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Single Ventricle Physiology and Fontan Circulation

Staged surgical palliation for univentricular heart disease

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Çocuk Sağlığı ve Hastalıkları department. Book Appointment →

What is Single Ventricle Physiology and Fontan Circulation?

Single ventricle physiology encompasses anatomical variants such as hypoplastic left heart syndrome, tricuspid atresia, double-inlet ventricle, and unbalanced atrioventricular septal defect.

Surgical palliation is staged: Norwood/BT shunt (neonatal), Glenn (4–6 months), and Fontan (2–4 years).

The Fontan circulation passively directs systemic venous return to the pulmonary arteries, bypassing the heart's pumping function for the lungs.

It is preload-dependent—any volume loss or elevated pulmonary vascular resistance compromises cardiac output.

Long-term complications include protein-losing enteropathy, plastic bronchitis, hepatic congestion, and arrhythmias.

Lifetime cardiology follow-up and consideration of transplantation in late failure are essential.

Symptoms

Cyanosis at birth or in infancy (depending on lesion)
Failure to thrive, feeding difficulty
Tachypnea, hepatomegaly (heart failure signs)
Murmur on auscultation
Decreased oxygen saturation (typically 75–85% before Fontan completion)
After Fontan: exercise intolerance, peripheral edema, fatigue

Risk Factors

Maternal diabetes
Genetic syndromes (Turner, Noonan, Heterotaxy)
First-degree family history of congenital heart disease
Maternal teratogen exposure
Advanced maternal age
Twin pregnancies

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Prenatal diagnosis on fetal echocardiography enables planned delivery at a tertiary center.
  • Neonatal cyanosis or shock requires emergent cardiology evaluation.
  • Worsening cyanosis, edema, or feeding intolerance between surgical stages requires urgent assessment.
  • Adolescents and adults with Fontan circulation should remain in adult congenital heart disease clinics.
  • New arrhythmia, ascites, hepatic dysfunction, or worsening exercise tolerance signals Fontan failure.

Treatment Methods

01
Stage I: Norwood procedure or Sano modification with BT shunt (neonatal).
02
Stage II: Bidirectional Glenn shunt at 4–6 months.
03
Stage III: Fontan procedure (extracardiac conduit or lateral tunnel) at 2–4 years.
04
Anticoagulation with aspirin or warfarin to prevent thrombosis.
05
Diuretics, ACE inhibitors, and pulmonary vasodilators (sildenafil) in Fontan failure.
06
Catheter-based interventions for Fontan baffle obstruction or fenestration management.
07
Heart or heart-liver transplantation for end-stage Fontan failure.
08
Multidisciplinary care including cardiology, hepatology, and exercise rehabilitation.

Which Department to Visit?

You can visit our Çocuk Sağlığı ve Hastalıkları department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

Learn About Çocuk Sağlığı ve Hastalıkları Department

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.