3Dechocardiography
the art of defining cardiac morphology

TGA with sub PS

4 week old baby boy weighing 4 kgs has saturation of 70% in air. He is diagnosed as having Transposition of the great arteries, severe sub-pulmonary stenosis and ventricular septal defects. He is breast fed and thriving well. Normal left sided aortic arch. Transthoracic cross sectional echocardiographic images are displayed below.

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Fig: 1 Zoomed view of the sub-pulmonary area from the apical 4 chamber view. The cephalad deviation of the subpulmonary septum is clearly seen with a muscular outlet VSD just below it.

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Fig2. Parasternal long axis view of the same heart with posterior deviation of the infundibular septum. The anterior aorta arising from the hypertrophied RV iss now well in view.

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Fig:3 Colour Doppler image of the sub-pulmonary VSD with turbulant flow across the sub-pulmonary stenosis.

What is your plan of management?

  1. Augment pulmonary blood flow with an additional shunt and perform a Rastelli procedure when child is bigger.
  2. Disconnect the pulmonary arteries and protect the lung for a future single ventricular repair.
  3. Perform a BT shunt and do an atrial switch (Mustard / Senning) procedure when child is older.
  4. Resect the sub-pulmonary obstruction, perform an arterial switch and close the ventricular septal defect.

Discussion

FIg: MPR of the sub pulmonary region showing severe sub-pulmonary stenosis as evident on cross sectional 2D imaging.

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Fig: 2 MPR above the posteriorly deviated muscular infundibulum clearly demonstrates a good size aortic annulus with a tri cuspid pulmonary valve. It was concluded from the 3DMPR that the sub pulmonary infundibular septum could be resected and the aortic annulus is adequate for an arterial switch procedure. Hence we went for option 4.

Post-operative imaging

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