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Case Reports

Prolonged Extracorporeal Membrane Oxygenation and Circulatory Support as Bridge to Lung Transplant

^a ECMO Department, Karolinska University Hospital, Stockholm, Sweden
^b Department of Pediatric Surgery, Karolinska University Hospital, Stockholm, Sweden
^c Department of Cardiothoracic Surgery, Sahlgrens University Hospital, Gothenburg, Sweden

Accepted for publication March 26, 2008.

^_* Address correspondence to Dr Broomé, ECMO Department, Karolinska University Hospital, Stockholm, 171 76, Sweden (Email: michael.broome{at}karolinska.se).

	Abstract

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A 38-year-old man with progressive alveolitis secondary to polymyositis was treated for 52 days with venovenous and venoarterial extracorporeal membrane oxygenation as a bridge to bilateral lung transplantation. The patient survived, despite multiple complications, and is now back home with good pulmonary function. He is working part-time nearly 3 years post-transplant. This case shows that long-term extracorporeal lung assist is a viable but demanding alternative for bridging patients to pulmonary transplantation. This case also shows that right ventricular failure necessating conversion to veno-arterial assist does not necessarily predict right ventricular failure post-transplant.

	Introduction

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The majority of pulmonary transplants are performed in patients with slowly progressive pulmonary disease, such as chronic obstructive pulmonary disease, idiopathic fibrosis, cystic fibrosis, and primary pulmonary hypertension [1]. Therefore, lung transplantation is seldom an emergency procedure. Although extracorporeal lung assist for short-term use is technically possible, it is very seldom used for bridging patients to lung transplantation [2, 3]. We describe a case in which a middle-aged man with progressive alveolitis secondary to polymyositis was treated for 52 days with venovenous and venoarterial extracorporeal membrane oxygenation as a bridge to successful bilateral lung transplantation.

A previously healthy 38-year-old man had polyarthritis, muscle pain, and dermatitis develop for a few months that was compatible with systemic rheumatologic disease. Based on serological antibody tests (ie, Pos ANA, Pos SS-A, Neg Jo-1) and dermatological changes, the disease was classified as belonging to the dermatomyositis family [4]. Treatment with steroids and cyclophosphamide was started when dyspnea and roentgenogram indicated that signs of diffuse alveolitis had evolved. After 1 month of treatment the patient deteriorated in a septic state without positive blood cultures. The patient was awake and treated with noninvasive mask ventilation in an intensive care unit. The roentgonogram showed mediastinal air and pneumomediastinum, and the patient continued to deteriorate (Fig 1). Although some reversibility of the acute respiratory insufficiency was expected with sepsis treatment, the long-term prognosis for the patient's alveolitis was considered poor, because no effect of treatment with steroids and cyclophosphamide was seen [4].

View larger version (99K): [in this window] [in a new window]   Fig 1. Pulmonary roentgenogram on day 0 before cannulation.

The septic state of the patient resolved, and the general state of the patient improved during the next few days. The lungs continued to deteriorate the following week, despite continued treatment with steroids, cyclophosphamide, and immunoglobulins. Therefore, the immunosuppressive treatment was withdrawn to avoid life-threatening infections while waiting for donor organs, because lung transplantation was considered the only realistic long-term treatment option. Although the patient occasionally suffered from muscle soreness, no sure signs of the patient's systemic inflammatory disease were seen.

The patient suffered from septic episodes on days 24 to 27, days 34 to 37, and from day 43 until transplantation on day 52. Repeated cultivations from blood, lungs, and cannulation sites during these secondary septic episodes showed growth of hemophilus influenza, coagulase negative staphylococci, Enterococcus species, Enterobacter species, Klebsiella, Proteus, Clostridium, and Escherichia coli. Cytomegalovirus, candidosis, and aspergillosis were also found and treated, but the clinical significance of these positive cultures and serological tests was difficult to assess.

Conversion to venoarterial bypass was performed on day 38 because of right ventricular failure secondary to high pulmonary vascular resistance and pulmonary hypertension in the range of 70 to 100 mm Hg (estimated with continuous Doppler echocardiography). The right femoral artery was used to access the arterial system. The patient was also treated during less than a week from day 42 on with pericardial drainage because of plasma-like effusions with hemodynamic consequences and a self-limiting bleeding complicating pericardial puncture.

The patient was kept awake most of the time and was well informed about transplantation being the only long-term treatment option. He was well motivated for continued treatment and took an active part in the discussions regarding his treatment. The patient was fed enterally during the main part of the treatment. The support (including the delivery of the patient's favorite foods from his relatives) was also considered an important part in keeping the patient in a reasonably good mood.

