Ebstein’s Anomaly and Left Ventricular Non Compaction Cardiomyopathy: A Not So Unusual Association

نویسندگان

  • Daniele Andreini
  • Gianluca Pontone
  • Saima Mushtaq
  • Manuela Muratori
  • Marco Guglielmo
  • Edoardo Conte
  • Patrizia Carità
  • Mauro Pepi
چکیده

Left ventricular non-compaction (LVNC) is a rare congenital cardiomyopathy characterized by the presence of an extremely thickened endocardial layer with prominent trabeculations and deep recesses in communication with ventricular chamber and determining a typical spongeous aspect [1]. Noncompaction of the ventricular myocardium is thought to be a congenital arrest of endomyocardial embryogenesis in utero [2]. It has been reported a strong association with uncommon neuro-muscular disorders, such as Barth syndrome, EmeryDreifuss muscular dystrophy, myotubular myopathy, as well as mitochondrial abnormalities [3-5]. LVNC has been also associated with other cardiac abnormalities such as Ebstein’s anomaly [6]. Ebstein’s anomaly is a rare congenital cardiac disease, affecting about 1 in 200.000 live births [7]. It is characterized by apical displacement of the septal leaflet of the tricuspid valve causing significant tricuspid regurgitation and reduction of the functional right ventricle, right atrial and right ventricular dilatation and arrhythmias [8]. Recent studies have identified a genetic mutation (MYH7) as a cause of Ebstein’s anomaly associated with LVNC and other congenital heart disease [7].

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تاریخ انتشار 2016