Understanding Double Outlet Right Ventricle - Spectrum, Variations, and Subtypes

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. 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

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. eMedicine Subspecialties > Pediatrics > Cardiology • 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;166:925-928. • Royal Children's Hospital Melbourne, http://www.rch.org.au/cardiology/defects.cfm?doc_id=5087; 4/25/2007. • Lucile Packard Children's Hospital, http://www.lpch.org/diseaseHealthInfo/healthLibrary/cardiac/chd.html; 4/25/2007.