Understanding the Fontan Circulation
The Fontan procedure is a palliative surgical operation performed in children with single-ventricle congenital heart defects — conditions in which only one functional ventricle is present, such as hypoplastic left heart syndrome, tricuspid atresia, or double inlet left ventricle. Rather than constructing a two-ventricle circulation, the Fontan operation connects the superior and inferior vena cava directly to the pulmonary artery, allowing systemic venous blood to flow passively through the lungs without the aid of a pumping chamber. This arrangement allows patients to survive and grow into adulthood, but the Fontan circulation is passive, non-pulsatile, chronically under-perfused in the pulmonary vasculature, and dependent on low pulmonary vascular resistance and well-preserved single-ventricle function.
Fontan Failure: Recognizing the Spectrum
Fontan failure is not a single event but a spectrum of deteriorating physiology. Early signs may include worsening exercise intolerance, new pleural or pericardial effusions, and elevated ventricular filling pressures. More advanced manifestations include protein-losing enteropathy (PLE) — characterized by intestinal protein loss causing hypoalbuminemia, edema, and diarrhea — and plastic bronchitis, in which proteinaceous casts form in the airways. Fontan-associated liver disease (FALD) — chronic hepatic congestion leading to fibrosis and eventually cirrhosis — develops in virtually all Fontan patients over time and requires coordinated management with hepatology. Ventricular dysfunction and Fontan circuit obstruction may require catheter-based intervention or revision surgery.
Have questions about your care?
Speak directly with our team — no referral needed for most consultations.
Expert Fontan Management in New York
Congenital Heart Compass Medical PLLC provides specialized Fontan management for adults across New York State. Our services include annual Fontan surveillance assessments (echocardiography, laboratory evaluation, liver assessment), medication optimization, coordination with hepatology for FALD staging, referral for catheter-based intervention when circuit pressures or obstructions are identified, and advanced therapy coordination including evaluation for heart transplantation when Fontan failure is present. We work with major ACHD and transplant programs while maintaining accessible telemedicine follow-up for patients across Rochester, Buffalo, Syracuse, Albany, and the broader Upstate and Western New York region.
Reviewed by Dr. Pradeepkumar Charla, MD, MBA, FAAP, FACC
Pediatric & Adult Congenital Cardiologist — Congenital Heart Compass Medical PLLC
Last reviewed:
Medical disclaimer: This content is for educational purposes only and is not a substitute for individualized medical advice, diagnosis, or treatment. Always consult a qualified cardiologist for decisions about your congenital heart disease care.