Case study: Reversing Fontan-related liver disease in a preteen

We’ve learned that single-ventricle Fontan circulation has significant impacts on the heart as well as other organ systems. As a preventive measure, the patient received a series of surveillance tests.

The patient

A 12-year-old boy born with double-inlet left ventricle (DILV), mitral stenosis, left-sided superior vena cava coronary sinus and pulmonary stenosis: The patient had been through surgical palliation as a baby, leaving him with Fontan circulation.

The challenge

Over the past decade we’ve learned that single-ventricle Fontan circulation has significant impacts on the heart as well as other organ systems. At Norton Children’s Heart Institute Fontan Clinic we have an established protocol for patients, which includes continuous surveillance of many organ systems. This patient in particular was at risk for developing Fontan-associated liver disease characterized by progressive liver fibrosis, due to increased pressure in the veins.

Initial echocardiogram showing double-inlet left ventricle

The solution

As part of an ongoing study (detecting the pathophysiology of Fontan-associated liver disease), the patient received surveillance, stress testing, cardiac MRI, liver elastography and lab work in order to screen for the condition.

At the Norton Children’s Heart Institute Fontan Clinic, our team is able to monitor the condition and find complications before they become end-stage. The multidisciplinary clinic setting allows patients to receive care during a single visit — where they can be seen by a Fontan cardiologist, advanced heart failure specialist, dietitian, social worker, specialist in transition care for adult congenital heart disease and an exercise physiologist. Also, all further referrals are provided during the multidisciplinary clinic appointment.

Angiogram showing the Glenn to pulmonary artery and Fontan to pulmonary artery connection

The result

After initial work-up, the patient has no signs of Fontan-associated liver disease; he has normal end organ function and is doing well clinically. His elastography is elevated, but he has no clinical or imaging signs of Fontan-associated liver disease at this time. His exercise capacity is what is expected for someone with Fontan physiology. With his normal systolic function and good exercise capacity he does not have many exercise restrictions and will be starting in a competitive baseball league.

Most-recent echocardiogram continues to show DILV, but now the Fontan connection can be seen behind the atria.

Norton Children’s Heart Institute Fontan Clinic providers


Joshua D. Kurtz, M.D., pediatric cardiologist at Norton Children’s Heart Institute, affiliated with the UofL School of Medicine

Syeda S. Maqsood, M.D.
Norton Children’s Heart Institute, affiliated with the UofL School of Medicine
Ashley Eastman, R.N.
Laura Dean, RDN, CSP, L.D

Refer a patient

To refer a patient to Norton Children’s Heart Institute Fontan Clinic, visit Norton EpicLink and choose EpicLink referral to Pediatric Cardiology.

The multidisciplinary team is able to monitor the condition and find complications before they become end-stage. Disciplines sharing expertise in the clinic include a Fontan cardiologist, advanced heart failure specialist, dietitian, social worker, transition care for adult congenital heart disease (ACHD) and an exercise physiologist.

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