Related Experiment Videos
Noncompacted myocardium in Ebstein's anomaly: initial description in three patients
Christine H Attenhofer Jost1, Heidi M Connolly, Carole A Warnes
1Division of Cardiovascular Diseases and Internal Medicine, Mayo Clinic and Mayo Foundation, Rochester, MN 55905, USA.
Summary
Ebstein's anomaly can affect the left ventricle (LV), causing noncompaction. This rare finding may explain exercise intolerance and heart failure in patients with this congenital heart condition.
Area of Science:
- Cardiology
- Congenital Heart Disease
- Cardiac Imaging
Background:
- Exercise intolerance in Ebstein's anomaly is typically linked to right-to-left shunting and right ventricular issues.
- Left ventricular (LV) involvement is not commonly considered a primary cause of symptoms in Ebstein's anomaly.
Observation:
- Three patients with severe Ebstein's anomaly presented with left ventricular (LV) myocardium abnormalities.
- These abnormalities resembled features of LV noncompaction, characterized by hypertrabeculation and intertrabecular recesses.
Findings:
- The observed LV pathology ranged from mild to severe noncompaction.
- Diastolic dysfunction was noted in two patients, and all experienced diminished exercise tolerance.
- One patient with biventricular failure required heart transplantation.
Implications:
- Ebstein's anomaly may be associated with left ventricular noncompaction, expanding its known pathophysiology.
- LV noncompaction offers a potential explanation for exercise intolerance and left heart failure symptoms in affected individuals.
- This highlights the importance of evaluating LV structure and function in Ebstein's anomaly patients presenting with unexplained symptoms.