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Ebstein's anomaly
Etiology:
- maternal lithium ingestion during pregnancy
Pathology:
- congenital anomaly of the tricuspid valve in which the cusps are thin with downward displacement of a portion of the valve below the AV ring (ventriculization of the right atrium)
- associated conditions
a) atrial septal defect: ostium secundum
b) patent foramen ovale
c) pre-excitation syndrome (WPW syndrome)
Clinical manifestations:
1) tricuspid regurgitation
a) mild to moderate if at all
b) results in holosystolic murmur at left sternal border that increases with respiration
2) cool extremities, often with peripheral cyanosis, especially patients with concomitant ostium secundum
- clubbing due to chronic hypoxemi
3) one or more systolic clicks
4) patients may present with neurological events
Special laboratory:
1) electrocardiogram:
a) tall p waves
b) right bundle branch block
2) electrophysiologic studies to delineate bypass tract if indicated
Radiology:
1) chest X-ray
a) narrow pedicle with enlarged globular silhouette
b) right atrial enlargement
c) lung fields normal or oligemic
2) echocardiogram
3) cardiac catherization generally unnecessary
Related
atrial septal defect (ASD)
pre-excitation
General
congenital heart disease; congenital cyanotic heart disease
valvular heart disease
References
- DeGowin & DeGowin's Diagnostic Examination, 6th edition,
RL DeGowin (ed), McGraw Hill, NY 1994, pg 388
- Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed)
Lippincott-Raven, Philadelphia, 1998, pg 48-49