1. Double Outlet Right Ventricle
Sireen T. Reddy

2. Topics of Discussion Spectrum of Disease Definition/Controversy
Variations
Key Subtypes

3. Most Common DORV Variant
Normal Heart
DORV, Subaortic VSD

4. Spectrum of Disease TGA + VSD
TOF
DORV
Tetralogy of Fallot
Pulmonary Outflow Obstruction
Right Ventricular Hypertrophy
Overriding Aorta
Ventricular Septal Defect

5. ToF vs. DORV
Tetralogy of Fallot
DORV, Subaortic VSD

6. Spectrum of Disease TGA + VSD
TOF
DORV
Transposition of the Great Arteries with Ventricular Septal Defect
Aorta arises from Right Ventricle
Pulmonary Artery arises from Left Ventricle
Ventricular Septal Defect

7. Transposition of the Great Arteries
TGA vs. DORV
Transposition of the Great Arteries
DORV, Subpulmonary VSD

8. Definition: Lev and associates
One Complete Arterial Trunk and At Least Half of the Other Arterial Trunk Arises from the Right Ventricle
May or May Not Be Mitral-Aortic or Mitral-Pulmonary Continuity
This last point refers to the fact that the mitral valve and aortic valve are normally in fibrous continuity.

9. Definition: Neufeld and associates
Both Great Arteries and Arterial Trunks Arise Exclusively from the Morphologic Right Ventricle
Neither Semilunar Valve is in Fibrous Continuity with Either Atrioventricular Valve
Usually a VSD is Present and Represents the Only Outlet from the Left Ventricle

10. Spectrum of Disease TGA + VSD
ToF
DORV
Strict Criteria
Excluding cases of Aortic/Pulmonary–Mitral continuity
Sharply Divides ToF and DORV
Does Not Reflect Normally Occuring Continuum of Disease (Gradual Diminution in Aortic-Mitral Continuity)

11. Spectrum of Disease TGA + VSD
ToF
DORV
Strict Criteria
Excluding cases of Aortic/Pulmonary–Mitral continuity
Sharply Divides TGA+VSD and DORV
Does Not Reflect Normally Occuring Continuum of Disease (Continuum of PA Arising from RV, to Overriding Septum, to Arising from LV)

12. Most Basic Definition
Aorta and Pulmonary Artery Arise Entirely or Predominately from the Right Ventricle

13. Relationship of the Great Arteries

14. Relationship of the Great Arteries
Right Posterior Aorta
Right Lateral Aorta
Right Anterior Aorta
Left Anterior Aorta

15. Positions of the Ventricular Septal Defect
Subaortic
Subpulmonary
Doubly Committed
Noncommitted (Remote)

16. Possible Variations

17. Possible Variations

18. Possible Variations

19. Coronary Anatomy Normal Abnormal, similar to ToF
LAD off RCA
Occurred in Patients with Subaortic VSD
Abnormal, Similar to TGA
RCA from Right Posterior Aortic Cusp
LCA from Left Posterior Aortic Cusp
Occurred in Patients with Subpulmonary VSD and Right Anterior Aorta (≈ D-TGA)

20. Classification: Manifestation
Subaortic VSD

21. Classification: Manifestation
Subaortic VSD without Pulmonary Stenosis

22. Classification: Manifestation
Subaortic VSD without Pulmonary Stenosis
Pre-Dev. Pulmonary Vascular Obstructive Disease
Post-Dev. Pulmonary Vascular Obstructive Disease

23. Subaortic VSD without Pulmonary Stenosis
Pre-Dev. Pulmonary Vascular Obstructive Disease
Oxygenated Blood from LV Directed to Aorta
Desaturated Systemic Blood Directed to PA
Little or No Cyanosis
Failure to Thrive
Large VSD * CHF

24. Subaortic VSD without Pulmonary Stenosis
Pre-Dev. Pulmonary Vascular Obstructive Disease
Oxygenated Blood from LV Directed to Aorta
Desaturated Systemic Blood directed to PA
Little or No Cyanosis
Failure to Thrive
Large VSD * CHF

25. Subaortic VSD without Pulmonary Stenosis
Post-Dev. Pulmonary Vascular Obstructive Disease
Oxygenated Blood from LV Directed to Aorta
Decrease Pulmonary flow
Desaturated Systemic Blood Redirected to Aorta
Cyanosis and Clubbing
Large VSD * CHF * Pulmonary HTN (Eisenmenger’s)

26. Subaortic VSD without Pulmonary Stenosis
Post-Dev. Pulmonary Vascular Obstructive Disease
Oxygenated Blood from LV directed to Aorta
Decrease Pulmonary flow
Desaturated Systemic Blood Shunts Across VSD
Cyanosis and Clubbing
Large VSD * CHF * Pulmonary HTN (Eisenmenger’s)

