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cardio.achd-ebstein.chronic.v1PRODUCTION
cardio.achd-ebstein.chronic.v1

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

cardiologychronicadult
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12/12 authored

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

Current phase

Frame

Detailed

Ebstein severity + accessory-pathway + shunt physiology

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Ebstein phenotype framed

Patient inputs (10)

Adult surveillance + surgical timing

TV displacement/TR severity + RV function — surgical-timing driver

Functional RV size/dysfunction — repair feasibility + timing

Symptomatic severe TR = surgical indication

Contrast for CMR/cath; drug dosing

Accessory pathway/WPW (10–25%) — ablation + preexcited-AF danger

ASD/PFO R→L shunt — cyanosis / paradoxical embolism; close at surgery

Cyanosis (R→L shunt) — erythrocytosis/iron management + closure indication

Atrial flutter/AF — rate/rhythm + anticoagulation

mWHO by severity/cyanosis/arrhythmia

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Severity triggers (9)

9 need judgement
  • informationallife_threateningpreexcited_af_avoid_av_nodal_blockers
    Preexcited AF (Ebstein accessory pathway, often multiple) — AV-nodal blockers CONTRAINDICATED (→ VF); urgent ablation; unstable → cardioversion — 2019 ESC SVT
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningrv_failure_branch
    Decompensated/end-stage RV failure despite repair — cautious diuresis; transplant / 1.5-ventricle strategies — 2020 ESC ACHD
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseveresymptomatic_severe_tr_cone_repair
    Symptomatic severe TR / RV dysfunction / cyanosis / paradoxical embolism / progressive cardiomegaly — surgical cone reconstruction — 2018 AHA/ACC ACHD
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereasd_pfo_cyanosis_branch
    ASD/PFO with right-to-left shunt → cyanosis / paradoxical embolism — closure (at cone surgery); cyanotic-CHD precautions — 2018 AHA/ACC ACHD
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereatrial_arrhythmia_branch
    Atrial flutter/AF (common; often accessory-pathway-related) — rate/rhythm + anticoagulation; concomitant maze at surgery — 2020 ESC ACHD
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverepregnancy_special_pop
    Pregnancy with Ebstein — mWHO II–IV by TR/RV/cyanosis/arrhythmia; cyanosis + arrhythmia worsen maternal/fetal risk; cardio-obstetric — ESC 2018 Pregnancy
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereaccessory_pathway_ablation_branch
    Accessory pathway (WPW, often multiple in Ebstein) without preexcited AF — EP study + ablation (before/at cone surgery) — 2019 ESC SVT
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderatechronic_cyanosis_branch
    Chronic cyanosis (R→L shunt) — secondary erythrocytosis: treat iron deficiency, avoid routine phlebotomy, hyperviscosity precautions — 2018 AHA/ACC ACHD
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderateckd_special_pop
    CKD — contrast minimisation (CMR/cath); renal-adjust AC/diuretics — KDIGO 2024
    Trigger could not be auto-evaluated — needs clinician judgement.

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Recommended regimen

Ebstein — cone repair + accessory-pathway/arrhythmia + cyanosis management (2018 AHA/ACC ACHD; 2020 ESC ACHD; 2019 ESC SVT)
axis: ebstein_surgery_arrhythmia_cyanosisstep 1 - Step 1 — Characterise severity + accessory pathway + shunt
Selected step "Step 1 — Characterise severity + accessory pathway + shunt" — Any adult Ebstein

outpatient playbook — drug actions (2)

  1. 1. cone reconstruction ± ASD closure + maze referral
    procedure • surgical • n/a
    trigger: Symptomatic severe TR / RV dysfunction / cyanosis (2018 AHA/ACC ACHD)
    Durable repair + concomitant lesions
  2. 2. accessory-pathway ablation; AC if AF/shunt
    procedure; apixaban 5 mg BID • catheter/PO • n/a/BID
    trigger: Accessory pathway / AF / paradoxical embolism (2019 ESC SVT)
    Arrhythmia + thromboembolic prevention

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: Echo: apical TV-leaflet displacement, atrialized RV, TR; RV failure / reduced exercise tolerance / fatigue; ECG preexcitation / AVRT / atrial arrhythmia.

Objective

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

Assessment

**Ebstein anomaly of the tricuspid valve (adult, chronic ACHD)** (cardio.achd-ebstein.chronic.v1).
Phenotype framing: Ebstein TR vs primary/functional TR; severity grade; cyanotic vs acyanotic
Scope: Ebstein severity + accessory-pathway + shunt physiology

No severity triggers fired against current inputs.

