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2006-05-30-11 Double-outlet right ventricle © Cuillier www.thefetus.net/


Double-outlet right ventricle with a complete atrioventricular septal defect and situs inversus

Cuillier F, MD, Narboni T, MD

* Department of Gynecology, Felix Guyon Hospital  ** Radiologist, Goyaves Street - Saint Denis, Ile de la Reunion, France
 

Case report: This is a 22-year-old-woman, G5P4, with no family history of malformations or genetic disorders referred to our unit at 32 weeks. She did not perform the first and second trimester screenings. At 28 weeks, a cardiac anomaly and a duodenal stenosis were diagnosed. At 32 weeks, the following ultrasound findings were diagnosed:

  • Polyhydramnios (32 cm)
  • A visceral situs inversus
  • The liver was on the left side
  • The stomach and the heart were on the right side
  • A "double bubble"  image corresponding to a duodenal stenosis
  • The spleen was located on the right hypochondrium
  • The kidneys were normal
  • The heart was located on the right hemithorax with dextrocardia
  • A complete atrioventricular septal defect (single atrio-ventricular valve)
  • The Doppler examination revealed a small ventricular-atrial regurgitation
  • The right ventricle had a smooth wall
  • The left ventricle has some trabeculations in the wall
  • Two arteries were seen emerging from the right ventricle (suggestive of a double-outlet right ventricle)
  • The diameter of the aorta seemed normal (9 mm)
  • The pulmonary artery seemed small (6 mm)
  • The inferior vena cava was seen
  • The pulmonary veins (at least two) seemed connected to the right atria

At 34 weeks, an amniocentesis was performed to reduce the polyhydramnios. A karyotype was performed (46 XX). The patient refused to perform an interruption of the pregnancy. The baby was born at 38 weeks (2550g) with normal external morphology. The radiography confirmed the visceral and cardiac situs inversus with dextrocardia. The abdominal scan confirmed the left position of the liver. The spleen was on the right position. The kidneys were normal. A postnatal echocardiography confirmed:

  • A cardiac and visceral situs inversus
  • The pulmonary veins were connected on the right part of atrial mass
  • The inferior vena cava was connected to the right atrial mass
  • The superior vena cava was small  (suggestive of a venous innominate trunk)
  • A left vena cava was not seen
  • A large atrioventricular septal defect with a single atrio-ventricular valve
  • The two ventricles had the same size
  • The left ventricular was on the right side of the heart and it was connected with the venous part of the atrial mass
  • The right ventricular was on the left side of the heart
  • The two great vessels emerged from the right ventricle.
  • The atrioventricular septal defect was permeable and maintained permeable up to the cardiac surgery.

The final diagnosis was double-outlet right ventricular with pulmonary stenosis, atrioventricular septal defect and situs inversus.

At day 6, the digestive malformation was operated (termino-terminal anastomosis, with appendicectomy). There was no common mesentery. At day 45, the baby was transferred to Paris (Necker Hospital) to perform intestinal anastomoses. After the surgery, the baby came back in good health.

 

The heart is located on the right hemithorax with dextrocardia. A complete atrioventricular septal defect is seen. The descending aorta is displaced on the right side.


Note the dextroposition of ascending aorta, which is larger than the main pulmonary artery (anterior located)


Note the two vessels emerging from the right ventricle. This ventricle was suggestive of a double-outlet right ventricle. Note the difference between aorta and pulmonary artery diameters.

Note the small main pulmonary artery and the dextroposition of ascending aorta. 


Sagittal view of the aorta and inferior vena cava


Lateral view of inferior vena cava

Transverse abdominal view showing the «double bubble»


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