# An Uncommon Finding in an Adult: A Case Report of Hypoplastic Left Heart Syndrome and Protein-Losing Enteropathy

## TL;DR

This case report describes a rare condition in an adult with heart syndrome and a rare gut disorder, highlighting the importance of proper diagnosis and treatment.

## Contribution

The novelty lies in reporting a post-Fontan HLHS case with PLE in an adult, emphasizing awareness and management for refractory hypoproteinemia.

## Key findings

- HLHS with PLE was managed post-Fontan procedure in an adult.
- The case highlights the need for awareness in managing refractory hypoproteinemia.
- Proper clinical management based on literature is emphasized for such rare cases.

## Abstract

Protein-losing enteropathy (PLE) is a rare disorder with diverse causes, but the treatments are limited and understudied. It is often associated with significant mortality and morbidity. The survival of hypoplastic left heart syndrome (HLHS) in infants without any intervention is usually 4.5 days, and 30-day mortality is 95%. However, with surgical intervention, survival at 20 years is 80%. HLHS can lead to protein-leading enteropathy and is corrected by the three-step procedures (Norwood, Glenn, and Fontan) during infancy. We report a case of Fontan procedure postoperative HLHS associated with PLE and describe its clinical course and outcome. The main intention of reporting this case is to provide awareness among physicians while dealing with refractory cases of hypoproteinemia and appropriate management based on the literature.