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2014-11-21-22 Case of the week #383 © Anwer Sadat www.TheFetus.net 

 
Answer to the case of the week #383

November 27, 2014 - December 11, 2014


Anwer Sadat Kithir Mohamed, DMRD
 

Tiruvarur medical centre, No 5, Javulikara street, Tiruvarur, Tamil Nadu-610001, India.

 

Case report

A 24-year-old woman (G2P1) was refered to our center at 30 weeks of gestation. Her previous ultrasonographic exam at 20 weeks was reported to be normal.

Our ultrasonographic examination found a fetus of normal growth concordant with 30 weeks of gestation. Detailed anatomical survey revealed following findings:

- Inferior vena cava and aorta located on the same side - on the right side of the spine;
- Stomach located on the left side; symmetric liver;
- Absent spleen;
- Atrioventricular septal defect with cardiac axis oriented to the right side; 
- Pyramidal shape of both cardiac atrial appendages with broad junction with the atria;
- Cardiac type of total anomalous pulmonary venous drainage into the right sided atrium;
- Aorta and pulmonary artery were both arising from the left ventricle;
- Superior vena cava was seen on the left side.

Images 1, 2, 3, 4, and 5; videos 1, 2: The images and videos show juxtaposition of the aorta and inferior vena cava on the right side; left-sided mediolateral position of the stomach; cardiac axis deviation to the right; abnormal morphology of the heart - atrioventricular septal defect.

 


 


 

Image 6
: The image shows transverse section of the fetal thorax at the level of the four-chamber view of the heart - atrial appendages are symmetric with right-sided morphology.


Images 7, 8, video 3: The images show abnormal cardiac morphology at the level of the four-chamber view of the heart - atrioventricular septal defect. 

 



Image 9: The image show transverse scan of the fetal thorax with superior vena cava located on the left side.



Video 4: The video shows both aorta and pulmonary artery arising from the right ventricle.



Video 5, 6: The videos show four chamber view of the heart with anomalous pulmonary venous return to the right-sided atrium of the heart. 

 

Definition

Heterotaxy syndrome is a spectrum of abnormal thoracic and abdominal organ arrangement and it is classified into two main groups - bilateral right sidedness (right atrial isomerism) and bilateral left sidedness (left atrial isomerism).

Right atrial isomerism is characterized by presence of double right sided structures with underdevelopment or absence of left sided structures[1].

Synonyms

Asplenia syndrome, Ivemark syndrome.

Incidence

Heterotaxy syndrome has the incidence rate of 2.2 to 4.2% of infants with congenital heart disease. Right isomerism is less common than left isomerism [1].

Prenatal sonographic findings in the right isomerism

Abdominal findings

- Juxtaposition of the aorta and inferior vena cava on the right or left side of the spine is the typical finding;
- Absent spleen (color Doppler can identify absent splenic artery);
- Midline or right sided liver;
- Stomach can be located on the left, middle, or right side;
- Hiatal hernia can occur as an associated abnormality.

Thoracic findings

- Both cardiac atrial appendages have right morphology (pyramidal shape with broadly communicating with the atria);
- Cardiac axis is often oriented to the right, but midline or left orientation is also possible;
- Univentricular heart is often present;
- Most cases  are associated with unbalanced atrioventricular septal defect;
- Ventriculo-arterial connection is often discordant (double outlet ventricle, malposition of great arteries - often associated with pulmonary atresia or stenosis);
- Partial or total anomalous pulmonary venous return is more common in the right isomerism (in principle there is absence of anatomic left atrium);
- Persistent left superior vena cava.

Prognosis

Prognosis is generally worse in the right isomerism because more complex anomalies are usually present.

References

1. Alfred Abuhamad Rabih Chaoui ,A practical guide to Fetal Echocardiography,Normal and Abnormal Hearts,Second edition 2010,chapter22,page 311to322

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