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José Alberto de Agustín, Leopoldo Perez de Isla, José Luis Zamorano, Ebstein anomaly and hypertrophic cardiomyopathy, European Heart Journal, Volume 29, Issue 20, October 2008, Page 2525, https://doi.org/10.1093/eurheartj/ehn186
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Extract
An asymptomatic 72-year-old man without cardiovascular risk factors was evaluated due to a systolic murmur on an examination. Transthoracic echocardiogram revealed severe left ventricular hypertrophy associated with Ebstein anomaly (Panel A). An asymmetric septal hypertrophy was observed (thickness = 22 mm). Left ventricular cavity was reduced (Panel B). A peak gradient of 40 mmHg was obtained in the left ventricular outflow tract (Panel C). The tricuspid valve insertion was very distal, close to the apex, with almost complete atrialization of the right ventricle with the exception of a small infundibular component. However, tricuspid regurgitation was no more than moderate. Calculated pulmonary artery systolic pressure was 40 mmHg. Right chambers were not enlarged. A cardiac magnetic resonance was performed confirming the findings (Panels D and E). It showed apical displacement of the hinge point of the septal and posterior leaflet from the atrioventricular ring. Ebstein anomaly was type B, with a large atrialized component of the right ventricle, but the anterior leaflet moves freely (Panel F). The patient had no previous history of supraventricular tachycardias, and familial screening was negative.