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PubMed This is a summary of 19 peer-reviewed journal articles Updated
Pediatric Cardiology

Long-Term Health After the Fontan Procedure

At a Glance

Life after the Fontan procedure for Hypoplastic Left Heart Syndrome requires lifelong medical monitoring. Long-term care focuses on preserving right ventricle function, screening for liver and lymphatic complications like FALD, and supporting developmental and psychosocial needs.

Completing the three-stage surgical journey is a monumental milestone. For many children with Hypoplastic Left Heart Syndrome (HLHS), the Fontan procedure provides a stable circulation that allows them to lead active, meaningful lives [1]. However, life with “half a heart” requires lifelong, vigilant monitoring to manage the unique ways the body adapts to Fontan circulation [1][2].

Monitoring the “Single Pump”

Because the right ventricle is doing the work of the entire heart, it is under constant demand. Doctors focus on two main areas of long-term heart health:

  • Right Ventricular (RV) Function: Over time, the RV may become tired or “dilated” (stretched out) [3][4]. Regular echocardiograms and cardiac MRIs are used to monitor how well the muscle is squeezing and if the tricuspid valve (the main valve on the right side) is leaking [3][5].
  • Arrhythmias: Scar tissue from previous surgeries or the high pressure in the heart can cause abnormal heart rhythms, such as atrial flutter [6][7]. These can often be managed with medication or specialized procedures called “ablations” [6].

Understanding Lymphatic and Liver Complications

Fontan circulation involves higher-than-normal pressure in the veins. This chronic high pressure affects other organ systems, creating specific long-term risks:

1. Lymphatic Complications: High venous pressure can cause the lymphatic system to “back up,” leading to two rare but serious conditions [8][9]:

  • Protein-Losing Enteropathy (PLE): Protein-rich fluid leaks into the intestines [10][8]. Symptoms include chronic diarrhea, abdominal swelling, and low albumin in the blood [8][9].
  • Plastic Bronchitis (PB): Lymphatic fluid leaks into the lungs and hardens into rubbery “casts” [11][8]. The primary symptom is a severe, chronic cough [8].
  • Treatment: Lymphatic embolization is a newer, minimally invasive procedure where doctors find the leak and “plug” it [12][13].

2. Fontan-Associated Liver Disease (FALD): Because blood from the lower body flows passively through the liver to the lungs, the liver experiences chronic congestion. Over time, this leads to liver fibrosis (scarring) in almost all adult Fontan survivors. Lifelong, routine liver screening using bloodwork, ultrasounds, and specialized MRIs is an essential part of survivorship care.

Physical Activity and Daily Life

A common anxiety for parents is envisioning what their child’s daily life will look like. The good news is that most children with a Fontan circulation are encouraged to be physically active. While highly competitive or intense endurance sports (like marathon running) might not be possible, light-to-moderate recreational sports, biking, and playing with friends are typically encouraged to keep the heart muscle healthy. Always work with your cardiologist to set specific, safe exercise boundaries.

The Developmental Marathon

Neurodevelopmental challenges are common in the HLHS population due to the heart’s different blood flow patterns during fetal life and the stress of multiple surgeries [14][15].

  • The Strategy: One-time screenings are not enough. Survivors need serial neurodevelopmental evaluations throughout childhood to ensure they receive early intervention, physical therapy, or school accommodations (such as an IEP or 504 plan) as soon as they are needed [16][17].

The Psychological Toll

Living with a chronic condition like HLHS is an emotional marathon. The need for constant “watchfulness” can lead to significant stress and anxiety for parents [18][19]. It is essential to integrate psychosocial support into your child’s care—not just for the child, but for you as the caregiver [18]. Connecting with a specialized Adult Congenital Heart Disease (ACHD) program early can help ease the transition into a successful and supported adulthood [1].

