerning for increased provider error. We suggest a novel visual cue system that may prevent unintentional epinephrine overdoses in the setting of regional anesthesia.

Risk Factors for Intracardiac Thrombus During Liver Transplantation.

Intracardiac thrombus (ICT) is an intraoperative complication with high mortality that occurs during orthotopic liver transplantation (OLT). Patients with end-stage liver disease have compromised coagulation pathways, and when combined with stressors of surgery, thrombi can form. However, it is unknown which patients are most likely to develop ICT. We performed a retrospective cohort study of all OLT patients at our hospital from 2010 to 2017 to identify risk factors for ICT. An analysis was performed with conventional bivariate tests and logistic regression. The incidence of ICT during OLT was 4.2% (22/528) with a 45.5% (10/22) mortality. Patients who developed ICT had higher physiologic Model for End-Stage Liver Disease scores at the time of transplant (25.1 versus 32.4; P = 0.004), received grafts from donors with a higher body mass index (28.1 versus 32.2 kg/m2 ; P = 0.007), and had longer intraoperative warm ischemia times (53.1 versus 67.5 minutes; P = 0.001). The odds of developing ICT were significantly lower after administration of intravenous (IV) heparin prior to inferior vena cava (IVC) clamping compared with no administration of heparin (odds ratio, 0.25; 95% confidence interval, 0.08-0.75; P = 0.01). In conclusion, the incidence of ICT at our institution is higher than previously reported, which may be explained by our routine use of transesophageal echocardiography. Although many factors associated with ICT in this study are nonmodifiable, administration of IV heparin prior to IVC cross-clamping is modifiable and was found to be protective. Further studies will be needed to confirm findings and ultimately aid in preventing these lethal events.

Intermittent Boluses of Local Anesthetic Through Quadratus Lumborum Catheters for Analgesia in a Living Donor Hepatectomy.

The demand for liver transplants in the United States far exceeds the supply of organs. As need has increased, so has use of living donors. Coagulopathy and various side effects often preclude the use of neuraxial regional techniques and opioids for postoperative analgesia in patients with large "J" incisions. Here, we present a 25-year-old male undergoing a living donor hepatectomy who received quadratus lumborum catheters placed percutaneously after closure of incision and prior to emergence to provide excellent analgesia and a viable opioid-sparing approach. Quadratus lumborum catheters are a safe option for a multimodal, opioid-sparing approach to analgesia.

Optimal DNA tier for the IRT/DNA algorithm determined by CFTR mutation results over 14 years of newborn screening.

BACKGROUND: There has been great variation and uncertainty about how many and what CFTR mutations to include in cystic fibrosis (CF) newborn screening algorithms, and very little research on this topic using large populations of newborns. METHODS: We reviewed Wisconsin screening results for 1994-2008 to identify an ideal panel. RESULTS: Upon analyzing approximately 1 million screening results, we found it optimal to use a 23 CFTR mutation panel as a second tier when an immunoreactive trypsinogen (IRT)/DNA algorithm was applied for CF screening. This panel in association with a 96th percentile IRT cutoff gave a sensitivity of 97.3%, but restricting the DNA tier to F508del was associated with 90% (P<.0001). CONCLUSIONS: Although CFTR panel selection has been challenging, our data show that a 23 mutation method optimizes sensitivity and is advantageous. The IRT cutoff value, however, is actually more critical than DNA in determining CF newborn screening sensitivity.

Opportunities for quality improvement in cystic fibrosis newborn screening.

BACKGROUND: With the rapid implementation of cystic fibrosis (CF) newborn screening (NBS), quality improvement (QI) has become essential to identify and prevent errors. Using Process Failure Modes and Effects Analysis (PFMEA), we adapted this method to determine if it could be applied to discover and rank high priority QI opportunities. METHODS: Site visits to three programmes were conducted, and PFMEA exercises were accomplished in Colorado, Massachusetts and Wisconsin with 23 experienced professionals. During each of these comprehensive sessions, participants identified and ranked potential failures based on severity, occurrence and detection to calculate risk priority number (RPN) values. RESULTS: A total of 96 failure modes were generated and ranked in a list of the 20 riskiest problems that show no significant discordances by site, although there were differences by profession of the rater, particularly nurses. CONCLUSIONS: Our results illustrate that the PFMEA method applies well to CF NBS and that steps requiring communication and information transfer are perceived to be the highest risks. The number of identified failures makes and their potential impact demonstrate considerable overall risk and a need for ongoing QI.