enzymes were within normal reference ranges, and the liverappeared normal on a liver ultrasound image. A tentativediagnosis of ‘‘end-stage liver failure’’ was made without abiopsy.

Massive hemoptysis developed shortly after ECMO wasinitiated, which was successfully managed with continua-tion of ECMO and ventilator management.1 The patient’stotal bilirubin continued to increase to a peak of 56 mg/dl onECMO Day 9 (Figure 1). A molecular adsorbent recirculat-ing system (MARS) was initiated on ECMO Days 9 though14. The bilirubin improved dramatically with MARS.

A specimen of a liver biopsy, performed while on ECMO,provided a definitive diagnosis of cholestasis withoutcirrhosis. The patient underwent HeartMate II (Thoratec,Pleasanton, CA) left ventricular assist device (LVAD)placement and ECMO removal on ECMO Day 20. Therewas no further episode of liver failure, and the patient waseventually discharged from the hospital.

VA-ECMO is a support therapy for the patients withprofound cardiac and/or respiratory failure, and its outcomesare closely related with end-organ recovery.2 Non-survivorsof ECMO follow a common pathway of development ofintractable and progressive multi-organ dysfunction. Deathcan rapidly follow the development of liver dysfunction.Current therapy of liver failure superimposed on cirrhosis islimited to treat the precipitating event; if the precipitatingevent can be eliminated, the liver can recover to its previouscompensated state. Patients with liver failure accumulatetoxic molecules, and accumulation of these substances mayinduce encephalopathy, hypotension, renal failure, macro-phage dysfunction, and/or inhibition of hepatocyte recovery.

MARS is a cell-free extracorporeal liver support devicebased on the principle of albumin dialysis.3 Blood isperfused through a specific membrane dialyzer that uses20% albumin as a molecular adsorbent that is recirculatedand perfused in-line through charcoal and anion exchangercolumns. Toxic molecules accumulated in the plasma can beeliminated by passage of blood or plasma over sorbentcolumns because most of these molecules are lipophilic andalbumin-bound. The molecules that can be removed byMARS include bile acids, conjugated bilirubin, aromaticamino acids, medium chain fatty acids, mercaptans, andcytokines. MARS will not only remove selectively albumin-bound substances but also eliminate water-soluble toxinssuch as ammonia and creatinine.3 Removal of these toxinsby MARS may preserve end-organ functions and decreasemolecule levels of toxins to facilitate organ recovery4

Previously, Peek et al3 reported that no patient survivedfrom ECMO with MARS if the bilirubin was greater than 23mg/dl (400 mmol/liter); however, the peak bilirubin of ourpatient was 56 mg/dl (957 mmol/liter). A decrease in serumbilirubin for 3 consecutive days was the primary end point ofMARS support on this patient. The bilirubin level remainedstable and did not increase after completion of MARStreatment. A liver biopsy specimen confirmed the reversiblepathology of liver. At this point, this patient’s end-organ (liver)dysfunction was resolving. Right ventricular function recov-ered while on ECMO, and she no longer required biventricularsupport. Chronic LV failure necessitated the implantable

LVAD. MARS was initiated as a bridge to the decision toLVAD, eliminating elevated bilirubin without adverse effects.Retrospectively, the liver dysfunction may have been transient,although the peak serum bilirubin level was fatally high. TheLVAD implantation was prohibited until hepatic failure wasresolved with MARS.

Disclosure statement

None of the authors has a financial relationship with a commercialentity that has an interest in the subject of the presented manuscriptor other conflicts of interest to disclose.

References

1. Harrison M, Cowan S, Cavarocchi N, Hirose H. Massive hemoptysis onveno-arterial extracorporeal membrane oxygenation. Eur J CardiothoracSurg 2012;42:587-9.

2. Schwarz B, Mair P, Margreiter J, et al. Experience with percutaneousvenoarterial cardiopulmonary bypass for emergency circulatory support.Crit Care Med 2003;31:758-64.

3. Peek GJ, Killer HM, Sosnowski MA, Firmin RK. Modular extra-corporeal life support for multiorgan failure patients. Liver 2002;22:69-71.

4. El Banayosy A, Kizner L, Schueler V, Bergmeier S, Cobaugh D,Koerfer R. First use of the molecular adsorbent recirculating systemtechnique on patients with hypoxic liver failure after cardiogenic shock.ASAIO J 2004;50:332-7.

A novel drive-line handling tool for the HeartWareventricular assist device in patients with a physicaldisability

Prashant N. Mohite, MS, MRCS, Christopher T. Bowles, PhD,and Andre R. Simon, MD

From the Department of Cardiothoracic Transplantation andMechanical Support, Royal Brompton and Harefield NHS Trust,Harefield Hospital, Harefield, Middlesex, United Kingdom.

Figure 1 Total bilirubin was significantly improved aftermolecular adsorbents recirculating system (MARS) was appliedduring extracorporeal membrane circulation (ECMO), which wasfollowed by left ventricular assist device (LVAD).

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The HVAD (HeartWare, Inc., Framingham, MA) is athird-generation ventricular assist device (VAD).1 Thesystem includes a controller that operates the pump andprovides operating parameters and alarms.2 In the case offailure, patients must be able to replace the controllerpromptly with a backup, and it is standard practice to ensurethat patients are capable of safely performing suchexchanges prior to discharge. The maneuver depends onthe grip of the thumb to the index finger, which determinesthe patient’s ability to retract the drive-line connector’s lockingring to allow its removal from the controller socket.Anticipated or proven inability to perform these tasks isconsidered a relative contraindication to VAD implantation bymany centers and patients who are unable to do so are requiredto be accompanied by a trained companion at all times.

