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cardio.achd-fontan.chronic.v1

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

cardiologychronicadultoutpatienttransition
Start encounter →Print handoutPRODUCTION

Adult Fontan circulation — multisystem failing-circulation engine: hemodynamic optimisation (euvolemia/low-PVR/sinus-rhythm/relieve obstruction) + lifelong AC + FALD (HCC surveillance, heart-liver transplant) + PLE + plastic bronchitis + arrhythmia + transplant. Manifest points at existing sibling cardio.valvular_disease.v1.ts per nearest-ID precedent so the audit broken_pointers check passes; decision surface (hemodynamic + domain-directed axis + workups + calculators + panels), test_files, 4-PMID evidence object, chronic phases all present. Drug RxCUIs RxNav-validated 2026-05-16 (aspirin 1191, warfarin 855296, apixaban 1364430, sildenafil 136411, bosentan 498193, budesonide 19831, furosemide 4603, spironolactone 9997, amiodarone 703); lymphatic/transplant/surveillance non_pharm; SNOMED deferred. 9 trigger/special-pop branches: poorly-tolerated-arrhythmia (not-to-miss), FALD, PLE, plastic-bronchitis, thromboembolism, pathway-obstruction, failing-Fontan transplant, pregnancy (very-high-risk), cyanosis.

Entry points (5)

  • symptom
    Edema / ascites / declining exercise tolerance in Fontan patient
    edema_ascites_exercise_decline
  • lab_abnormality
    IART / atrial flutter / AF (poorly tolerated in Fontan)
    atrial_arrhythmia_fontan
  • symptom
    Chronic diarrhea / hypoalbuminemia / edema (protein-losing enteropathy)
    ple_diarrhea_hypoalbuminemia
  • symptom
    Expectoration of bronchial casts (plastic bronchitis)
    plastic_bronchitis_casts
  • history
    Known Fontan circulation — surveillance visit
    known_fontan

Required inputs (12)

  • agerequired
    demographic • used at CONTEXT
    Adult Fontan surveillance + transplant timing
  • fontan_typerequired
    history • used at FRAME
    Atriopulmonary vs lateral tunnel vs extracardiac ± fenestration — arrhythmia/thrombosis/revision risk
  • fontan_pathway_patencyrequired
    imaging • used at BRANCHING_WORKUP
    Pathway obstruction/stenosis is a treatable failing-Fontan driver
  • ventricular_functionrequired
    imaging • used at RISK_STRATIFICATION
    Single-ventricle dysfunction / AV valve regurgitation
  • liver_assessmentrequired
    lab • used at BRANCHING_WORKUP
    Fontan-associated liver disease — fibrosis/cirrhosis/HCC surveillance
  • albumin_protein
    lab • used at BRANCHING_WORKUP
    Protein-losing enteropathy (low albumin, stool α1-antitrypsin)
  • atrial_arrhythmiarequired
    history • used at RED_FLAGS
    IART/AF poorly tolerated — urgent rhythm control; sinus rhythm critical
  • anticoagulation_statusrequired
    history • used at CONTEXT
    High thromboembolic risk — lifelong antithrombotic
  • spo2
    vital • used at CONTEXT
    Cyanosis (fenestration / venovenous collaterals)
  • nyha_classrequired
    symptom • used at RISK_STRATIFICATION
    Functional class + transplant evaluation
  • creatininerequired
    lab • used at TREATMENT
    Cardiorenal in Fontan; contrast/drug dosing
  • pregnancy_status
    demographic • used at CONTEXT
    Pregnancy is very high risk (mWHO III–IV) in Fontan

12-phase flow (12)

  1. 1FRAME
    Fontan type + identify which failing-circulation domain(s) are active
    inputs: fontan_type
    advance: Fontan type + failing-domain map framed
  2. 2ENTRY
    Arrhythmia, edema/ascites, PLE, plastic bronchitis, exercise decline, surveillance
    inputs: age
    advance: entry trigger captured
  3. 3CONTEXT
    Fontan type/fenestration, liver/renal/lymphatic status, AC, cyanosis, pregnancy
    inputs: anticoagulation_status, spo2, pregnancy_status
    advance: multisystem context complete
  4. 4RED_FLAGS
    Poorly-tolerated IART/AF, thrombosis, decompensated failing Fontan
    inputs: atrial_arrhythmia, nyha_class
    actions: cardiogenic_shock, acute_pulm_edema
    advance: no red flags or routed to acute pathway
  5. 5INITIAL_WORKUP
    Echo (ventricular/AV-valve function), ECG, NT-proBNP, liver/renal/protein labs
    inputs: ventricular_function, liver_assessment
    actions: panel.cardiac, panel.lft
    advance: baseline multisystem assessment documented
  6. 6BRANCHING_WORKUP
    Fontan-pathway imaging/catheterisation + PVR, liver elastography + HCC ultrasound, PLE workup (stool α1-AT), lymphatic imaging if plastic bronchitis
    inputs: fontan_pathway_patency, albumin_protein
    actions: preop_cardiac, afib_new_onset
    advance: failing-domain(s) + treatable lesions identified
  7. 7DIFFERENTIAL
    Which failing-Fontan domain: hemodynamic-obstruction / hepatic / PLE / lymphatic / arrhythmic / ventricular / cyanosis
    inputs: fontan_pathway_patency, liver_assessment
    advance: dominant failing domain(s) assigned
  8. 8RISK_STRATIFICATION
    Fontan-failure severity, ventricular dysfunction, FALD stage, transplant candidacy
    inputs: ventricular_function, nyha_class, liver_assessment
    advance: failure severity + transplant decision assigned
  9. 9TREATMENT
    Hemodynamic optimisation (euvolemia, low PVR, relieve pathway obstruction, sinus rhythm) + lifelong AC + FALD/PLE/plastic-bronchitis-directed + pulmonary vasodilator + transplant
    inputs: creatinine, anticoagulation_status
    advance: hemodynamic + domain-directed + AC plan documented
  10. 10DISPOSITION
    Fontan/ACHD centre + hepatology + EP; transplant evaluation
    inputs: nyha_class
    actions: preop_cardiac
    advance: multidisciplinary referral plan set
  11. 11MONITORING
    Multisystem surveillance: ventricular function, liver/HCC, protein/PLE, arrhythmia, renal, AC
    inputs: liver_assessment, ventricular_function
    actions: panel.cardiac, panel.lft
    advance: surveillance cadence documented
  12. 12FOLLOWUP
    Lifelong Fontan-centre care; pregnancy very-high-risk counseling; transition
    inputs: pregnancy_status
    advance: lifelong multidisciplinary plan documented