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cardio.achd-fontan.chronic.v1PRODUCTION
cardio.achd-fontan.chronic.v1

Adult Fontan circulation (single-ventricle palliation, chronic ACHD)

cardiologychronicadult
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Encounter flow

12/12 authored

Canonical 12-phase frame with authored status for this dossier.

Current phase

Frame

Detailed

Fontan type + identify which failing-circulation domain(s) are active

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Fontan type + failing-domain map framed

Patient inputs (12)

Pathway obstruction/stenosis is a treatable failing-Fontan driver

Fontan-associated liver disease — fibrosis/cirrhosis/HCC surveillance

Adult Fontan surveillance + transplant timing

High thromboembolic risk — lifelong antithrombotic

Atriopulmonary vs lateral tunnel vs extracardiac ± fenestration — arrhythmia/thrombosis/revision risk

IART/AF poorly tolerated — urgent rhythm control; sinus rhythm critical

Single-ventricle dysfunction / AV valve regurgitation

Functional class + transplant evaluation

Cardiorenal in Fontan; contrast/drug dosing

Protein-losing enteropathy (low albumin, stool α1-antitrypsin)

Cyanosis (fenestration / venovenous collaterals)

Pregnancy is very high risk (mWHO III–IV) in Fontan

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Severity triggers (9)

9 need judgement
  • informationallife_threateningpoorly_tolerated_atrial_arrhythmia
    IART/atrial flutter/AF in Fontan — poorly tolerated (loss of atrial kick + passive flow); urgent rhythm control + anticoagulation + ablation; pacing for sinus-node dysfunction — 2020 ESC ACHD
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningfailing_fontan_transplant
    Refractory failing Fontan (ventricular dysfunction / multiorgan failure) — transplant (heart, or combined heart-liver if advanced FALD) — 2019 AHA Fontan statement
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningpregnancy_special_pop
    Pregnancy with Fontan — very high risk (mWHO III–IV): arrhythmia, thrombosis, PLE, ventricular failure, fetal loss; expert cardio-obstetric; many advised against pregnancy — ESC 2018 Pregnancy
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverefald_branch
    Fontan-associated liver disease — serial elastography + HCC ultrasound (±AFP); cirrhotic FALD informs combined heart-liver transplant — 2019 AHA Fontan statement
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereprotein_losing_enteropathy_branch
    Protein-losing enteropathy (hypoalbuminemia, edema, diarrhea, ↑stool α1-AT) — multimodal: high-protein diet, albumin, budesonide, anticoagulation, fenestration, transplant — 2019 AHA Fontan statement
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereplastic_bronchitis_branch
    Plastic bronchitis (bronchial casts) — lymphatic imaging + thoracic-duct/selective lymphatic embolization + Fontan optimisation — 2019 AHA Fontan statement
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverethromboembolism_branch
    Fontan thromboembolism (pathway thrombus / stroke / PE) or high-risk — lifelong anticoagulation (warfarin; DOAC selected); aspirin lower-risk — 2019 AHA Fontan statement
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverefontan_pathway_obstruction_branch
    Fontan-pathway stenosis/obstruction or elevated PVR — treatable failing-Fontan driver: catheter intervention/stent ± pulmonary vasodilator — 2020 ESC ACHD
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderatecyanosis_branch
    Cyanosis from fenestration / venovenous collaterals — assess closability vs needed pop-off; erythrocytosis/iron management — 2018 AHA/ACC ACHD
    Trigger could not be auto-evaluated — needs clinician judgement.

