A combination of right ventricular hypertrabeculation/noncompaction and Ebstein's anomaly.
A 35-year-old man admitted with iterative extremities edema for three months and mild chest complaint associated with activity without chest distress for five days. He underwent an initial 2DE that did not confirm HVM/NVM and 3DE that strengthened the diagnosis of HVM/NVM. The limitations of 2DE may lead to misinterpretation of prominent trabeculations as "false tendons," and may also lead to underestimation of the severity of HVM/NVM. In contrast to 2DE, 3DE provides for pyramid-shaped datasets that encompass the entire right ventricular. Specifically, right ventricular can be sectioned in userselected planes and an unlimited number and angles of such planes can be used. Intracavitary echodensities that are suspicious for trabeculations can be tracked in multiple directions from base to apex. To our knowledge, this is the first report on only right ventricular HVM/NVM associated with Ebstein's malformation. The leaflets of the tricuspid valve develop from endocardial cushion tissue and the myocardium. The tricuspid valve leaflets become freely movable after delamination of the tension apparatus within the myocardium. Although the coexistence of right ventricular HVM/NVM and Ebstein's malformation could be a coincidence, I believe that both defects could be caused by a developmental arrest of the right ventricular myocardium. That is to say, the genetic change that is responsible for Ebstein's anomaly also plays a role in the differentiation of the right myocardium.
Song
Ze-Zhou
ZZ
D000328:Adult; D004437:Ebstein Anomaly / Q000150:complications* / Q000000981:diagnostic imaging*; D004452:Echocardiography; D006352:Heart Ventricles / Q000002:abnormalities; D006801:Humans; D008297:Male; D014261:Tricuspid Valve / Q000002:abnormalities; D018497:Ventricular Dysfunction, Right / Q000150:complications* / Q000000981:diagnostic imaging*