FONTAN FAILURE

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Fontan failure management refers to the medical strategies used to address complications in patients who have undergone the Fontan procedure—a surgical approach used to treat complex congenital heart defects where only one functional ventricle is present. Over time, many patients experience Fontan failure, which can manifest as heart failure, arrhythmias, liver dysfunction, protein-losing enteropathy, or plastic bronchitis.

Key Aspects of Fontan Failure Management:

1. Medical Therapy:

   • Diuretics to manage fluid overload
   • ACE inhibitors or angiotensin receptor blockers (ARBs) for afterload reduction
   • Pulmonary vasodilators (e.g., sildenafil) to improve pulmonary blood flow
   • Anticoagulation to prevent thrombosis

2. Rhythm Management:

   • Antiarrhythmic drugs for atrial arrhythmias
   • Catheter ablation or pacemaker/ICD implantation if needed

3. Liver and Kidney Monitoring:

   • Regular screening for Fontan-associated liver disease (FALD)
   • Managing ascites and cirrhosis-related complications

4. Nutritional and Gastrointestinal Support:

   • Addressing protein-losing enteropathy (PLE) with steroids, heparin, or albumin infusion
   • Managing plastic bronchitis with pulmonary clearance therapies

5. Interventional and Surgical Options:

   • Fontan revision or conversion to an extracardiac conduit
   • Fenestration creation to decompress the system
   • Heart or heart-liver transplantation in severe cases

6. Exercise and Lifestyle Modifications:

   • Supervised exercise programs to optimize cardiac output
   • Avoiding dehydration and high-risk activities

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