Patient handout

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

PRODUCTION

1. Your condition

This handout is for ebstein anomaly of the tricuspid valve (adult, chronic achd). Your care team identified this based on: echo: apical tv-leaflet displacement, atrialized rv, tr.

Other reasons your team may use this plan: rv failure / reduced exercise tolerance / fatigue; ecg preexcitation / avrt / atrial arrhythmia; cyanosis / paradoxical embolism (asd/pfo right-to-left).

3. When to call your provider

Contact your care team if any of the following happen:

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

4. When to seek emergency care

Call 911 or go to the nearest emergency room right away if you have:

Preexcited AF (Ebstein accessory pathway, often multiple) — AV-nodal blockers CONTRAINDICATED (→ VF); urgent ablation; unstable → cardioversion — 2019 ESC SVT(life-threatening)
Symptomatic severe TR / RV dysfunction / cyanosis / paradoxical embolism / progressive cardiomegaly — surgical cone reconstruction — 2018 AHA/ACC ACHD
ASD/PFO with right-to-left shunt → cyanosis / paradoxical embolism — closure (at cone surgery); cyanotic-CHD precautions — 2018 AHA/ACC ACHD
Atrial flutter/AF (common; often accessory-pathway-related) — rate/rhythm + anticoagulation; concomitant maze at surgery — 2020 ESC ACHD
Decompensated/end-stage RV failure despite repair — cautious diuresis; transplant / 1.5-ventricle strategies — 2020 ESC ACHD(life-threatening)
Pregnancy with Ebstein — mWHO II–IV by TR/RV/cyanosis/arrhythmia; cyanosis + arrhythmia worsen maternal/fetal risk; cardio-obstetric — ESC 2018 Pregnancy
Accessory pathway (WPW, often multiple in Ebstein) without preexcited AF — EP study + ablation (before/at cone surgery) — 2019 ESC SVT

5. Follow-up

Lifelong ACHD; pregnancy + transition planning

6. Sources

Guideline: 2018 AHA/ACC Adult Congenital Heart Disease Guideline (Stout) + 2020 ESC Adult Congenital Heart Disease Guideline (Baumgartner) + 2019 ESC SVT Guideline (accessory-pathway)