Complex congenital heart defects in children with heterotaxy syndrome and right isomerism: MDCT assessment

Congress:

ECR 2018

Poster Number:

C-0780

Type:

Scientific Exhibit

Keywords:

Congenital, Contrast agent-intravenous, CT-Angiography, CT, Cardiovascular system, Cardiac

Authors:

N. Rokytska¹, R. Tammo¹, T. Yalynska², H. Morkovkina¹, A. Halchenko³; ¹Kyiv/UA, ²Kyiv, Kyiv/UA, ³Kiev/UA

DOI:

10.1594/ecr2018/C-0780

DOI-Link:

https://dx.doi.org/10.1594/ecr2018/C-0780

Fig. 1: Picture of situs ambiguous, right isomerism

Fig. 2: Picture of situs ambiguous, left isomerism

Fig. 3: Photograph of outside anatomical preparation of right atrium.

Fig. 4: Photograph of inside anatomical preparation of right atrium.

Aims and objectives

Heterotaxy is a class of congenital disorders resulting from failure to establish normal left – right (L – R) asymmetry during embryonic development. which leads to symmetry of morphologically asymmetric organs. [1] The lungs, liver, spleen, stomach, and atria are usually asymmetric organs and the term situs is used to define the position of the atria and viscera relative to the midline or sagital plane. Any unusual symmetry of asymmetric thoracic and abdominal organs, and the atrial appendages within the heart we can call a...

Methods and materials

Between 2011–2017, 29 patients (5 girls, 24 boys) with a mean age 7,4 ± 5 months, with HS with RI underwent MDCT to recognition of the spectrum of CHD. The MDCT images were acquired on a 16 slices scanners in all patients after performing echocardiography. CT dose parameters of 80 kV and 40 mAs with tube current modulation were set for all patients. CT data were obtained using the following parameters: detector collimation, 1.5 mm; slice thickness, 2 mm; Increment, 1 or 2 mm; Feed...

Results

CHD in patients with HS with RI were as follows: atrioventricular septal defects (AVSD) was detected in 72% (n = 21) (Fig. 6, 7), total anomaly pulmonary vein connection (TAPVC) – 82 % (n = 24) (Fig. 6, 7, 8), transposition of great arteries (TGA) – 55% (n =16) (Fig. 7, 8, 9), pulmonary atresia (PA) – 41% (n = 12) (Fig. 8 9), double outlet right ventricle (DORV) – 38 % (n =11) (Fig. 6), hypoplasia left heart syndrome (HLHS) – 3% (n =...

Conclusion

Low dose MDCT is the best method for clear assessment of complex CHD in patients with HS with RI and visualizing a wide spectrum of organ malpositions.

Personal information

Nadiia Rokytska MD, radiologist of Radiology department of Ukrainian children cardiac centre (UCCC), Kyiv Raad Tammo MD, PhD, radiologist of Radiology department of Ukrainian children cardiac centre (UCCC), Kyiv Tetyana Yalynska MD, PhD, chef of Radiology department of Ukrainian children cardiac centre (UCCC), Kyiv Hanna Morkovkina MD, radiologist of Radiology department of Ukrainian children cardiac centre (UCCC), Kyiv Halchenko Oleksandr MD, radiologist of Radiology department of Ukrainian children cardiac center (UCCC), Kyiv

References

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