Ebstein anomaly is a congenital abnormality of the tricuspid valve and right ventricular myocardium characterised by the following
Patients present with cyanosis, palpitations due to tachyarrhythmias and sudden cardiac death. Heart failure can occur, particularly in right heart failure.
Ebstein's anomaly may be associated with Wolff Parkinson White syndrome, along with atrial septal defects and PFO.
Important echo feature of Ebstein anomaly
In patent ductus arteriosus, there is diastolic flow reversal in the pulmonary arteries, not veins. It is a strong sign of PDA. The left heart may be dilated.
A Chiari malformation is tissue strands from the inferior vena cava. They may be associated with atrial septal aneurysms. As the IVC drains into the right atrium, it is obvious that the Chiari malformation will be seen in the right atrium.
References
1. Soizic Paranon, Philippe Acar Congenital heart disease: Ebstein’s anomaly of the tricuspid valve: from fetus to adult Heart 2008;94:237-243
2. Oechslin, E.; Buchholz, S.; Jenni, R. Ebstein's Anomaly in Adults: Doppler Echocardiographic Evaluation Thorac cardiovasc Surg 2000; 48: 209-213
3. Kunwar P. Bhatnagar, G.S.Nettleton, Ferrell R. Campbell, Charles E. Wagner, Nobuyuki Kuwabara,Horia Muresian Chiari anomalies in the human right atrium Clinical anatomy 2006 19;6:510-516
- Apical displacement of the tricuspid valve
- Atrialisation of the right ventricle
- Tricuspid regurgitation
Patients present with cyanosis, palpitations due to tachyarrhythmias and sudden cardiac death. Heart failure can occur, particularly in right heart failure.
Ebstein's anomaly may be associated with Wolff Parkinson White syndrome, along with atrial septal defects and PFO.
Important echo feature of Ebstein anomaly
- Apical displacement of the septal leaflet of the tricuspid valve > 8mm/sqmtr is a very specific sign
- If this is not measured, the tricuspid valve is displaced apically >10mm when compared to the mitral valve
- Dilated RA and RV with impaired RV function
- Moderate to severe TR
- There is abnormal eccentric coaptation of the septal and anterior tricuspid valve leaflet
- Delayed closure of tricuspid valve after mitral valve
In patent ductus arteriosus, there is diastolic flow reversal in the pulmonary arteries, not veins. It is a strong sign of PDA. The left heart may be dilated.
A Chiari malformation is tissue strands from the inferior vena cava. They may be associated with atrial septal aneurysms. As the IVC drains into the right atrium, it is obvious that the Chiari malformation will be seen in the right atrium.
References
1. Soizic Paranon, Philippe Acar Congenital heart disease: Ebstein’s anomaly of the tricuspid valve: from fetus to adult Heart 2008;94:237-243
2. Oechslin, E.; Buchholz, S.; Jenni, R. Ebstein's Anomaly in Adults: Doppler Echocardiographic Evaluation Thorac cardiovasc Surg 2000; 48: 209-213
3. Kunwar P. Bhatnagar, G.S.Nettleton, Ferrell R. Campbell, Charles E. Wagner, Nobuyuki Kuwabara,Horia Muresian Chiari anomalies in the human right atrium Clinical anatomy 2006 19;6:510-516
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