Fontan Procedure: Advanced Surgery for Single-Ventricle Defects

Introduction & Overview

The Fontan Procedure is a life-saving heart surgery for patients with single-ventricle defects, such as Tricuspid Atresia, Hypoplastic Left Heart Syndrome (HLHS), and Double Inlet Left Ventricle. These conditions leave the heart unable to pump blood effectively through both circulations.

The Fontan Procedure redirects venous blood directly to the pulmonary arteries, bypassing the right ventricle, allowing oxygen-poor blood to reach the lungs without passing through a nonfunctional ventricle.

This surgery is often the final stage of a multi-step surgical approach (typically after Norwood and Glenn procedures), performed between ages 2-5.

Types of Defects Treated

✔ Tricuspid Atresia

✔ Hypoplastic Left Heart Syndrome (HLHS)

✔ Double Inlet Left Ventricle

✔ Pulmonary Atresia with Intact Ventricular Septum

✔ Single Ventricle Physiology

Biological & Medical Insights

✔ The procedure aims to optimize oxygen delivery while minimizing strain on the single functioning ventricle.

✔ Long-term effects include increased venous pressure, which can impact liver and kidney function, necessitating careful monitoring.

Procedure: Step-by-Step

• Pre-Operative Evaluation:
  • Cardiac MRI, echocardiogram, and catheterization.
  • Liver function tests (Fontan-associated liver disease is a risk).
• Surgical Process:
  • Connection of the inferior vena cava directly to the pulmonary arteries.
  • Use of a lateral tunnel or extracardiac conduit for blood flow.
  • Often includes a fenestration (small hole) to reduce pressure build-up.
• Post-Operative Care:
  • ICU monitoring, anticoagulation therapy, and fluid management.
  • Long-term surveillance for arrhythmias and protein-losing enteropathy.

Before & After Treatment

Before: Cyanosis, fatigue, failure to thrive.

After: Improved oxygen levels, increased activity tolerance, and extended life expectancy.

Advantages of Fontan Procedure

✔ Enables near-normal oxygenation.

✔ Improves quality of life for single-ventricle patients.

✔ Delays or eliminates the need for heart transplantation.

Precautions & Risks

✔ Risk of arrhythmias: Post-surgery patients may require pacemaker implantation.

✔ Fontan-associated liver disease (FALD): Increased venous pressure may cause progressive liver fibrosis.

✔ Long-term risk of heart failure: Some patients may eventually require a transplant.

Insights

✔ Emerging research on Fenestrated Fontan shows benefits in reducing pressure buildup.

✔ Liver fibrosis is a known complication; ongoing research focuses on minimizing this long-term risk.

Why Choose MediHelp for Fontan Procedures?

✔ Experienced Congenital Heart Team: Leading pediatric surgeons with expertise in single-ventricle physiology.

✔ Comprehensive Post-Op Monitoring: Long-term surveillance protocols for complications like FALD.

✔ Family-Centered Care: Education and support for families navigating complex congenital defects.