Double Outlet Right Ventricle Sireen T. Reddy. Topics of Discussion Spectrum of Disease Spectrum of Disease Definition/Controversy Definition/Controversy

Double Outlet Double Outlet Right VentricleRight Ventricle

Sireen T. ReddySireen T. Reddy

Topics of DiscussionTopics of Discussion

Spectrum of DiseaseSpectrum of Disease Definition/ControversyDefinition/Controversy VariationsVariations Key SubtypesKey Subtypes

Most Common DORV Most Common DORV VariantVariant

Normal Heart DORV, Subaortic VSD

Spectrum of DiseaseSpectrum of Disease

Tetralogy of Fallot Pulmonary Outflow Obstruction Right Ventricular Hypertrophy Overriding Aorta Ventricular Septal Defect

DORVTOFTOFTGA

+VSD

ToF vs. DORVToF vs. DORVTetralogy of Fallot DORV, Subaortic VSD


Transposition of the Great Arteries with Ventricular Septal Defect

Aorta arises from Right Ventricle Pulmonary Artery arises from Left Ventricle Ventricular Septal Defect

DORVTOFTGATGA

++VSDVSD

TGA vs. DORVTGA vs. DORVTransposition of the Great

ArteriesDORV, Subpulmonary VSD

Definition: Lev and Definition: Lev and associatesassociates

One Complete Arterial Trunk and At One Complete Arterial Trunk and At Least Half of the Other Arterial Least Half of the Other Arterial Trunk Arises from the Right Trunk Arises from the Right VentricleVentricle

May or May Not Be Mitral-Aortic or May or May Not Be Mitral-Aortic or Mitral-Pulmonary ContinuityMitral-Pulmonary Continuity

Definition: Neufeld and Definition: Neufeld and associatesassociates

Both Great Arteries and Arterial Both Great Arteries and Arterial Trunks Arise Exclusively from the Trunks Arise Exclusively from the Morphologic Right VentricleMorphologic Right Ventricle

Neither Semilunar Valve is in Neither Semilunar Valve is in Fibrous Continuity with Either Fibrous Continuity with Either Atrioventricular ValveAtrioventricular Valve

Usually a VSD is Present and Usually a VSD is Present and Represents the Only Outlet from the Represents the Only Outlet from the Left VentricleLeft Ventricle


Strict CriteriaStrict Criteria Excluding cases of Aortic/Pulmonary–Mitral continuityExcluding cases of Aortic/Pulmonary–Mitral continuity Sharply Divides Sharply Divides ToF and DORVToF and DORV Does Not Reflect Normally Occuring Continuum of Does Not Reflect Normally Occuring Continuum of

Disease (Gradual Diminution in Aortic-Mitral Continuity)Disease (Gradual Diminution in Aortic-Mitral Continuity)

DORVToFToFTGA

+VSD


Strict CriteriaStrict Criteria Excluding cases of Aortic/Pulmonary–Mitral Excluding cases of Aortic/Pulmonary–Mitral

continuitycontinuity Sharply Divides Sharply Divides TGA+VSD and DORVTGA+VSD and DORV Does Not Reflect Normally Occuring Continuum of Does Not Reflect Normally Occuring Continuum of

Disease (Continuum of PA Arising from RV, to Disease (Continuum of PA Arising from RV, to Overriding Septum, to Arising from LV)Overriding Septum, to Arising from LV)

DORVToFToFTGA

+VSD

Most Basic DefinitionMost Basic Definition

Aorta and Pulmonary Artery Arise Aorta and Pulmonary Artery Arise Entirely or Predominately from the Entirely or Predominately from the Right VentricleRight Ventricle

Relationship of the Great Relationship of the Great ArteriesArteries

Relationship of the Great Relationship of the Great ArteriesArteries

Right Posterior Right Posterior AortaAorta

Right Lateral Right Lateral AortaAorta

Right Anterior Right Anterior AortaAorta

Left Anterior Left Anterior AortaAorta

SubaorticSubaortic

SubpulmonarySubpulmonary

Doubly Doubly CommittedCommitted

Noncommitted Noncommitted (Remote)(Remote)

Positions of the Ventricular Positions of the Ventricular Septal DefectSeptal Defect

Possible VariationsPossible Variations



Coronary AnatomyCoronary Anatomy

NormalNormal Abnormal, similar to ToFAbnormal, similar to ToF

LAD off RCALAD off RCA Occurred in Patients with Subaortic VSDOccurred in Patients with Subaortic VSD

Abnormal, Similar to TGAAbnormal, Similar to TGA RCA from Right Posterior Aortic CuspRCA from Right Posterior Aortic Cusp LCA from Left Posterior Aortic CuspLCA from Left Posterior Aortic Cusp Occurred in Patients with Subpulmonary Occurred in Patients with Subpulmonary

VSD and Right Anterior Aorta (≈ D-TGA)VSD and Right Anterior Aorta (≈ D-TGA)

