DOUBLE OUTLET RIGHT VENTRICLE (DORV)

DORV occurs when more than 50% of both the aorta and pulmonary artery arises from the right ventricle (1), secondary to maldevelopment of the conotruncus.
 

CLASSIFICATION
  1. DORV with a subaortic VSD (68%).
  2. DORV with a subpulmonary VSD (22%).
  3. DORV with both a subaortic and subpulmonic VSD (3%).
  4. DORV with a remote VSD (7%).

DORV + intact ventricular septum has been described but is very rare.


 

TYPES OF RELATIONSHIPS BETWEEN GREAT VESSELS
  1. Aorta is posterior to and to the right of the pulmonary artery.
  2. Aorta and pulmonary artery are parallel, with the aorta to the right (Taussig-Bing). Aorta posterior to pulmonary artery.
  3. Aorta and pulmonary are parallel, with the aorta anterior and to the left (levo-malposition).
  4. Aorta is right and anterior to the pulmonary artery (dextra-malposition)

 

ULTRASOUND

 

  • Aorta and Pulmonary arteries arise from RV (Parallel Great Vessels).
  • Aorta overriding the ventricular septum.
  • Aorta posterior / parallel / anterior to pulmonary artery.
  • VSD (100%).
  • Pulmonary stenosis (50%).

 

Aorta and PA both arise from the RV.

The aorta and PA are parallel to one another.

Other findings include: hypoplastic LV, VSD. Right sided aortic arch and absent pulmonary valve.

 

 

 

 

 

Video clip of Double Outlet Right Ventricle (DORV)

 

 

 

DIFFERENTIAL DIAGNOSIS
  • Transposition of the great vessels.
  • Tetralogy of Fallot.

 

ASSOCIATED CONDITIONS AND SYNDROMES

 

Link to Associated Conditions and Syndromes (1)


 

HEMODYNAMICS

The hemodynamics are dependent on the type of DORV and the associated anomalies. It does not cause cardiac failure.
 

 

 

REFERENCES
  1. Stewart PA, Wladimiroff JW, Becker AE. Early prenatal detection of double outlet right ventricle by echocardiography. Br Heart J 1985;54:340-342.