Protein Losing Enteropathy in a Post-Cardiac Transplant Adult: A Case Report

Introduction Protein-losing enteropathy (PLE) is a clinical syndrome that can be associated with a number of different pathologies, including congenital heart disease (CHD). However, typically PLE associated with CHD resolves following orthotopic heart transplant (OHT). We report a case of PLE in an adult CHD patient that developed many years following successful OHT. Case report A 32 year old man presented to transplant clinic with anasarca and mild dyspnea on exertion, and was subsequently admitted to hospital. He was born with transposition of the great arteries (TGA) and ultimately underwent cardiac transplantation at age 16. Overall, based on his follow up transplant clinic visits, he had been relatively well since OHT. He had been compliant with his anti-rejection medications. However, over the past 3 years his renal function has gradually declined in spite of careful medication titration. Furthermore, he continues to have a high salt diet, in spite of dietary recommendations. Given his presenting symptoms as well as a significantly elevated NT-pro BNP level, he was initially treated as heart failure due to possible chronic cardiac transplant rejection. He received aggressive intravenous diuresis. However, this only resulted in modest symptomatic improvement, and a rapid decline in his renal function was noted. His echocardiogram at this point showed preserved LV systolic function, and his coronary angiogram was normal. In addition, his cardiac biopsy showed no evidence of rejection. Therefore, other etiologies were sought. While he did have hypoalbuminemia, his renal work up showed no evidence of nephrotic syndrome. In addition, his liver ultrasound as well as other hepatic function tests was unremarkable. Ultimately, a gastroenterology workup for possible PLE showed abnormally elevated active alpha 1 antitrypsin level in his stool, in keeping with PLE. Conclusion This is the first case of PLE post cardiac transplant in an adult CHD patient. The two previous reports in the literature of PLE following cardiac transplant are both in children with hypoplastic heart syndrome and severe tricuspid regurgitation following transplant. As our case illustrates, in spite of the patientu0027s strong cardiac history, renal dysfunction, and a clinical presentation very similar to either heart failure or nephrotic syndrome, PLE can be a rare but important cause of anasarca in this patient population.