Ebstein anomaly

Case contributed by Ronald Musngi Amurao


Heart arrhythmia, ventricular septal defect?

Patient Data

Age: 1 year
Gender: Male

Low right ventricular ejection fraction (35%) partially due to poor interventricular septum paradoxical movement due to atrialized portion of right ventricle.

Case Discussion

MRI features are consistent with Ebstein anomaly.

The acquisition of a cardiac MRI in the setting of congenital heart disease in an infant can pose quite a challenge. Here is a case of Ebstein anomaly in an infant done in our hospital.

Indication: Rapid heart rate, mottled skin and history of vomiting, poor feeding and was known congenital heart disease with intercostal retraction.

Technique: Cardiac MRI was done in 3 Tesla Skyra, under GA sedation and images were taken in apnea technique.

Apnea technique: acquisition was taken under the anesthesiologist guidance in holding the breathing by controlling through the MR conditional ventilator machine located inside the MR room.

1. Haste T1 Dark blood in Axial/Sagittal/Coronal

2. Trufi_CINE in 2/3/4 Chambers with Short Axis imaging was performed.

RVOT 2 and 3 Chambers and LVOT.

3. Angio 3D_CEMRA was the last acquisition taken. This was done through a care-bolus technique.  Gadolinium contrast was given by hand injection, at the same time the apnea technique was also applied, the anesthetist controlled the ventilating machine, while I was monitoring the contrast flowing in the "inline display" and upon seeing the pulmonary arteries lightened due to the contrast, the scan was triggered that took 14 seconds of scanning. The synchronization between the anesthetist and the MR technologist is vital in performing this examination.

As you noticed with the uploaded images, some artifacts are present due to the sensitivity of the 3 Tesla MR system to flow artifacts, even breathing artifacts. This are the most common pitfalls in the high field magnet. The correction for this artifacts are crucial, and sometimes, it requires another "TI  Scout" in a similar plane where the artifacts appeared e.g short axis, 2 chamber acquisition, just to get the proper frequency. Once the acquisition was done, the images should be reviewed and get the perfect TI and enter manually under Protocol parameters - Sequence - Part 2 - Trufi delta freq. Then repeat the scan the same plane the TI scout was taken. All of the techniques applied from the beginning until the end of the exam were "ECG triggered retrospectively" due to the high heart rate of the child.

4. Post-processing: Syngovia - VRT

The procedure went well and finished in an hour time.

The child was awakened and everything went well after the sedation and the MRI scan.

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