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Recommended regimen

Fontan — hemodynamic optimisation + failing-domain-directed therapy (2018 AHA/ACC ACHD; 2020 ESC ACHD; 2019 AHA Fontan statement)
axis: fontan_hemodynamic_and_failing_domainstep 1 - Step 1 — Hemodynamic optimisation (the foundation for every failing domain)
Selected step "Step 1 — Hemodynamic optimisation (the foundation for every failing domain)" — Any adult Fontan
  • euvolemia + low-PVR maintenance + relieve Fontan-pathway obstruction (stent) + maintain sinus rhythm
    first line
    hemodynamic_optimisation
    triggers: fontan_circulation
    Fontan output is preload-dependent + PVR-sensitive — avoid dehydration, treat pathway stenosis, preserve sinus rhythm (2019 AHA Fontan statement)

outpatient playbook — drug actions (3)

  1. 1. hemodynamic optimisation + lifelong AC
    aspirin 81 mg or warfarin INR-guided • PO • daily
    trigger: Any Fontan (2019 AHA Fontan statement)
    Foundation: euvolemia, sinus rhythm, thromboprophylaxis
  2. 2. domain-directed (PLE budesonide / pulmonary vasodilator / lymphatic)
    per domain • PO/procedure • per domain
    trigger: Specific failing domain (2019 AHA Fontan statement)
    Targeted multimodal therapy
  3. 3. rhythm control if IART/AF
    amiodarone ± ablation • PO/procedure • daily/n.a.
    trigger: Poorly-tolerated atrial arrhythmia (2020 ESC ACHD)
    Sinus rhythm is critical in Fontan

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: Edema / ascites / declining exercise tolerance in Fontan patient; IART / atrial flutter / AF (poorly tolerated in Fontan); Chronic diarrhea / hypoalbuminemia / edema (protein-losing enteropathy).

Objective

- No vitals, labs, or imaging entered for this encounter.

Assessment

**Adult Fontan circulation (single-ventricle palliation, chronic ACHD)** (cardio.achd-fontan.chronic.v1).
Phenotype framing: Which failing-Fontan domain: hemodynamic-obstruction / hepatic / PLE / lymphatic / arrhythmic / ventricular / cyanosis
Scope: Fontan type + identify which failing-circulation domain(s) are active

No severity triggers fired against current inputs.

Plan

Regimen axis: **Fontan — hemodynamic optimisation + failing-domain-directed therapy (2018 AHA/ACC ACHD; 2020 ESC ACHD; 2019 AHA Fontan statement)** — step "Step 1 — Hemodynamic optimisation (the foundation for every failing domain)".
1. euvolemia + low-PVR maintenance + relieve Fontan-pathway obstruction (stent) + maintain sinus rhythm (hemodynamic_optimisation, first line) — Fontan output is preload-dependent + PVR-sensitive — avoid dehydration, treat pathway stenosis, preserve sinus rhythm (2019 AHA Fontan statement)

Setting playbook (outpatient) — Optimise Fontan hemodynamics, lifelong AC, multisystem failing-domain surveillance + directed therapy, timely transplant (2018 AHA/ACC ACHD; 2019 AHA Fontan statement)
2. hemodynamic optimisation + lifelong AC aspirin 81 mg or warfarin INR-guided PO daily — Any Fontan (2019 AHA Fontan statement) (Foundation: euvolemia, sinus rhythm, thromboprophylaxis)
3. domain-directed (PLE budesonide / pulmonary vasodilator / lymphatic) per domain PO/procedure per domain — Specific failing domain (2019 AHA Fontan statement) (Targeted multimodal therapy)
4. rhythm control if IART/AF amiodarone ± ablation PO/procedure daily/n.a. — Poorly-tolerated atrial arrhythmia (2020 ESC ACHD) (Sinus rhythm is critical in Fontan)

Non-pharmacologic actions:
- Fontan/ACHD centre + hepatology + EP multidisciplinary care — 2019 AHA Fontan statement
- Transplant evaluation (heart vs heart-liver) for failing Fontan — 2019 AHA Fontan statement
- Pregnancy very-high-risk counseling; transition-of-care — 2018 AHA/ACC ACHD

