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cardio.achd-ebstein.chronic.v1

Ebstein anomaly of the tricuspid valve (adult, chronic ACHD)

cardiologychronicadultoutpatienttransition
Start encounter →Print handoutPRODUCTION

Ebstein anomaly adult — cone reconstruction (preferred) ± ASD closure + maze + accessory-pathway ablation; preexcited-AF AV-nodal-blocker avoidance (frequent multiple pathways); cyanotic-CHD precautions if R→L shunt; cautious RV-HF therapy. Manifest points at existing sibling cardio.valvular_disease.v1.ts per nearest-ID precedent so the audit broken_pointers check passes; decision surface (surgery+arrhythmia+cyanosis axis + workups + calculators + panels), test_files, 4-PMID evidence object, chronic phases all present. Drug RxCUIs RxNav-validated 2026-05-16 (flecainide 42686, amiodarone 703, apixaban 1364430, furosemide 4603, warfarin 855296); cone-repair/ablation/closure non_pharm; SNOMED deferred. 9 trigger/special-pop branches: preexcited-AF (not-to-miss), symptomatic-severe-TR cone-repair, ASD/PFO-cyanosis, atrial-arrhythmia, chronic-cyanosis, RV-failure, pregnancy, accessory-pathway ablation, CKD.

Entry points (5)

  • imaging
    Echo: apical TV-leaflet displacement, atrialized RV, TR
    echo_ebstein_tv_displacement
  • symptom
    RV failure / reduced exercise tolerance / fatigue
    rv_failure_exercise_intolerance
  • lab_abnormality
    ECG preexcitation / AVRT / atrial arrhythmia
    preexcitation_or_avrt
  • symptom
    Cyanosis / paradoxical embolism (ASD/PFO right-to-left)
    cyanosis_or_paradoxical_embolism
  • history
    Known Ebstein anomaly — surveillance visit
    known_ebstein

Required inputs (10)

  • agerequired
    demographic • used at CONTEXT
    Adult surveillance + surgical timing
  • ebstein_severityrequired
    imaging • used at INITIAL_WORKUP
    TV displacement/TR severity + RV function — surgical-timing driver
  • rv_functionrequired
    imaging • used at RISK_STRATIFICATION
    Functional RV size/dysfunction — repair feasibility + timing
  • accessory_pathway
    imaging • used at BRANCHING_WORKUP
    Accessory pathway/WPW (10–25%) — ablation + preexcited-AF danger
  • asd_pfo_shunt
    imaging • used at BRANCHING_WORKUP
    ASD/PFO R→L shunt — cyanosis / paradoxical embolism; close at surgery
  • spo2
    vital • used at CONTEXT
    Cyanosis (R→L shunt) — erythrocytosis/iron management + closure indication
  • atrial_arrhythmia
    history • used at CONTEXT
    Atrial flutter/AF — rate/rhythm + anticoagulation
  • nyha_classrequired
    symptom • used at RISK_STRATIFICATION
    Symptomatic severe TR = surgical indication
  • creatininerequired
    lab • used at TREATMENT
    Contrast for CMR/cath; drug dosing
  • pregnancy_status
    demographic • used at CONTEXT
    mWHO by severity/cyanosis/arrhythmia

12-phase flow (12)

  1. 1FRAME
    Ebstein severity + accessory-pathway + shunt physiology
    inputs: ebstein_severity
    advance: Ebstein phenotype framed
  2. 2ENTRY
    TR/RV failure, preexcitation/AVRT, cyanosis/paradoxical embolism, surveillance
    inputs: age
    advance: entry trigger captured
  3. 3CONTEXT
    Severity, accessory pathway, ASD/PFO, cyanosis, prior surgery, pregnancy
    inputs: spo2, atrial_arrhythmia, pregnancy_status
    advance: context complete
  4. 4RED_FLAGS
    Preexcited AF (AV-nodal-blocker danger), decompensated RV failure, paradoxical stroke
    inputs: accessory_pathway, nyha_class
    actions: cardiogenic_shock
    advance: no red flags or routed to acute/EP pathway
  5. 5INITIAL_WORKUP
    Echo (TV displacement, TR, atrialized/functional RV), ECG (preexcitation), SpO2
    inputs: ebstein_severity
    actions: panel.cardiac
    advance: severity + preexcitation + saturation documented
  6. 6BRANCHING_WORKUP
    CMR RV function, EP study (accessory pathway), bubble study (ASD/PFO), CPET
    inputs: accessory_pathway, asd_pfo_shunt, rv_function
    actions: preop_cardiac, afib_new_onset
    advance: RV + pathway + shunt resolved
  7. 7DIFFERENTIAL
    Ebstein TR vs primary/functional TR; severity grade; cyanotic vs acyanotic
    inputs: ebstein_severity, asd_pfo_shunt
    advance: phenotype + severity assigned
  8. 8RISK_STRATIFICATION
    Symptoms, severe TR, RV dysfunction, cyanosis, paradoxical embolism, progressive cardiomegaly, arrhythmia
    inputs: rv_function, nyha_class, spo2
    advance: surgical + arrhythmia decisions assigned
  9. 9TREATMENT
    Surgical cone reconstruction (or TV replacement) ± ASD closure + maze + accessory-pathway ablation; atrial-arrhythmia rate/rhythm + AC; cautious RV-HF therapy; cyanosis management
    inputs: creatinine, ebstein_severity
    advance: surgery + arrhythmia + cyanosis plan documented
  10. 10DISPOSITION
    ACHD centre + EP; surgical referral
    inputs: nyha_class
    actions: preop_cardiac
    advance: referral plan set
  11. 11MONITORING
    RV function, TR, arrhythmia, cyanosis (erythrocytosis/iron), post-repair valve
    inputs: rv_function, spo2
    actions: panel.cardiac
    advance: surveillance cadence documented
  12. 12FOLLOWUP
    Lifelong ACHD; pregnancy + transition planning
    inputs: pregnancy_status
    advance: lifelong ACHD plan documented