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2010-2-16-9 Transposition of great arteries with pulmonary atresia © Cerekja

Transposition of great arteries with pulmonary atresia 

Albana Cerekja, MD, PhD*, Flavia Ventriglia, MD**; Vanessa Martucci, MD**; Juan Piazze MD, PhD.*** 

*   Ultrasound Division, ASL Roma B, Rome, Italy.
**  Cardiologia pediatrica. Clinica pediatrica. Policlinico Umberto I. Università “La Sapienza” Rome, Italy.
*** Ultrasound Division, Ceprano Hospital, Ceprano, Italy.

Case report

A 23-year-old woman G1P0 was scanned at 20 weeks of gestation for a second trimester ultrasound screening. She had no contributive personal history and was not exposed to any teratogen agents during her pregnancy. 
Her family history was positive for a congenital heart disease. Mother's cousine was born with a corrected transposition of the great arteries. The father of the baby was a healthy 23-year-old man with non contributive personal or family history, no history of congenital heart disease.

We suspected a hypoplastic left heart syndrome with transposition of the great arteries while perfoming the ultrasound at 20 weeks of gestation. There were no other anomalies detected.

Echocardiography examination of the fetal heart was performed one week later with the following findings:

  • Transposition of the great arteries
  • Pulmonary atresia resulting in hypoplastic left heart
  • Dilated coronary sinus

After prenatal counselling, parents decided to continue with the pregnancy. Subsequent echocardiography examination at 28 weeks revealed a minimal tricuspid insufficiency.

A male baby of 2690 grams was delivered via cesarean section at 38 weeks. Neonatal echocardiography confirmed diagnosis of hypoplastic left ventricle, transposition of the great arteries, pulmonary atresia with hypoplastic pulmonary branches. There was total anomalous pulmonary venous connection - type II. All four pulmonary veins draining directly to the right heart via coronary sinus.

Prostaglandins (Prostin VR 0.01 mcg/kg/min) were administrated at birth to maintain ductus arteriosus patency and cardiac catheterization was done afterwards .
Surgery was performed 9 days after delivery. The diagnosis was confirmed and a pulmonary systemic shunting was established.

At the last echocardiography, at 4 months, the baby had 5350 grams. The shunt was functioning well keeping the oxgyne saturation at 85%.

The following surgery will be a bidirectional Glenn procedure (shunt between superior vena cava and pulmonary artery) which is scheduled for 6 months of age.

Images 1
: Image shows a 4-chamber-view, arrow indicates hypoplastic left ventricle heart.


Images 2,3: Images show a dilation of the coronary sinus due to an abnormal venous return, all pulmonary veins are draining into to the coronary sinus


Images 4,5:  Images show aorta and pulmonary artery parallel to each other.


Images 6,7: Image 6 shows a parallel position of the aorta and pumonary artery in the sagittal view. Image 7 shows inferior and superior vena cava draining the right atrium.


Images 8,9: Aorctic arch.


Images 10,11: Sagittal view at the image 10 shows an aortic arch indicated arch (blue arrow), common pulmonary artery (red arrow) and descending aorta (purple arrow). Image 11 shows the same vessels on the transverse view at the level of the green line. Doppler shows a turbulent flow in the pulmonary artery.




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