Protein-Losing Enteropathy and Plastic Bronchitis After the Fontan Operation

Area of Science:

• Cardiology
• Pediatric Cardiology
• Critical Care Medicine

Background:

• Protein-losing enteropathy (PLE) and plastic bronchitis (PB) are serious complications following the Fontan procedure.
• These conditions are associated with single-ventricle physiology and Fontan circulation, leading to increased morbidity and mortality.
• Despite advances, treatment remains challenging.

Purpose of the Study:

• To review the pathophysiology of PLE and PB in the context of Fontan physiology.
• To discuss current medical and surgical management strategies for these conditions.
• To highlight risk factors and potential interventions for patients with single-ventricle physiology.

Main Methods:

• Literature review of studies on PLE and PB in Fontan patients.
• Analysis of hemodynamic alterations contributing to these complications.
• Synthesis of information on diagnostic criteria and treatment outcomes.

Main Results:

• Fontan physiology leads to chronically low cardiac output, elevated central venous pressure, and congestive heart failure, contributing to PLE.
• Altered hemodynamics increase mesenteric vascular resistance, causing venous hypertension and gut congestion in PLE.
• High lymphatic pressures in Fontan circulation can lead to acellular bronchial cast formation in PB.

Conclusions:

• PLE and PB are significant challenges in Fontan survivors, linked to altered hemodynamics.
• Understanding the pathophysiology is crucial for effective management.
• A multidisciplinary approach involving medical and surgical interventions is necessary for optimal patient outcomes.