Insights into Heart Transplantation for Protein Losing Enteropathy: A 24 Year Experience

Abstract

Purpose Protein losing enteropathy(PLE) is an ominous complication of Fontan palliation. Heart transplant(Htx) for PLE is associated with high mortality from infection and bleeding; yet, rejection is rarely reported. We hypothesize that there is increased risk of rejection due to prior allosensitization. Methods and Materials We reviewed the clinical characteristics and outcomes for HTx at our center from 5/1988 to 10/2012 for failed Fontan indication and identified subjects with PLE. We performed descriptive stats and generated K-M survival curves. Results Of 190 Htx recipients, 21 pts(11%) had Htx for failed Fontan -14 had PLE, 6 had ventricular dysfunction, and 1 had plastic bronchitis. Median duration of symptoms of PLE prior to Htx was 21 months (range 6-84). Attempted PLE therapy included steroids in 9, sildenafil in 5, heparin in 3, and diuretics in all. Median age at Htx for PLE was 12 yrs(range 4-39). At listing,13/14 were status 1A. Despite hypogammaglobulinemia and lymphopenia, 10/14(71%) had allosensitization (median PRA of 64% for class I and 36% for class II). Crossmatch was positive in 4. At first biopsy, 4 had AMR with elevated donor specific antibodies (1 with pAMR1 and 3 with pAMR2) requiring plasmapheresis. There were 7 episodes of grade 2 cellular rejection. PLE resolved as evidenced by serum albumin median of 3.3 at 1 month and 3.8 at 3 months. Thirty day,1, and 5 year survival were 79%,71% and 53% respectively. There were 4 early deaths(1 early graft failure and 3 infections). Risk factors for early mortality include infection, older recipient age, longer duration of PLE, and allosensitization.There was one late death from allograft vasculopathy. Conclusions Resolution of PLE occurs rapidly after HTx. Highest mortality in patients occurred early from infection; however, rejection was also frequent, likely related to prior HLA sensitization.With earlier referral for Htx and careful immune monitoring with serial C3D and donor specific antibody testing, outcomes in this challenging cohort may improve.