A size and blood group compatible donor was found on day 52. A decision was taken to accept the patient for transplantation, despite a septic state (C-reactive protein, 321 mg/L) and lack of a definitive cross-matching test. A 400-km transport to the transplant center was arranged with the help of an intensive care ambulance loaded into a military cargo plane.

The patient was taken directly to the operating room, and a successful double-lung transplant was performed through a sternotomy despite some surgical difficulties due to pulmonary adhesions and inflammatory changes (Fig 2).

View larger version (95K): [in this window] [in a new window]   Fig 2. Pulmonary roentgenogram on day 52 before transplantation.

After 54 days in the intensive care unit, including 41 days on the ventilator, the patient improved and was finally discharged from the hospital post-transplant day 124. He is now doing well without any rejection or infection episodes for nearly 3 years post-transplant. His pulmonary function test 2 years post-transplant showed a forced expiratory volume in 1 second of 1.5 L (39% of predicted) and forced vital capacity of 2.8 L (60% of predicted). His main concern is a partial peroneal paralysis, some residual generalized weakness, and peripheral numbness, probably caused by a polyneuropathy of unknown origin, but he is continuously improving.

	Comment

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Prolonged extracorporeal lung assist as a bridge to lung transplantation was successful in this particular case, but the story raises many questions. The worldwide lack of donor organs makes prioritization among the possible recipients crucial. Progressive alveolitis and fibrosis secondary to systemic inflammatory autoimmune disease is an uncommon indication for lung transplantation with variable results [1], although anti-rejection immunosuppressive treatment (including tacrolimus) may be close to an optimal treatment for the autoimmune disease as well [4].

Our patient had to accept an extremely long waiting time for his donor organs, despite high priority, optimal size, and blood group. If bridging patients to lung transplantation with extracorporeal circulation becomes an established treatment option a system with "urgent calls" for donor organs will be needed. New development in the field of donor organ evaluation and preservation [5] also means a hope for better organ availability in the future.

The mental pressure put on both the awake patient and the caretakers was extreme. It is sometimes tempting to anesthetize the patient continuously during extracorporeal life support, but in our experience this increases the risk of complications, such as infections, circulatory instability, and fluid retention, and should therefore be avoided. Morphine, midazolam, and dexmedetomidine were used for "awake sedation" to minimize pain and anxiety. The patient was also treated with anti-depressives (ie, selective serotonin reuptake inhibitors) prophylactically, and he required a low dose of neuroleptics during the last 2 weeks to minimize vivid hallucinations. The patient was also able to talk a little during the most critical days with help of a titrated air leak in the tracheostomy, despite tidal volumes below 100 mL (Fig 2). He was also mentally clear enough to use a laptop and created a web page describing his disease, which was updated with pictures and text after more than 45 days on extracorporeal support. We believe that successful "awake sedation" was a cornerstone in our therapeutic efforts during "endless" waiting for donor organs.

We conclude that extracorporeal membrane oxygenation as a long-term bridge to lung transplant is possible. We also conclude that a successful outcome is possible, despite the need for conversion to veno-arterial bypass because of right heart failure. We believe it is adequate to bridge selected cases to lung transplant with extracorporeal lung assist.

	References

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1. Orens JB, Estenne M, Arcasoy S, et al. International guidelines for the selection of lung transplant candidates: 2006 update—a consensus report from the Pulmonary Scientific Council of the International Society for Heart and Lung Transplantation J Heart Lung Transplant 2006;25:745-755.[Medline]
2. Zwischenberger JB, Alpard SK. Artificial lungs: a new inspiration Perfusion 2002;17:253-268.[Abstract/Free Full Text]
3. Kopp R, Dembinski R, Kuhlen R. Role of extracorporeal lung assist in the treatment of acute respiratory failure Minerva Anestesiol 2006;72:587-595.[Medline]
4. Fathi M, Lundberg IE. Interstitial lung disease in polymyositis and dermatomyositis Curr Opin Rheumatol 2005;17:701-706.[Medline]
5. Wierup P, Haraldsson A, Nilsson F, et al. Ex vivo evaluation of nonacceptable donor lungs Ann Thorac Surg 2006;81:460-466.[Abstract/Free Full Text]

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This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Folke Nilsson Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Broomé, M. Articles by Nilsson, F. Search for Related Content PubMed PubMed Citation Articles by Broomé, M. Articles by Nilsson, F. Related Collections Extracorporeal circulation