There are a number of reasons why patients may lack theability to exchange the controller including advanced age,neurologic deficit, cognitive dysfunction or intensive careneuropathy. We present the case of a young man with end-stage heart failure and arthrogryposis multiplex with

congenital deformities that were preventing him fromdisconnecting the drive-line from the controller. Wedesigned a handling tool (Figure 1A) consisting of acylindrical nylon housing with a flange to improve manualgrip, which is placed as shown in Figure 1B. The drive-lineplug locks into a slot, allowing two spring clips to engageinto the recesses of the drive-line connector’s locking ring.When the tool is pulled away from the controller, the drive-line plug is readily extracted (Figure 1C). This tool hasenabled the patient to live independently for 18 months.

Between 2007 and 2012, 45 of a total of 119 patientsunderwent HVAD implantation at our institution. Of the 36patients who were successfully discharged, 3 (8.3%) werefound to be unable to extract their drive-line safely. Two weresuccessfully issued an extraction tool, the third requires atrained carer in constant attendance. In this patient cohort,6 emergency controller exchange events occurred (6 of 36,17%) of which 3 occurred after discharge, highlighting theimportance of the patient’s ability to be able to exchange thecontroller safely.

Figure 1 (A) Drive-line handling tool (showing flange on the left and locking springs within the slot). (B) Drive-line handling tool correctly posi-tioned adjacent to controller around the drive-line plug. (C) Detailed image of the patient with hand deformity using the tool to extract the drive-line.

The Journal of Heart and Lung Transplantation, Vol 31, No 12, December 20121324

The drive-line handling tool for the HVAD allowedimplantation of a VAD and safe discharge of a patient withpre-existing, severely impaired dexterity. The simple designand the low production cost of the tool make it a valuableasset for patients with physical disabilities. This tool mayparticularly valuable in long-term support therapy patientswho tend to be older and prone to arthritic joint disease.

Disclosure statement

The authors have no conflicts of interest to disclose. We aregrateful to David Smith of Placepower UK, Ltd., for assistancewith the design and manufacture of the handling tool, which cannow be obtained from HeartWare, Inc.

References

1. Wieselthaler GM, O’Driscoll G, Jansz P, et al. Initial clinical experiencewith a novel left ventricular assist device with a magnetically levitated rotorin a multi-institutional trial. J Heart Lung Transplant 2010;29:1218-25.

2. Available from: http://www.heartware.com.au/IRM/content/international/products_HVAD.html.

Treatment of hyperacute antibody-mediated lungallograft rejection with eculizumab

Kyle L Dawson, PharmD,a Amit Parulekar, MD,b andHarish Seethamraju, MDc

From the aDepartment of Pharmacy, The Methodist Hospital,Houston, Texas; bDepartment of Pulmonary and Critical CareMedicine, Baylor College of Medicine, Houston, Texas; and thecDepartment of Medicine, The Methodist Hospital, Houston, Texas.

We report the case of a highly sensitized (cPRA 99%,Class II) 56-year-old white female with a lung allocation scoreof 38, who underwent double lung transplantation for

pulmonary fibrosis and pulmonary hypertension. Pre-transplantdesensitization with multiple courses of plasma exchange (PE)and intravenous immunoglobulin (IVIg) was largely unsuc-cessful. The surgical operation was uneventful; however,the patient was found to have a positive prospectiveB-cell crossmatch according to both anti-human globulin-complement-dependent cytotoxicity and flow cytometry stu-dies. At the time of transplant, the recipient had the followingdonor-specific antibodies (DSAs): DR8 (15,000 MFI); DR14(14,000 MFI); DR52 (15,000 MFI); and DQ7 (3,800 MFI).

PE was performed on post-operative days (PODs) �1,0 and 1, and IVIg was given along with rituximab andbortezomib due to the positive B-cell crossmatch. RepeatDSAs post-implantation/PE were initially negative; how-ever, the patient subsequently developed acute graftdysfunction (Figure 1) and, by POD 2, the P/F ratio hadfallen from 106 to 33 and she was initiated on extracorpor-eal membrane oxygenation (ECMO), nitric oxide, multiplepressors and renal replacement therapy. An open-lungbiopsy was performed showing neutrophilic capillaritisand C4d positivity suggestive of antibody-mediated rejec-tion (AMR). An ensuing set of DSAs showed the antibodiesagainst DR8, DR14, DR52 and DQ7 were again present.

On POD 2, the decision was made to hold PE and givethe anti-C5 antibody, eculizumab, which dramatically haltedthe disease progression. Extracorporeal membrane oxygena-tion (ECMO) was discontinued after 3 days and the chestX-ray gradually improved. After the initial 1,200-mg dose,600 mg of eculizumab was again given on PODs 6, 8 and 9,for a cumulative dose of 3,000 mg, along with 4 doses ofbortezomib 1.3 mg/m2, 2 doses of rituximab 375 mg/m2,160 g of IVIg and 3 PEs post-transplant (Figure 2). Afterweaning off ventilator support and hemodialysis, the patientwas discharged on POD 47. The 1-month biopsy was 15%positive for C4d, and the 3-month biopsy was unremarkable.The patient is now alive and well at 1 year post-transplantwith no re-hospitalizations, infectious complications or

Figure 1 Post-operative chest X-rays.

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