Combined Lung-Liver Transplantation Can Be Safely Performed in Patients with Hypokalemic Periodic Paralysis

Abstract

<h3>Introduction</h3> Combined lung-liver transplantation (CLLT) is rare and accounts for <0.01% of multiorgan transplants performed. Due to its infrequency, the criteria for CLLT candidacy have not been clearly elucidated. However, since post-transplant rehabilitation is critical for a successful outcome, any condition that may compromise a patient's postoperative mobility is considered a potential contraindication to transplantation. We report our experience with CLLT in a patient who had hypokalemic periodic paralysis (HPP), a rare genetic disorder that affects muscle ion channels and manifests as hypokalemia with episodes of severe weakness or paralysis. To our knowledge, we present the first reported case of transplantation in a HPP patient. <h3>Case Report</h3> The patient was a 63-year-old male with 7-year history of idiopathic pulmonary fibrosis and cryptogenic cirrhosis. Other comorbidities included HPP since childhood. His HPP exacerbations, which manifested as weakness without respiratory symptoms, were precipitated by various medications and were treated with correction of hypokalemia. He was previously declined for CLLT by 9 other programs due to his age and HPP, but our multidisciplinary team determined that the patient's HPP could be adequately managed and should not preclude him from CLLT. While hospitalized for progressive respiratory failure, donor organs became available. Bilateral lungs and a liver were recovered from a brain-dead donor. Bilateral lung transplantation was performed followed by orthotopic liver transplantation. There were no intraoperative complications. Standard post-transplant care was initiated after surgery. He was transferred out of the ICU on postoperative day (POD) #5, and was discharged to home on POD #22. The patient's postoperative hospitalization was complicated by antibody-mediated rejection (AMR) from <i>de novo</i> donor-specific antibodies. His AMR was adequately managed with methylprednisone, plasmapheresis, and intravenous immunoglobulins. Meticulously maintaining serum potassium levels above 4 mEq/L and avoiding precipitating medications sufficiently prevented any HPP exacerbations. The patient has been home for 8 months and has been doing well. <h3>Summary</h3> The limits on candidacy for CLLT have not been clearly defined, but we demonstrated for the first time that CLLT can be safely performed in patients with HPP.