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Ebstein’s Anomaly

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Definition: ‘atrialisation’ of the right ventricle with inferior placement of the septal and posterior leaflet of the tricuspid valve (most common redundant anterior leaflet). Associated defects: ASD.

Prevalence: <1% of congenital heart disease.

Incidence: 0.5-2.5:100.000.


Physiology:

Various clinical pictures depending on position of leaflet: TR, RV hypoplasia, RVOTO, systolic and diastolic dysfunction of LV and associated defects.

RA enlargement and accessory pathways predispose to arrhythmias.


Diagnosis:

Echo (Gose score), CXR (cardiomegaly)


Differential diagnosis:

Aberrant tendinous chords


Preoperative management:

  • Neonates: CCF due to TR and RV dysfunction, commence prostaglandin E1 = alprostadil (starting dose 20 ng/kg/min) to augment PBF. However a large PDA with left to right shunt can cause systemic hypoperfusion (circular shunt due to PR): cease alprostadil.
  • Adolescents/adults: right heart failure due to TR (can cause cardiomyopathy and arrhythmias).

Preoperative preparation:

ECG, CXR, CUS, FBE, clotting, UECs, PRBC (4), FFP (2), platelets (2), cryoprecipitate (2).

Methylprednisolone 10 mg/kg 12 hrs and 6 hrs pre surgery in neonates.


Surgery:

Depends on clinical and anatomical presentation: tricuspid valve repair: annuloplasty +/- prosthetic ring; tricuspid valve replacement (mechanical or bioprosthetic); 1 ½ ventricle repair (Glenn/Fontan surgery).


Postoperative management:

  • In tricuspid valve repair or replacement: anticipate RV dysfunction: milrinone (0.25-1 mcg/kg/min), arrhythmias
  • Anticoagulation: commence heparin 10 U/kg/hr once no bleeding, increase heparin dose further to adjust for therapeutic APTT
  • Vitamin K antagonist in mechanical valve (INR 2.5-3.5) once stable and all drains removed. Continue UFH until INR >2.0 for two consecutive days.
  • 1 ½ ventricle repair (Glenn Shunt)
  • Fontan surgey (Fontan circulation)

Specific problems:


Outcome:

Depends on presentation, clinical symptoms, anatomical presentation and type of surgery.


References:

[1] Heart. 2008 Feb;94(2):237-43: Paranon et al: Ebstein’s anomaly of the tricuspid valve: from fetus to adult: congenital heart disease.

[2] Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann 2009.12:63-65: Bove et al: How I Manage Neonatal Ebstein’s Anomaly


All Marc’s PICU cardiology FOAM can be found on PICU Doctor and can be downloaded as a handy app for free on iPhone or AndroidA list of contributors can be seen here.

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