Contemporary Management of the Failing Fontan.

Area of Science:

• Cardiology
• Adult Congenital Heart Disease
• Cardiovascular Physiology

Background:

• Adult congenital heart disease (ACHD) patient numbers now exceed pediatric cases due to surgical advances and better survival rates.
• Fontan circulation is a complex congenital heart defect frequently seen in the adult ACHD population.
• Patients with Fontan circulation exhibit unique physiology, predisposing them to numerous complications.

Purpose of the Study:

• To review the common clinical presentations (phenotypes) of Fontan circulatory failure.
• To discuss contemporary management strategies for these Fontan failure phenotypes.
• To provide an updated overview for clinicians managing adult Fontan patients.

Main Methods:

• Literature review of contemporary studies and clinical guidelines.
• Synthesis of information on Fontan circulatory failure phenotypes.
• Analysis of current treatment approaches for Fontan-associated complications.

Main Results:

• Common phenotypes of Fontan circulatory failure include thrombosis, pulmonary vascular disease, heart failure, arrhythmias, valve regurgitation, and protein-losing enteropathy.
• Management strategies are tailored to specific phenotypes, involving medical, interventional, and surgical options.
• Advances in care have improved survival but necessitate ongoing vigilance for complications.

Conclusions:

• Fontan circulatory failure presents diverse phenotypes requiring specialized management in adult ACHD.
• Contemporary strategies focus on early recognition and multidisciplinary care to mitigate Fontan-associated risks.
• Continued research and clinical updates are crucial for optimizing long-term outcomes in Fontan survivors.