AVOID / contraindication checks:
- Avoid hypovolemia and over diuresis Fontan output is preload dependent — 2019 AHA Fontan statement
- Maintain sinus rhythm atrial arrhythmia is poorly tolerated urgent — 2020 ESC ACHD
- Lifelong thromboprophylaxis Fontan thrombosis risk is high — 2019 AHA Fontan statement
- HCC surveillance in FALD do not omit — 2019 AHA Fontan statement
- Combined heart liver transplant if advanced cirrhotic FALD — 2019 AHA Fontan statement

Monitoring

Regimen monitoring:
- serial ventricular and AV valve function echo — 2018 AHA/ACC ACHD
- liver elastography and HCC ultrasound AFP — 2019 AHA Fontan statement
- albumin and stool alpha1 antitrypsin for PLE — 2019 AHA Fontan statement
- arrhythmia surveillance and AC monitoring — 2020 ESC ACHD
- renal function and exercise capacity — 2019 AHA Fontan statement

Setting (outpatient) monitoring:
- Multisystem surveillance (cardiac/hepatic/protein/renal/arrhythmia) — 2019 AHA Fontan statement

Follow-up plan: Lifelong Fontan-centre care; pregnancy very-high-risk counseling; transition
- Close-out criterion: lifelong multidisciplinary plan documented

Monitoring phase: Multisystem surveillance: ventricular function, liver/HCC, protein/PLE, arrhythmia, renal, AC

Disposition

Current setting: outpatient — Optimise Fontan hemodynamics, lifelong AC, multisystem failing-domain surveillance + directed therapy, timely transplant (2018 AHA/ACC ACHD; 2019 AHA Fontan statement)

Disposition criteria:
- Stable Fontan → multidisciplinary surveillance
- Failing domain → directed therapy ± transplant referral
- Advanced FALD + failing Fontan → combined heart-liver transplant evaluation

Escalation triggers (move to higher acuity):
- Poorly-tolerated IART/AF → urgent rhythm control + AC + ablation — 2020 ESC ACHD
- Refractory PLE/plastic bronchitis / failing Fontan → transplant evaluation — 2019 AHA Fontan statement
- HCC on surveillance → hepatology/oncology + transplant pathway — 2019 AHA Fontan statement

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [LIFE_THREATENING] IART/atrial flutter/AF in Fontan — poorly tolerated (loss of atrial kick + passive flow); urgent rhythm control + anticoagulation + ablation; pacing for sinus-node dysfunction — 2020 ESC ACHD
- [LIFE_THREATENING] Refractory failing Fontan (ventricular dysfunction / multiorgan failure) — transplant (heart, or combined heart-liver if advanced FALD) — 2019 AHA Fontan statement
- [LIFE_THREATENING] Pregnancy with Fontan — very high risk (mWHO III–IV): arrhythmia, thrombosis, PLE, ventricular failure, fetal loss; expert cardio-obstetric; many advised against pregnancy — ESC 2018 Pregnancy

Citations

- 2018 AHA/ACC Adult Congenital Heart Disease Guideline (Stout) + 2020 ESC Adult Congenital Heart Disease Guideline (Baumgartner) + 2019 AHA Fontan Scientific Statement (Rychik) [PMID:30121239](https://pubmed.ncbi.nlm.nih.gov/30121239/)
- Cited evidence (PMID 32860028) [PMID:32860028](https://pubmed.ncbi.nlm.nih.gov/32860028/)
- Cited evidence (PMID 31256636) [PMID:31256636](https://pubmed.ncbi.nlm.nih.gov/31256636/)
- Cited evidence (PMID 32673028) [PMID:32673028](https://pubmed.ncbi.nlm.nih.gov/32673028/)

Last reconciled with current guidelines: 2026-05-16.
References
  • 2018 AHA/ACC Adult Congenital Heart Disease Guideline (Stout) + 2020 ESC Adult Congenital Heart Disease Guideline (Baumgartner) + 2019 AHA Fontan Scientific Statement (Rychik) — PMID:30121239
  • Cited evidence (PMID 32860028) — PMID:32860028
  • Cited evidence (PMID 31256636) — PMID:31256636
  • Cited evidence (PMID 32673028) — PMID:32673028