Hypoplastic left heart syndrome

Fabrice Cuillier, MD*; P. Lemaire, MD**; Alice Bertha***

*    Department of Gynecology, Félix Guyon Hospital, 97400 Saint-Denis, Ile de la Réunion, France;
**   Department of Gynecology, Intercommunal"s Hospital, 97400 Saint-Benois, Ile de la Réunion, France;
***  Student, Arizona State University. University Drive and Mill Avenue Tempe, Arizona 85281.


Case report
 

A a 22-year-old woman (G3P1) was referred to our antenatal unit at 23 weeks of her pregnancy for an ultrasonographic examination. Her family history was negative for congenital diseases and she was not exposed to any teratogenic drugs.

The ultrasonography revealed an abnormal configuration of the fetal heart:

  • Small left ventricle with mitral hypoplasia (Images 1-3)
  • Normal pulmonary artery (Images 4-7).
  • Hypoplastic aorta (Images 5-7).   

The fetal face looked normal (Image 8). The parents opted for the continuation of the pregnancy. An amniocentesis was done revealing normal karyotype. The diagnosis was confirmed postnatally and the baby underwent a cardiac surgery in Paris.

Images 1, 2: 23 weeks of the pregnancy; the images show transverse scans of the fetal thorax at the level of the four-chamber view of the heart. The left ventricle is hypoplastic (Vg = left ventricle, Vd = right ventricle, Og = right atrium, Od = left atrium).

1A
1B

Images 3, 4: 23 weeks of the pregnancy; the image 3 shows transverse scan of the fetal thorax at the level of the four-chamber view of the heart, with hypoplastic left ventricle. Rim of endocardial fibroelastosis covering the walls of the hypoplastic left ventricle can also be seen. The image 4 shows normal pulmonary artery (left part of the image; AP = pulmonary artery) and small left ventricle at the level of the four-chamber view of the heart (right part of the image).

1C
2A

Images 5, 6: The image 5 compares the size of the normal pulmonary artery (left part of the image) and of the thin aorta (right part of the image). The image 6 compares the size of the normal pulmonary artery (right part of the image) and of the thin aorta (left part of the image).

2B
2C

Image 7: Normal Doppler of the pulmonary artery.

2D

Image 8: 3D image of the normal fetal face.

3

Images 9, 10: The videos show hypoplastic left ventricle of the heart with the mitral stenosis.

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