Common questions in this guide

What kind of heart monitoring is needed after the Fontan procedure?
Because the right ventricle has to pump blood to the entire body, it can become stretched or tired over time. Doctors use regular echocardiograms and cardiac MRIs to monitor how well the heart muscle is squeezing and to ensure the main valve is not leaking.
What is Fontan-Associated Liver Disease (FALD)?
After a Fontan procedure, blood from the lower body flows passively through the liver, creating chronic high pressure and congestion. Over time, this leads to scarring known as FALD, which requires lifelong routine screening using bloodwork, ultrasounds, and MRIs.
What are the warning signs of lymphatic complications after Fontan surgery?
High venous pressure can cause the lymphatic system to back up. If fluid leaks into the intestines (Protein-Losing Enteropathy), symptoms include chronic diarrhea and abdominal swelling. If it leaks into the lungs (Plastic Bronchitis), it causes a severe, persistent cough.
Can my child play sports after having the Fontan procedure?
Most children with Fontan circulation are highly encouraged to be physically active, as it helps keep the heart muscle healthy. While intense endurance sports may be restricted, light-to-moderate recreational sports are usually safe and beneficial once cleared by a cardiologist.
Why are ongoing neurodevelopmental evaluations necessary for HLHS survivors?
Children with HLHS face unique developmental challenges due to altered fetal blood flow and the stress of multiple open-heart surgeries. Serial evaluations throughout childhood ensure that any delays are caught early so the child can receive physical therapy or school accommodations.

Questions for Your Doctor

5 questions

  • •How is my child’s right ventricular function trending over time, and what specific measurements (like RVFAC or Global Longitudinal Strain) are you watching?
  • •When should my child have their next formal neurodevelopmental evaluation, and what specific domains will be tested?
  • •What is the schedule for routine liver screening (FALD) as my child grows older?
  • •Are there signs of 'lymphatic congestion' on recent imaging that could put my child at risk for PLE or Plastic Bronchitis?
  • •What are the specific guidelines for my child regarding physical activity, sports, and exercise?

Questions for You

2 questions

  • •Have I noticed any new symptoms, like a persistent cough, swelling in the legs, or changes in my child's digestion, that I should report?
  • •Am I taking care of my own mental health and seeking support for the emotional 'marathon' of chronic medical monitoring?

References

References (19)
  1. 1

    The impact of morphological characteristics on late outcomes in patients born with hypoplastic left heart syndrome†.

    Newland DP, Poh CL, Zannino D, et al.

    European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery 2019; (56(3)):557-563 doi:10.1093/ejcts/ezz052.

    PMID: 30805594
  2. 2

    Plastic Bronchitis: Extensive Cast Expectoration in a 6-Year-Old Boy with Fontan Circulation.

    Pfeifer J, Poryo M, Fries P, Abdul-Khaliq H

    Diagnostics (Basel, Switzerland) 2025; (15(22)) doi:10.3390/diagnostics15222864.

    PMID: 41300888
  3. 3

    Longitudinal Assessment of Right Ventricular Function in Hypoplastic Left Heart Syndrome.

    Balasubramanian S, Smith SN, Srinivasan P, et al.

    Pediatric cardiology 2021; (42(6)):1394-1404 doi:10.1007/s00246-021-02624-y.

    PMID: 33987707
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    Modeling of the Tricuspid Valve and Right Ventricle in Hypoplastic Left Heart Syndrome With a Fontan Circulation.

    Nam HH, Flynn M, Lasso A, et al.

    Circulation. Cardiovascular imaging 2023; (16(3)):e014671 doi:10.1161/CIRCIMAGING.122.014671.

    PMID: 36866669
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    A Novel Case of Marfan Syndrome in an Infant With Hypoplastic Left Heart Syndrome.

    Karunanandaa A, Plant KC, Herlong JR, et al.

    World journal for pediatric & congenital heart surgery 2019; (10(5)):641-642 doi:10.1177/2150135119852341.

    PMID: 31496403
  6. 6

    Radiofrequency catheter ablation as a treatment option in a patient with hypoplastic left heart syndrome and atrial flutter after Fontan operation-Case report.

    Jagosz M, Kowalska W, Lenarczyk R, et al.

