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A 17-year-old previously healthy Taiwanese girl presented with syncope on a background of 1 month of weakness, polyarthritis, and heliotrope rash. Her presentation and investigations, which included an elevated creatine phosphokinase level and diffuse myositis on lower limb MRI, were consistent with juvenile dermatomyositis (JDM).

Case Presentation A 24-year-old woman with ΔF508/Y1092X cystic fibrosis (CF) complicated by severe obstructive lung disease (FEV1 of 30% predicted) was admitted for IV antibiotics for planned sinus surgery resulting from severe chronic sinusitis causing frequent exacerbations and declining lung function. She had persistent airway infection with multidrug-resistant Pseudomonas aeruginosa, methicillin-resistant Staphylococcus aureus, and growth of a fungus presumed to be an airway colonizer, identified as Stephanoascus ciferrii 1 year before presentation. Two days after surgery, she developed acute respiratory failure requiring mechanical ventilation. On day 4 of mechanical ventilation, venovenous-extracorporeal membrane oxygenation (VV-ECMO) was initiated for refractory respiratory failure. The following day, she was listed for bilateral lung transplant and was transplanted 4 days later. Following transplantation, she was decannulated from ECMO; however, over the next 12 hours, oxygenation deteriorated requiring reinstitution of VV-ECMO for presumed severe primary graft dysfunction. Despite treatment with broad spectrum antimicrobial coverage with piperacillin/tazobactam, ciprofloxacin, linezolid, micafungin, voriconazole, and ganciclovir, she failed to improve and developed complex bilateral pleural effusions.

TOPIC: Transplantation TYPE: Fellow Case Reports INTRODUCTION: SARS-CoV2, commonly known as COVID-19, has been noted to manifest severe illness in certain patient populations. The clinical manifestations of this coronavirus in solid organ transplant recipients is just beginning to be described, and is not well understood. SARS-CoV2 and associated illness have been described in multiple patient populations, so far limited data has been published on the course of illness in lung transplant recipients. Here we present a case series of 26 patients who were unfortunately infected with SARS-CoV2. CASE PRESENTATION: We conducted a single-center, retrospective review of 26 lung transplant recipients infected with SARS-CoV2. Data collection and patient consent were covered by OSU IRB protocol. DISCUSSION: 15 of these patients (55.5%) required admission for hypoxemia, and 4 required intubation and mechanical ventilation. 1 patient required extracorporeal membrane oxygenation (ECMO). 46% (12/26) patients received dexamethasone and remdesivir, 27% (7/26) received convalescent plasma, 1 received hydroxychloroquine, and 2 received azithromycin as part of their treatments. Prior to COVID-19 infection all patients but 1 were on a standard triple immune suppression regimen with calcineurin inhibitors, steroids, and cell cycle inhibitors. Calcineurin dosing was decreased in 2 patients with severe COVID-19 and cell cycle inhibitors were reduced by 50% or held for 2 weeks 50% patients. 1 patient succumbed to COVID-19 ARDS despite ECLS support and a second 3 months after initial diagnosis due to a massive CVA. Mortality 3 months after initial diagnosis was 7.6% which is lower than other reported series. The average change in FEV1 was a loss of 0.52 liters. An average loss of 0.69 liters of FVC was noted in survivors. CONCLUSIONS: To our knowledge this is one of the largest currently reported case series of lung transplant recipients with SARS-CoV2. Overall, mortality was higher in this group than in the general population but at 7.6% 3 months after diagnosis is lowest of the single center reports published. As expected, a decrease in lung function was noted in survivors. Three was no general difference in lung function in patients who received certain treatments over others. REFERENCE #1: Tsuang WM, Budev MM. COVID-19 and lung transplant patients. Cleve Clin J Med. 2020. REFERENCE #2: Aigner C, Dittmer U, Kamler M, Collaud S, Taube C. COVID-19 in a lung transplant recipient. J Heart Lung Transplant. 2020;39(6):610-611. REFERENCE #3: Keller BC, Le A, Sobhanie M, et al. Early COVID-19 infection after lung transplantation. Am J Transplant. 2020;20(10):2923-2927. DISCLOSURES: No relevant relationships by Mena Botros, source=Web Response No relevant relationships by Molly Howsare, source=Web Response

