Physiology and Functional Pathology of the Lymphatic System with Implications for the Fontan Patient

Area of Science:

• Lymphology
• Cardiovascular Science
• Physiology

Background:

• Collecting lymphatic vessels rely on smooth muscle cells, valves, surrounding tissues, and nervous system regulation for contractile function.
• The Fontan procedure is a surgical intervention for complex congenital heart disease.

Purpose of the Study:

• To describe the potentially deleterious effects of the Fontan procedure on lymphatic vessel function.
• To highlight the link between elevated central venous pressure and lymphatic dysfunction.
• To discuss life-threatening complications arising from impaired lymphatic function post-Fontan.

Main Methods:

• Review of existing literature and clinical observations regarding lymphatic function and the Fontan procedure.
• Analysis of the physiological impact of elevated central venous pressure on lymphatic transport.
• Synthesis of data presented at the 2023 ISL International Congress of Lymphology.

Main Results:

• Elevated central venous pressure, a consequence of the Fontan procedure, can impair the contractile function of collecting lymphatic vessels.
• This impairment can disrupt lymphatic fluid transport and immune cell trafficking.
• Potential for severe complications, including fluid overload and organ dysfunction, exists.

Conclusions:

• The Fontan procedure poses risks to lymphatic system integrity and function.
• Monitoring and understanding lymphatic function are crucial in managing Fontan patients.
• Further research into mitigating these risks is warranted.