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.
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 Android. A list of contributors can be seen here.
If you enjoyed this post, why not check out our live conference on 26th August. Find out more about DFTB: Live + Connected
Ebstein’s Anomaly
Tags: ebstein, tricuspid
Anders, M. Ebstein’s Anomaly, Don't Forget the Bubbles, 2013. Available at:
http://doi.org/10.31440/DFTB.3465
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:
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:
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 AnomalyAll Marc’s PICU cardiology FOAM can be found on PICU Doctor and can be downloaded as a handy app for free on iPhone or Android. A list of contributors can be seen here.
About Marc Anders
View all posts by Marc Anders | Website