TOPIC: Critical Care TYPE: Medical Student/Resident Case Reports INTRODUCTION: ECMO has long been used in the ICU as a salvage or bridge therapy for selected ICU patients. There had been a variety of cannulas available in the market. We report 2 cases with positive outcomes associated with the use of jugular dual lumen cannula. CASE PRESENTATION: An obese 42-year-old obese female, with PMH of hypothyroidism and ulcerative colitis, was admitted for acute hypoxic respiratory failure due to COVID-19. Patient underwent conventional treatment at the time (dexamethasone, remdesivir, convalescent plasma and antibiotics) for COVID-19 but was ultimately intubated on day 8 due to profound hypoxia requiring VV ECMO on day 10. She remained on full ECMO support until day 56. Her FiO2 was eventually weaned down to 21% but she continued to require a sweep of 3L secondary to profound muscular weakness and obesity. After transitioning to the jugular dual lumen cannula on day 75 for long-term ECMO, her sedation was weaned down, that allowed participation of physical therapy. She was decannulated on day 94 and was discharged to LTAC on day 100. A 48-year-old female was tested positive for COVID-19 after being exposed 12 days before. Due to worsening symptoms, she eventually sought care at the hospital. Despite the standard treatment, her respiratory status continued to worsen. She was intubated on Day 2 before receiving full support on VV ECMO on day 6. Despite effort to wean down to FiO2 of 21%, she still required a sweep of 1L. After being transitioned to a jugular dual lumen cannula on day 71, she was weaned off of sedation and able to participate in therapy. She was eventually decannulated on day 79, and discharged to a rehab facility on day 85 to continue her recovery. DISCUSSION: Transition from a dual cannula ECMO system to a single cannula ECMO system allowed increased mobility and participation of physical therapy while in the ICU for patients requiring extended time on ECMO. Study has suggested a reduction of up to 35% in muscle mass among ECMO patients by day 20 of their cannulation. In an economic study performing on transplant patients, a 73% reduction in post-transplant ICU cost was reported for those who underwent rehabilitation while being supported on ECMO. Both studies demonstrated the potential benefits with promoting early rehabilitation for ECMO patients. Regarding the technique involved, there was a recent report of a similar transition without any ECMO interruption, allowing the patient uninterrupted time on ECMO while benefiting from early rehab. CONCLUSIONS: We present two cases of severe COVID-19 patients with an extended period of time on VV ECMO became severely debilitated. They were transitioned to a jugular dual lumen cannula, allowing early participation in rehab, resulting in their eventual discharge. These cases demonstrated these cannulas were valuable tool to reduce patients' reliance on support, before being transitioned off of ECMO. REFERENCE #1: Hayes K, Holland AE, Pellegrino VA, Mathur S, Hodgson CL. Acute skeletal muscle wasting and relation to physical function in patients requiring extracorporeal membrane oxygenation (ECMO). J Crit Care. 2018;48:1-8. doi:10.1016/j.jcrc.2018.08.002 REFERENCE #2: Bain JC, Turner DA, Rehder KJ, et al. Economic Outcomes of Extracorporeal Membrane Oxygenation With and Without Ambulation as a Bridge to Lung Transplantation. Respir Care. 2016;61(1):1-7. doi:10.4187/respcare.03729 REFERENCE #3: Chan EG, Chan PG, Harano T, Sanchez PG. Transition of femoral-jugular to dual-stage left subclavian without discontinuation of extracorporeal membrane oxygenation. J Card Surg. 2020;35(10):2794-2797. doi:10.1111/jocs.14881 DISCLOSURES: No relevant relationships by Vernon Chan, source=Web Response No relevant relationships by Marina Dolina, source=Web Response

