Protein Losing Enteropathy - Clinical Resolution Post Heart Transplant and Recurrence after CMV Enteritis

Abstract

Introduction Protein losing enteropathy (PLE) is one of the main reasons for heart transplant referral in patients with a Fontan circulation PLE develops in 1-10% of Fontan patients and has a 5 year mortality rate as high as 50%. The largest contemporary cohort of Fontan patients with PLE who underwent heart transplantation, showed a 1 year survival rate of 83%. Of those that survived, 98% of patients had resolution of PLE. Case Report A 25 year old woman underwent orthotopic heart transplantation in May 2019 for advanced heart failure secondary to a failing Fontan Circulation and PLE. Biweekly paracentesis was used pre-transplant for the management of refractory ascites. Post-transplant, her ascites improved and as of Feb 2020, she no longer required paracentesis. In April 2020, prednisone was discontinued (lack of rejection). In June 2020, she developed CMV enteritis (PCR of 1.2\u202f×\u202f106 IU/mL from previous negative). Abdominal ultrasound showed recurrence of large volume ascites. She received IV ganciclovir. Fibroscan liver stiffness was 9 kPa (vs 21 kPa pre-transplant), excluding cirrhosis. Stool alpha-1-antitrypsin clearance/24 hours was 268mL, consistent with PLE. Paracentesis (Aug 2020) showed: Ascites albumin 6, Serum albumin 22, SAAG of 1.6g/dL (vs 1.2g/dL pre-transplant), consistent with elevated portal pressures. Prednisone (5mg) and spironolactone (25mg) were restarted, and furosemide (160mg) was up-titrated, with signs of clinical improvement. Summary To the authors knowledge, this is the first reported case of recurrent PLE post heart transplantation in the context of CMV-enteritis. Two major mechanisms are hypothesized: invasion and injury of the endothelial cells by CMV plus an increase in vascular permeability. The patient s ascites is improving with prednisone, spironolactone and furosemide. The authors hypothesize that this recurrence of PLE will resolve given treatment of the underlying precipitant (CMV) and the high rates of resolution described in the literature.