    Journal of arrhythmia 2021; (37(4)):1101-1104 doi:10.1002/joa3.12579.

    PMID: 34386138
  7. 7

    Native atrial septal restriction after Fontan palliation successfully treated with transcatheter Diabolo stent.

    Aldoss O, Reinking BE, Divekar A

    Annals of pediatric cardiology 2016; (9(1)):49-52 doi:10.4103/0974-2069.171411.

    PMID: 27011693
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    Protein-Losing Enteropathy and Plastic Bronchitis After the Fontan Operation.

    Peyton C

    Critical care nurse 2018; (38(6)):e5-e12 doi:10.4037/ccn2018784.

    PMID: 30504504
  9. 9

    Diagnosis and Management of Lymphatic Disorders in Congenital Heart Disease.

    Kelly B, Mohanakumar S, Hjortdal VE

    Current cardiology reports 2020; (22(12)):164 doi:10.1007/s11886-020-01405-y.

    PMID: 33037935
  10. 10

    Commentary: Lymphatic fluid flows uphill in both hemispheres-and leaks in the same places.

    Woods RK, Hraska V

    The Journal of thoracic and cardiovascular surgery 2021; (161(6)):2166-2167 doi:10.1016/j.jtcvs.2020.08.031.

    PMID: 32900477
  11. 11

    Protein-losing enteropathy and plastic bronchitis after the Fontan procedure.

    Sharma VJ, Iyengar AJ, Zannino D, et al.

    The Journal of thoracic and cardiovascular surgery 2021; (161(6)):2158-2165.e4 doi:10.1016/j.jtcvs.2020.07.107.

    PMID: 32928546
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    Protein-Losing Enteropathy in Patients With Congenital Heart Disease.

    Itkin M, Piccoli DA, Nadolski G, et al.

    Journal of the American College of Cardiology 2017; (69(24)):2929-2937 doi:10.1016/j.jacc.2017.04.023.

    PMID: 28619193
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    Lymphatic Management in Single-Ventricle Patients.

    Itkin M, Pizarro C, Radtke W, et al.

    Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual 2020; (23()):41-47 doi:10.1053/j.pcsu.2020.03.001.

    PMID: 32354546
  14. 14

    The neurodevelopmental implications of hypoplastic left heart syndrome in the fetus.

    Lloyd DF, Rutherford MA, Simpson JM, Razavi R

    Cardiology in the young 2017; (27(2)):217-223 doi:10.1017/S1047951116001645.

    PMID: 27821206
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    Mitotic Block and Epigenetic Repression Underlie Neurodevelopmental Defects and Neurobehavioral Deficits in Congenital Heart Disease.

    Gabriel GC, Yagi H, Tan T, et al.

    bioRxiv : the preprint server for biology 2024; doi:10.1101/2023.11.05.565716.

    PMID: 38464057
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    Six-Year Neurodevelopmental Outcomes for Children With Single-Ventricle Physiology.

    Sananes R, Goldberg CS, Newburger JW, et al.

    Pediatrics 2021; (147(2)) doi:10.1542/peds.2020-014589.

    PMID: 33441486
  17. 17

    Cognitive Development of School-Age Hypoplastic Left Heart Syndrome Survivors: A Single Center Study.

    Oberhuber RD, Huemer S, Mair R, et al.

    Pediatric cardiology 2017; (38(6)):1089-1096 doi:10.1007/s00246-017-1623-8.

    PMID: 28508919
  18. 18

    Navigating family life with Hypoplastic Left Heart Syndrome: A qualitative study.

    Green MD, Prevost-Reilly A, Parker DM, Carpenter-Song E

    PLOS mental health 2024; (1(7)) doi:10.1371/journal.pmen.0000208.

    PMID: 40575012
  19. 19

    Caring for a Child with Hypoplastic Left Heart Syndrome: Parent and Medical Perspective.

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    PMID: 34826115

This page provides educational information on long-term health after the Fontan procedure for HLHS. Always consult your pediatric cardiologist or adult congenital heart disease specialist for personalized monitoring and care guidelines.

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