Challenges of management and therapy in patients with a functionally single ventricle after Fontan operation

Area of Science:

• Cardiology
• Pediatric Cardiac Surgery
• Vascular Surgery

Background:

• The Fontan procedure, first performed 40 years ago, addresses functionally single ventricle defects by connecting systemic venous circulation to pulmonary arteries.
• This palliative surgery creates a passive pulmonary circulation, with the single ventricle pumping only to the systemic circulation.
• Modifications over time have aimed to improve outcomes, yet fundamental hemodynamic challenges persist.

Purpose of the Study:

• To review the Fontan procedure's history, hemodynamic principles, and associated complications.
• To highlight the palliative nature of the Fontan operation and its long-term management challenges.
• To emphasize the need for specialized cardiology knowledge to manage Fontan survivors.

Main Methods:

• Review of historical surgical techniques and modifications.
• Analysis of Fontan-associated hemodynamic alterations and their consequences.
• Compilation of common clinical complications observed in Fontan patients.

Main Results:

• The Fontan procedure, while life-saving, results in non-physiological hemodynamics leading to numerous complications.
• Observed complications include arrhythmias, thromboemboli, hepatic dysfunction, protein-losing enteropathy, heart failure, cyanosis, and vascular abnormalities.
• Despite palliative limitations, the procedure allows many patients to reach adulthood.

Conclusions:

• The Fontan operation remains a palliative measure, with heart transplantation sometimes being the ultimate solution.
• Pharmacological and surgical conversion therapies often prove ineffective for managing complications.
• Managing Fontan survivors presents a significant challenge, necessitating advanced expertise in cardiology to optimize long-term outcomes.