27. Classification: Manifestation
Subaortic VSD without Pulmonary Stenosis
Pre-Dev. Pulmonary Vascular Obstructive Disease
Post-Dev. Pulmonary Vascular Obstructive Disease
Subaortic VSD with Pulmonary Stenosis

28. Subaortic VSD with Pulmonary Stenosis
Oxygenated Blood from LV Directed to Aorta
Some Desaturated Systemic Blood Directed to Aorta
Cyanosis
Decreased Pulmonary Blood Flow
Px: Cyanosis, Failure to Thrive, Exertional Dyspnea, Squatting, Polycythemia
Tetralogy of Fallot

29. Subaortic VSD with Pulmonary Stenosis
Oxygenated Blood from LV Directed to Aorta
Some Desaturated Systemic Blood Directed to Aorta
Cyanosis
Decreased Pulmonary Blood Flow
Px: Cyanosis, Failure to Thrive, Exertional Dyspnea, Squatting, Polycythemia
Tetralogy of Fallot

30. Classification: Manifestation
Subaortic VSD without Pulmonary Stenosis
Pre-Dev. Pulmonary Vascular Obstructive Disease
Post-Dev. Pulmonary Vascular Obstructive Disease
Subaortic VSD with Pulmonary Stenosis
Subpulmonary VSD

31. Subpulmonary VSD AKA (Taussig-Bing Malformation)
Oxygenated Blood from LV Directed to PA
Desaturated Systemic Blood directed to Aorta
Severe Cyanosis
Increased Pulmonary Blood Flow
Px: Severe Cyanosis, Failure to Thrive
Transposition of the Great Arteries

32. Subpulmonary VSD AKA (Taussig-Bing Malformation)
Oxygenated Blood from LV Directed to PA
Desaturated Systemic Blood directed to Aorta
Severe Cyanosis
Increased Pulmonary Blood Flow
Px: Severe Cyanosis, Failure to Thrive
Transposition of the Great Arteries

33. Classification: Manifestation
Subaortic VSD without Pulmonary Stenosis
Pre-Dev. Pulmonary Vascular Obstructive Disease
Post-Dev. Pulmonary Vascular Obstructive Disease
Subaortic VSD with Pulmonary Stenosis
Subpulmonary VSD
Doubly-Committed or Non-Committed VSD

34. Doubly-Committed or Non-Committed VSD
Mixture of Oxygenated Blood and Desaturated Blood Directed to Both Outflow Tracts.
Mild Cyanosis
Increased Pulmonary Blood Flow
Ventricular Septal Defect

35. Doubly-Committed or Non-Committed VSD
Mixture of Oxygenated Blood and Desaturated Blood Directed to Both Outflow Tracts.
Mild Cyanosis
Increased Pulmonary Blood Flow
Ventricular Septal Defect

36. FIESTA Coronal Non-Gated

37. FIESTA Coronal Non-Gated

38. FIESTA Coronal Non-Gated

39. FIESTA Coronal Non-Gated

40. FIESTA Coronal Non-Gated

41. FIESTA Coronal Non-Gated

42. FIESTA Coronal Non-Gated

43. FIESTA Coronal Non-Gated

44. FIESTA Coronal Non-Gated

45. FIESTA Coronal Non-Gated

46. FIESTA Two Chamber

47. Post-Surgical DORV
Damus-Kaye Stansel
Modified Fontan

48. Further Reading
Moss and Adams' Heart Disease in Infants, Children, and Adolescents: Including the Fetus and Young Adult.
Double Outlet Right Ventricle, Normally Related Great Arteries.
eMedicine Subspecialties > Pediatrics > Cardiology
Double Outlet Right Ventricle, with Transposition.
Pediatric Cardiac Surgery.
Petiatric Cardiology for Practitioners.

49. References
Allen, Hugh D.; Clark, Edward B.; Gutgesell, Howard P.; and Driscoll, David J. Moss and Adams' Heart Disease in Infants, Children, and Adolescents: Including the Fetus and Young Adult. 6th ed., Philadelphia, Pa, Lippincott Williams, & Wilkins, 2001.
Mavroudis, Constantine; Barker, Carl L. Pediatric Cardiac Surgery. 3rd ed., Philadelphia, PA, Mosby, 2003.
Park, Myung. Petiatric Cardiology for Practitioners. 4th ed., St. Louis, Mo, Mosby, 2002.
Donnelly LF, Higgins CB, MR Imaging of Conotruncal Abnormalities. AJR Am J Roentgenol 1996;166:
Royal Children's Hospital Melbourne, 4/25/2007.
Lucile Packard Children's Hospital, 4/25/2007.