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Fetal Diagnosis of Left-Ventricular Noncompaction Cardiomyopathy in Identical Twins With Discordant Congenital Heart Disease

Abstract

Left-ventricular noncompaction (LVNC) is characterized by prominent myocardial trabeculae with deep intratrabecular recesses. Associated with ventricular dysfunction, LVNC can present in isolation or in conjunction with structural heart defects (i.e., “nonisolated” LVNC). Although it was once considered to be difficult and unreliable, successful detection of this condition by way of fetal echocardiography has been well documented. We present what is to our knowledge the first prenatal diagnosis of LVNC in monochorionic-monozygotic twins. Nonisolated LVNC was identified in twins A and B at 23 weeks’ gestation. A noncompaction-to-compaction ratio >2 was documented in both cases. Discordance for congenital heart disease was observed in the twins: Twin A presented with pulmonary atresia with an intact ventricular septum, critical tricuspid stenosis, and severe right-ventricular hypoplasia, whereas twin B presented with mild mitral valve dysplasia. Despite an initially complicated postnatal course for twin A, this case shows that it is feasible to achieve good outcomes with accurate prenatal detection and aggressive postnatal management.

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Conflict of interest

The authors have no conflict of interest to disclose.

Ethical standards

Appropriate consent was obtained from the parents of the subjects described in this case report.

Author information

Correspondence to Cheryl A. Vinograd.

Electronic supplementary material

Below is the link to the electronic supplementary material.

Movie Clip 1. Twin A. 2D long sweep shows PA with IVS, severe RV hypoplasia, and dysmorphic LV with LVNC. (MP4 2074 kb)

Movie Clip 2. Twin B. 2D short-axis view shows normal ventricular function with biventricular NC. (MPEG 1630 kb)

Movie Clip 3. Twin B. 2D apical four-chamber view shows biventricular NC. (MP4 2342 kb)

Movie Clip 1. Twin A. 2D long sweep shows PA with IVS, severe RV hypoplasia, and dysmorphic LV with LVNC. (MP4 2074 kb)

Movie Clip 2. Twin B. 2D short-axis view shows normal ventricular function with biventricular NC. (MPEG 1630 kb)

Movie Clip 3. Twin B. 2D apical four-chamber view shows biventricular NC. (MP4 2342 kb)

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Vinograd, C.A., Srivastava, S. & Panesar, L.E. Fetal Diagnosis of Left-Ventricular Noncompaction Cardiomyopathy in Identical Twins With Discordant Congenital Heart Disease. Pediatr Cardiol 34, 1503–1507 (2013) doi:10.1007/s00246-012-0406-5

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Keywords

  • Congenital heart disease
  • Fetal diagnosis
  • Noncompaction cardiomyopathy