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Fetal and Postnatal Echocardiographic Diagnosis of Ebstein Anomaly of the Mitral Valve

Abstract

Ebstein anomaly of mitral valve (MV) is an extremely rare congenital heart disease. In the current report, we present a case of Ebstein of MV that was diagnosed prenatally. Fetal echocardiogram showed that the posterior leaflet of MV was tethered to the lateral wall of left ventricle (LV) with downward displacement into LV cavity. Postnatal transthoracic and transesophageal echocardiograms confirmed the diagnosis with apical displacement of the level of coaptation MV into the LV cavity. To the best of our knowledge, fetal diagnosis of Ebstein anomaly of MV has not yet been reported in the medical literature.

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References

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Author information

SA: Data collection, drafting the article, and approval of the article. JB: Data collection, critical revision of the article, and approval of the article. KD: Concept and design of the manuscript, critical revision of the article, and approval of the article. BAM: Data acquisition, critical revision of the article, and approval of the article.

Correspondence to Safwat Aly.

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All the authors declare no conflict of interest.

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This article does not contain any studies with human participants or animals performed by any of the authors.

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Informed consent was obtained from all individual participants included in the study.

Electronic supplementary material

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Video Clip 1: Fetal echocardiogram with 4 chamber view showing a tethered posterior leaflet of mitral valve. The leaflets of the MV are apically displaced as well (AVI 9143 KB)

Video Clip 1: Fetal echocardiogram with 4 chamber view showing a tethered posterior leaflet of mitral valve. The leaflets of the MV are apically displaced as well (AVI 9143 KB)

Video Clip 2: Transthoracic echocardiogram with 2 D obtained from the left low parasternal window showing a tethered posterior leaflet, a fenestrated anterior leaflet of mitral valve, and a large apical ventricular septal defect (AVI 7415 KB)

Video Clip 2: Transthoracic echocardiogram with 2 D obtained from the left low parasternal window showing a tethered posterior leaflet, a fenestrated anterior leaflet of mitral valve, and a large apical ventricular septal defect (AVI 7415 KB)

Video Clip 3: Transesophageal echocardiogram showing a tethered posterior leaflet of MV to the lateral wall of LV with apical displacement into LV cavity (AVI 3711 KB)

Video Clip 3: Transesophageal echocardiogram showing a tethered posterior leaflet of MV to the lateral wall of LV with apical displacement into LV cavity (AVI 3711 KB)

Video Clip 4: Transesophageal echocardiogram with color Doppler showing apical displacement of the level of coaptation of MV leaflets. There is mild mitral regurgitation (AVI 3464 KB)

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Aly, S., Bokowski, J., Diab, K. et al. Fetal and Postnatal Echocardiographic Diagnosis of Ebstein Anomaly of the Mitral Valve. Pediatr Cardiol 39, 1276–1279 (2018). https://doi.org/10.1007/s00246-018-1903-y

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Keywords

  • Ebstein
  • Mitral valve
  • Fetal echocardiography