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Ebstein's Anomaly, Left Ventricular Noncompaction, and Sudden Cardiac Death
Michael McGee1, Luke Warner1, Nicholas Collins1
1Cardiovascular Unit, John Hunter Hospital, Newcastle, NSW 2305, Australia.
Case Reports in Cardiology
|August 5, 2015
Summary
Ebstein's anomaly can coexist with left ventricular noncompaction (LVNC). This case highlights unrecognized LVNC in a sudden cardiac death patient, with screening confirming LVNC in a twin son.
Area of Science:
- Cardiology
- Congenital Heart Disease
- Cardiac Imaging
Background:
- Ebstein's anomaly is a congenital heart condition involving tricuspid valve displacement.
- It can be associated with other cardiac anomalies like left ventricular noncompaction (LVNC).
- LVNC is defined by excessive trabeculation in the left ventricle.
Purpose of the Study:
- To report a case of sudden cardiac death in a patient with Ebstein's anomaly and unrecognized LVNC.
- To emphasize the diagnostic utility of advanced imaging in complex congenital heart disease.
- To investigate the familial occurrence of LVNC in association with Ebstein's anomaly.
Main Methods:
- Review of post mortem examination findings.
- Echocardiography and magnetic resonance (MR) imaging for cardiac assessment.
- Genetic screening and familial evaluation.
Main Results:
- Sudden cardiac death occurred in a patient with Ebstein's anomaly and previously unrecognized LVNC.
- Post mortem examination revealed significant myocardial abnormalities consistent with LVNC.
- Screening identified LVNC in one of the patient's twin sons, suggesting a potential genetic link.
Conclusions:
- Unrecognized LVNC can be a critical, potentially fatal, comorbidity in patients with Ebstein's anomaly.
- Magnetic resonance imaging provides superior characterization of LVNC compared to echocardiography.
- Familial screening is crucial in cases of congenital heart disease with suspected genetic predisposition.
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