Plan

Regimen axis: **Ebstein — cone repair + accessory-pathway/arrhythmia + cyanosis management (2018 AHA/ACC ACHD; 2020 ESC ACHD; 2019 ESC SVT)** — step "Step 1 — Characterise severity + accessory pathway + shunt".

Setting playbook (outpatient) — Cone-repair symptomatic/severe Ebstein, ablate accessory pathway, manage shunt/cyanosis + arrhythmia/AC, lifelong ACHD (2018 AHA/ACC ACHD; 2020 ESC ACHD)
1. cone reconstruction ± ASD closure + maze referral procedure surgical n/a — Symptomatic severe TR / RV dysfunction / cyanosis (2018 AHA/ACC ACHD) (Durable repair + concomitant lesions)
2. accessory-pathway ablation; AC if AF/shunt procedure; apixaban 5 mg BID catheter/PO n/a/BID — Accessory pathway / AF / paradoxical embolism (2019 ESC SVT) (Arrhythmia + thromboembolic prevention)

Non-pharmacologic actions:
- ACHD centre + EP referral — 2018 AHA/ACC ACHD
- Cyanotic-CHD precautions (iron, avoid phlebotomy, air-filter) if R→L shunt — 2018 AHA/ACC ACHD
- Pregnancy + transition-of-care counseling — 2018 AHA/ACC ACHD

AVOID / contraindication checks:
- AV nodal blockers CONTRAINDICATED in preexcited AF Ebstein accessory pathway — 2019 ESC SVT
- Cone reconstruction preferred over replacement when feasible — 2018 AHA/ACC ACHD
- Close ASD PFO and ablate accessory pathway at cone surgery — 2020 ESC ACHD
- Avoid routine phlebotomy in compensated cyanotic erythrocytosis — 2018 AHA/ACC ACHD

Monitoring

Regimen monitoring:
- serial echo TR and RV function — 2018 AHA/ACC ACHD
- arrhythmia surveillance accessory pathway atrial — 2019 ESC SVT
- SpO2 and erythrocytosis iron if cyanotic — 2018 AHA/ACC ACHD
- post cone repair valve surveillance — 2018 AHA/ACC ACHD

Setting (outpatient) monitoring:
- Serial echo RV/TR; arrhythmia; SpO2/erythrocytosis — 2018 AHA/ACC ACHD

Follow-up plan: Lifelong ACHD; pregnancy + transition planning
- Close-out criterion: lifelong ACHD plan documented

Monitoring phase: RV function, TR, arrhythmia, cyanosis (erythrocytosis/iron), post-repair valve

Disposition

Current setting: outpatient — Cone-repair symptomatic/severe Ebstein, ablate accessory pathway, manage shunt/cyanosis + arrhythmia/AC, lifelong ACHD (2018 AHA/ACC ACHD; 2020 ESC ACHD)

Disposition criteria:
- Symptomatic severe → cone reconstruction
- Accessory pathway → ablation
- Mild/asymptomatic → ACHD surveillance

Escalation triggers (move to higher acuity):
- Preexcited AF → ED (no AV-nodal blockers) + urgent ablation — 2019 ESC SVT
- Decompensated RV failure → ACHD HF / transplant — 2020 ESC ACHD
- Progressive cyanosis / paradoxical embolism → expedite surgery — 2018 AHA/ACC ACHD

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [LIFE_THREATENING] Preexcited AF (Ebstein accessory pathway, often multiple) — AV-nodal blockers CONTRAINDICATED (→ VF); urgent ablation; unstable → cardioversion — 2019 ESC SVT
- [LIFE_THREATENING] Decompensated/end-stage RV failure despite repair — cautious diuresis; transplant / 1.5-ventricle strategies — 2020 ESC ACHD
- [SEVERE] Symptomatic severe TR / RV dysfunction / cyanosis / paradoxical embolism / progressive cardiomegaly — surgical cone reconstruction — 2018 AHA/ACC ACHD

Citations

- 2018 AHA/ACC Adult Congenital Heart Disease Guideline (Stout) + 2020 ESC Adult Congenital Heart Disease Guideline (Baumgartner) + 2019 ESC SVT Guideline (accessory-pathway) [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 31504425) [PMID:31504425](https://pubmed.ncbi.nlm.nih.gov/31504425/)
- Cited evidence (PMID 36017572) [PMID:36017572](https://pubmed.ncbi.nlm.nih.gov/36017572/)

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 ESC SVT Guideline (accessory-pathway) — PMID:30121239
  • Cited evidence (PMID 32860028) — PMID:32860028
  • Cited evidence (PMID 31504425) — PMID:31504425
  • Cited evidence (PMID 36017572) — PMID:36017572