Our case report describes a 32-year-old woman diagnosed with lymphangioleiomyomatosis who was placed on an organ transplant waiting list in February 2014, due to rapidly progressing respiratory insufficiency. Her history was significant for multiple lymphangioleiomyomas in the retroperitonium along with repeated spontaneous pneumothoraces that required drainage. Transesophageal echocardiography (TEE) was performed during preoperative assessment and the patient was found to have a low ejection fraction. In March 2014, the patient was intubated and ventilated due to respiratory failure. She went on to develop bronchopneumonia caused by multiresistant Pseudomonas aeruginosa, followed by sepsis and disseminated intravascular coagulopathy with severe bleeding from the nose, hypopharynx, and lower respiratory tract. At this point, mechanical ventilation was no longer sufficient to sustain oxygenation and venovenous ECMO (VV-ECMO) was commenced, as a bridge to urgent lung transplantation (Figure 1). Six days later a donor was found and sequential bilateral lung transplant with a clamshell incision was performed with the support of the arteriovenous ECMO (AV-ECMO). The cannulae from the VV-ECMO were left in place and an arterial cannula was inserted centrally into the ascending aorta. Cannulation of the vena jugularis interna dextra was terminated. Due to anatomic disproportion, the donor lungs were partially resected at the right middle lobe and lingula.
Perioperative TEE showed severely hypokinetic right and left ventricles along with a large thrombus in the left atrium near the pulmonary veins (Figure 2). The procedure was complicated by diffuse bleeding due to coagulopathy present in the septic state, which resulted in a 10 L blood loss. Postoperatively, the AV-ECMO was left in place due to the poor myocardial function; therefore, the sternotomy was not closed to allow the arterial cannula to remain in the aorta. During admission to the intensive care unit, severe pulmonary edema was noted with the continuous leakage of edematous fluid from the endotracheal tube. Tidal volumes were 30-40 mL with a peak pressure of 30 cm H2O and a positive end-expiratory pressure of 10 cm H2O. Cardiovascular support with high-dose norepinephrine (>1 microg/kg/min) was in place. Arterial blood gas results showed good oxygenation due to the support of AV-ECMO. Immunosuppression therapy was commenced with tacrolimus, mycophenolate mofetil, and corticosteroids.
Anticoagulation with heparin was initiated with a target of activated coagulation time (ACT) 150-200 seconds. Six hours later, a TEE was performed and thrombi were noted in both the left and right atria. Due to the high risk of embolism, we were faced with making a crucial decision: to start thrombolysis, which is generally contraindicated in immediate postoperative period, or not. We evaluated that the risk of massive embolism was superior to the risk of bleeding when the sternotomy was not closed. The primary thrombolysis was undertaken with 25 mg alteplase given over 2 hours into the pulmonary artery as the rescue procedure declared in medical documentation. The left atrial thrombus was quickly reduced, after which a second identical dose of alteplase was given. Pulmonary edema began to resolve after the completion of thrombolysis. A further TEE showed no thrombi in the left ventricle or pulmonary veins, although small thrombi remained in the right and left atria. The following day a full dose of thrombolytic agent was given - 50 mg alteplase over 2 hours intravenously - after which no further thrombi could be detected on TEE (Figure 3). Lung function continued to improve along with radiological findings within 1 week following the thrombolysis (Figure 4).
In the following days, the patient developed acute kidney injury for which she was placed on continuous renal replacement therapy. Gradually, her heart and lung function improved and on postoperative day 8, ECMO support was stopped. The sternotomy was closed after resolution of the P. aeruginosa infection. Weaning and rehabilitation were complicated by poor muscle strength. Due to worsening lung function, a transbronchial biopsy was performed which confirmed tissue rejection A 1-2 (histologic grading of lung allograft rejection- the Classification of Pulmonary Allograft Rejection: Lung Rejection Study Group) and was successfully treated with pulses of steroids. The following days were significant for the loss of consciousness that was thought to be caused by posterior reversible encephalopathy syndrome. An electroencephalograph showed an epileptic status, and antiepileptic medications were prescribed. Three months postoperatively, stenosis of the right main bronchus was diagnosed bronchoscopically, and a biodegradable stent ELLA  (ELLA-CS s.r.o. Milady Horakove, Hradec Kralove, Czech Republic) was implanted. Another setback of pulmogenic sepsis and multiorgan failure, with a need for continuous renal replacement therapy occurred. Six months postoperatively, the patient was extubated and began to actively rehabilitate. Seven months postoperatively, the patient was discharged to home. Currently, she enjoys a good quality of life without any signs of stenosis of the upper respiratory tract and with stationary spirometric values (last forced expiratory volume in 1 second [FEV1] 38%) (Figure 5).