EBSTEIN MALFORMATION

CAUSATION:

EBSTEIN MALFORMATION

In this anomaly there is downward displacement of the septal and posterior leaflets of the tricustoid valve into the right ventricle resulting in atrialisation of a large portion of the right ventricle. The tricuspid valve has a basket-like deformity resulting in incompetence. Tricuspid valve tissue is also dysplastic. Ostium secundum type of ASD id usually present in >50%. Cyanotic or acyanotic forms may be present. This Exposure to lithium cardonate as treatment of maniac depressive psychosis in first trimester of pregnancy is associated with high risk of this anomaly.

EBSTEIN MALFORMATION

CLINICAL FEATURE:

EBSTEIN MALFORMATION

Dyspnoea, fatigue, palpitation, heart failure are present. JVP is raised. Pulse is irregular (arterial fibrillation) and low in volume. Heart is quite, heart sounds are distant, irregular and systolic clicks are present. A systolic tricuspid murmur is usually present with wide and fixed splitting of s2. A characteristic short, superficial diastolic scratch murmur of tricustoid origin or s3 may be audible over lower left strenal border. X-ray of cheat shows enlargement of cardiac shadow due to right heart enlargement with stenciled outline. Echocardiogram reveals the chamber enlargement with delayed valve closure but two-dimensional echocardiography reveals the specific displacement of the tricuspid valve which is diagnostic. Angiocardiography will also demonstrate the same findings. Catheterisation (through dangerous at times) may show same pressure on the right side of the heart. By clectrode catheter and change of pressure from right ventricle to right atrium is present with no change in the ECG.

ECG will show complete or incomplete RBBB, WPW syndrome, atrial and ventricular arrhythmias. Right ventricular type of complex is seen when ECG is recorded proximal to the tricuspid valve. Complication include arrhythmias, heart failure and sudden.

EBSTEIN MALFORMATION

TREATMENT: EBSTEIN MALFORMATION

In severe cases a palliative operation consisting of plication of right atrium eliminating the atrial portion of the ventricle is all that can be done. Tricuspid valve replacement can also be performed.

EBSTEIN MALFORMATION

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