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:
- Arrhythmia (accessory pathways)
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
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