Idiopathic inflammatory myopathies are autoimmune processes that are characterized by skeletal muscle inflammation. The lung is the most commonly involved extramuscular organ, and, when present, pulmonary disease drives morbidity and mortality. A subset of patients can present with rapidly progressive hypoxemic respiratory failure due to myositis-related interstitial lung disease. Confirmatory autoantibody testing requires sending samples to a reference laboratory; thus, diagnosis of rapidly progressive myositis-associated interstitial lung disease relies on a high index of suspicion and careful history and physical examination. Although the cornerstone of therapy for these patients remains multimodality immunosuppression, emerging data support a role for advanced therapies (including extracorporeal membrane oxygenation and lung transplantation) in appropriately selected patients. It is hoped that greater awareness of the clinical features of this syndrome will allow for appropriate diagnosis and treatment of these potentially treatable patients, as well as raise awareness of the need for multicenter collaboration to prospectively study how to manage this complex disease.

Lung transplantation (LTx) has become an accepted treatment for carefully selected patients with end-stage lung disease. Critical care issues have gained importance concerning bridging of candidates by mechanical respiratory support and are involved in the care after transplantation. The nature of respiratory support varies from oxygen supply and noninvasive ventilation, to mechanical respiratory support either by mechanical ventilation and/or extracorporeal life support. Recent innovations in extracorporeal life support technology have resulted in its more widespread use. Retrospective studies have demonstrated promising outcomes in candidates on mechanical respiratory support as a bridge to lung transplantation. The role of mechanical respiratory support has influenced the selection criteria for LTx, although bridging remains technically and ethically challenging. Critical care is integral to manage and prevent postoperative complications of LTx. Primary graft dysfunction and prolonged mechanical ventilation are major obstacles to hospital survival after LTx. Clear evidence is lacking on how to ventilate and optimally manage patients after LTx. Prolonged extracorporeal life support after LTx may improve outcome in selected patients with a primary graft dysfunction.

The proportion of critically ill patients awaiting lung transplantation has increased since the implementation of the Lung Allocation Score (LAS) in 2005. Critically ill patients comprise a sizable proportion of wait-list mortality and are known to experience increased posttransplant complications. These critically ill patients have been successfully bridged to lung transplantation with extracorporeal membrane oxygenation (ECMO), but historically these patients have required excessive sedation, been immobile, and have had difficult functional recovery in the posttransplant period and high mortality. One solution to the deconditioning often seen in critically ill patients is the implementation of rehabilitation and ambulation while awaiting transplantation on ECMO. Ambulatory ECMO programs of this nature have been developed in an attempt to provide rehabilitation, physical therapy, and minimization of sedation prior to lung transplantation to improve both surgical and posttransplant outcomes. Favorable outcomes have been reported using this novel approach, but how and where this strategy should be implemented remain unclear. In this commentary, we review the currently available literature for ambulation and rehabilitation during ECMO support as a bridge to lung transplantation, discuss future directions for this technology, and address the important issues of resource allocation and regionalization of care as they relate to ambulatory ECMO.

Background The use of extracorporeal membrane oxygenation (ECMO) as a bridge to lung transplant (LTX) is still being debated. Methods We performed a retrospective two-center analysis of the relationship between ECMO bridging duration and survival in 25 patients. Further survival analysis was obtained by dividing the patients according to waiting time on ECMO: up to 14 days (Early group) or longer (Late group). We also analyzed the impact of the ventilation strategy during ECMO bridging (ie, spontaneous breathing and noninvasive ventilation [NIV] or intubation and invasive mechanical ventilation [IMV]). Results Seventeen of 25 patients underwent a transplant (with a 76% 1-year survival), whereas eight patients died during bridging. In the 17 patients who underwent a transplant, mortality was positively related to waiting days until LTX (hazard ratio [HR], 1.12 per day; 95% CI, 1.02-1.23; P = .02), and the Early group showed better Kaplan-Meier curves (P = .02), higher 1-year survival rates (100% vs 50%, P = .03), and lower morbidity (days on IMV and length of stay in ICU and hospital). During the bridge to transplant, mortality increased steadily with time. Considering the overall outcome of the bridging program (25 patients), bridge duration adversely affected survival (HR, 1.06 per day; 95% CI, 1.01-1.11; P = .015) and 1-year survival (Early, 82% vs Late, 29%; P = .015). Morbidity indexes were lower in patients treated with NIV during the bridge. Conclusions The duration of the ECMO bridge is a relevant cofactor in the mortality and morbidity of critically ill patients awaiting organ allocation. The NIV strategy was associated with a less complicated clinical course after LTX.

