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2007-04-16-11 Hypoplastic left heart syndrome and left dysplastic kidney © Cuillier www.thefetus.net/

Hypoplastic left heart syndrome and left dysplastic kidney 

Fabrice Cuillier, MD*; D Daguineau, MD**; J Bideault, MD**; JL Alessandri***.

*

Department of Gynecology, Felix Guyon Hospital, 97400 Saint- Denis, Ile de la Reunion, France; tel: 0262 90 55 22; fax: 0262 90 77 30;

**

Department of Obstetric, Mamoudzou"s Hospital, Ile de la Reunion, France;

***

Department of Neonatology, Felix Guyon Hospital, 97400, Saint-Denis, Ile de la Reunion, France.

 

Case report

We present the case of a 22-year-old woman (G1P0) with unremarkable history, no exposure to teratogens and normal first trimester ultrasonographic and biochemical screenings. At 24th week a hypoplastic left heart ventricle and polycystic left kidney were observed. Right ventricle of the heart was thickened and enlarged, the mitral valve seemed to be hypoplastic and rigid and small ventricular septal defect was visible. The ascending aorta was thin (1 mm) and the pulmonary artery was enlarged (7 mm). An abnormal right hand and penis were suspected. Amniotic fluid was normal.

The couple decided to continue the pregnancy. Amniocentesis was performed at 32nd week and revealed normal karyotype (46 XY). Serological test were also negative (TORCH and Chikungunya).

The patient delivered at 38th week (2800g) and the baby died eight days after birth. The postnatal sonography confirmed the cardiac and the nephrologic antenatal diagnoses.

Images 1 and 2: 24th weeks of gestation. Image 1 - gray scale ultrasound image showing the four-chamber view of the fetal heart with marked disproportion between small hypoplastic left ventricle and dilated right ventricle. Image 2 -  gray scale ultrasound image showing the disproportion between the thin aorta (left side of the image) and dilated pulmonary artery (right side of the image).

 

Images 3 and 4: 24th weeks of gestation - color Doppler ultrasound. Image 3 shows dilated right ventricle with massive blood flow. Image 4 shows disproportion between the small left and dilated right ventricles in color Doppler.

 

Images 5 and 6: 24th weeks of gestation - gray scale ultrasound. Image 5 shows dilated enlarged polycystic left kidney of the fetus. Image 6 shows comparison between normal appearance of the right fetal kidney (left side of the image) and enlarged multicystic left fetal kidney (right side of the image).

 

Images 7 and 8: 26th week of pregnancy. Gray scale (image 7) and color Doppler (image 8) scans showing disproportion between small left and enlarged right ventricles of the heart. Image 8 also shows massive blood flow from the right ventricle through the dilated pulmonary artery.

 

Video 1: Color Doppler image of the heart with the hypoplastic left ventricle at 26th week of pregnancy.

 

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