The proportion of critically ill patients awaiting lung transplantation has increased since the implementation of the Lung Allocation Score (LAS) in 2005. Critically ill patients comprise a sizable proportion of wait-list mortality and are known to experience increased posttransplant complications. These critically ill patients have been successfully bridged to lung transplantation with extracorporeal membrane oxygenation (ECMO), but historically these patients have required excessive sedation, been immobile, and have had difficult functional recovery in the posttransplant period and high mortality. One solution to the deconditioning often seen in critically ill patients is the implementation of rehabilitation and ambulation while awaiting transplantation on ECMO. Ambulatory ECMO programs of this nature have been developed in an attempt to provide rehabilitation, physical therapy, and minimization of sedation prior to lung transplantation to improve both surgical and posttransplant outcomes. Favorable outcomes have been reported using this novel approach, but how and where this strategy should be implemented remain unclear. In this commentary, we review the currently available literature for ambulation and rehabilitation during ECMO support as a bridge to lung transplantation, discuss future directions for this technology, and address the important issues of resource allocation and regionalization of care as they relate to ambulatory ECMO.

49-year-old man with end-stage lung disease secondary to sarcoidosis had a past medical history significant for diabetes mellitus and Child A liver cirrhosis. He had successful preoperative evaluation for lung transplant 3 months before surgery. Intraoperatively, routine clamshell incision through the fourth intercostal space across the distal sternum was performed after complete general anesthesia and endotracheal intubation. Gross features of end-stage disease of both lungs due to sarcoidosis with mediastinal lymphadenopathy were evident. Right pneumonectomy was performed fi rst, and implantation of the cadaveric lung was performed after mobilization of the entire right lung. There was major discrepancy in the size of the donor and the recipient pulmonary arteries. Because of this, the vascular anastomosis was done between the pulmonary artery of the donor and the superior segmental branch of the right main pulmonary artery of the recipient. The anastomosis was carried out with 4-0 Prolene as a continuous suture, and the remaining part of the right main pulmonary artery was stapled. The right atrial cuff of the donor was anastomosed with the atrial cuff of the recipient using the 4-0 Prolene through everting mattress continuous suture, and two intercostal tubes were inserted. Because of the long duration of the right lung implantation, which was 10 h, the left lung transplantation was deferred because of prolonged ischemic time. Later, the patient had reperfusion, and the anterior chest wall incision

Severe pulmonary reimplantation response after lung transplantation is not very common, although the mortality can be high. We present a patient who developed an extremely severe reperfusion injury after bilateral lung transplantation. Because of severe hypoxia and hemodynamic instability, despite aggressive ventilator settings, venoarterial extracorporeal membrane oxygenation (ECMO) was instituted using the femoral approach at the bedside. During ECMO, the patient developed a thoracic wall hematoma that was treated with transfusion alone. After 50 h of ECMO, his chest radiograph had dramatically improved, his oxygen need had been reduced to 50%, and he was successfully weaned from ECMO. Two years later, he is doing extremely well. Therefore, institution of ECMO using the femoral approach can be performed safely at the bedside in the ICU, and can be lifesaving in the context of a very severe reimplantation response after lung transplantation.

Single lung transplantation (SLT) is now accepted therapy for selected cases of severe pulmonary hypertension. A recognized complication is the postoperative development of reperfusion edema in the graft, a potentially fatal cause of respiratory failure. Because reperfusion edema may be a reversible process, temporizing support measures can be life-saving. We report the case of a 48-year-old woman who developed severe reperfusion edema following right SLT for primary (unexplained) pulmonary hypertension. Extracorporeal membrane oxygenation (ECMO) was instituted. Independent lung ventilation was later begun and resulted in markedly improved oxygenation allowing withdrawal of ECMO. We conclude that reperfusion edema following SLT for pulmonary hypertension may be uniquely amenable to treatment with independent lung ventilation and ECMO if needed.