Classification: Classification: ManifestationManifestation

Subaortic VSDSubaortic VSD


Subaortic VSD without Subaortic VSD without Pulmonary StenosisPulmonary Stenosis


Subaortic VSD without Subaortic VSD without Pulmonary StenosisPulmonary Stenosis Pre-Dev. Pulmonary Vascular Pre-Dev. Pulmonary Vascular

Obstructive DiseaseObstructive Disease Post-Dev. Pulmonary Vascular Post-Dev. Pulmonary Vascular

Obstructive DiseaseObstructive Disease

Subaortic VSD without Pulmonary Subaortic VSD without Pulmonary StenosisStenosis

Pre-Dev. Pulmonary Vascular Obstructive Pre-Dev. Pulmonary Vascular Obstructive DiseaseDisease

Oxygenated Blood from LV Directed to Oxygenated Blood from LV Directed to AortaAorta

Desaturated Systemic Blood Directed to PADesaturated Systemic Blood Directed to PA Little or No CyanosisLittle or No Cyanosis Failure to ThriveFailure to Thrive

Large VSD * CHFLarge VSD * CHF


Pre-Dev. Pulmonary Vascular Obstructive Pre-Dev. Pulmonary Vascular Obstructive DiseaseDisease


Desaturated Systemic Blood directed to PADesaturated Systemic Blood directed to PA Little or No CyanosisLittle or No Cyanosis Failure to ThriveFailure to Thrive

Large VSD * CHFLarge VSD * CHF


Post-Dev. Pulmonary Vascular Obstructive Post-Dev. Pulmonary Vascular Obstructive DiseaseDisease


Decrease Pulmonary flowDecrease Pulmonary flow Desaturated Systemic Blood Redirected to Desaturated Systemic Blood Redirected to

AortaAorta Cyanosis and ClubbingCyanosis and Clubbing

Large VSD * CHF * Pulmonary HTN Large VSD * CHF * Pulmonary HTN (Eisenmenger’s)(Eisenmenger’s)


Post-Dev. Pulmonary Vascular Obstructive Post-Dev. Pulmonary Vascular Obstructive DiseaseDisease

Oxygenated Blood from LV directed to Oxygenated Blood from LV directed to AortaAorta

Decrease Pulmonary flowDecrease Pulmonary flow Desaturated Systemic Blood Shunts Across VSDDesaturated Systemic Blood Shunts Across VSD Cyanosis and ClubbingCyanosis and Clubbing

Large VSD * CHF * Pulmonary HTN Large VSD * CHF * Pulmonary HTN (Eisenmenger’s)(Eisenmenger’s)


Subaortic VSD without Pulmonary Subaortic VSD without Pulmonary StenosisStenosis Pre-Dev. Pulmonary Vascular Pre-Dev. Pulmonary Vascular


Obstructive DiseaseObstructive Disease Subaortic VSD with Pulmonary Subaortic VSD with Pulmonary

StenosisStenosis

Subaortic VSD with Pulmonary Subaortic VSD with Pulmonary StenosisStenosis


Some Desaturated Systemic Blood Some Desaturated Systemic Blood Directed to AortaDirected to Aorta CyanosisCyanosis Decreased Pulmonary Blood FlowDecreased Pulmonary Blood Flow

Px: Cyanosis, Failure to Thrive, Px: Cyanosis, Failure to Thrive, Exertional Dyspnea, Squatting, Exertional Dyspnea, Squatting, PolycythemiaPolycythemia

Tetralogy of FallotTetralogy of Fallot

Subaortic VSD with Pulmonary Subaortic VSD with Pulmonary StenosisStenosis


Some Desaturated Systemic Blood Some Desaturated Systemic Blood Directed to AortaDirected to Aorta CyanosisCyanosis Decreased Pulmonary Blood FlowDecreased Pulmonary Blood Flow

Px: Cyanosis, Failure to Thrive, Px: Cyanosis, Failure to Thrive, Exertional Dyspnea, Squatting, Exertional Dyspnea, Squatting, PolycythemiaPolycythemia

Tetralogy of FallotTetralogy of Fallot


Subaortic VSD without Pulmonary Subaortic VSD without Pulmonary StenosisStenosis Pre-Dev. Pulmonary Vascular Pre-Dev. Pulmonary Vascular


Obstructive DiseaseObstructive Disease Subaortic VSD with Pulmonary Subaortic VSD with Pulmonary

StenosisStenosis Subpulmonary VSDSubpulmonary VSD

Subpulmonary VSDSubpulmonary VSD

AKA (Taussig-Bing Malformation)AKA (Taussig-Bing Malformation) Oxygenated Blood from LV Directed to Oxygenated Blood from LV Directed to

PAPA Desaturated Systemic Blood directed to Desaturated Systemic Blood directed to

AortaAorta Severe CyanosisSevere Cyanosis Increased Pulmonary Blood FlowIncreased Pulmonary Blood Flow

Px: Severe Cyanosis, Failure to ThrivePx: Severe Cyanosis, Failure to Thrive

Transposition of the Great ArteriesTransposition of the Great Arteries

Subpulmonary VSDSubpulmonary VSD

AKA (Taussig-Bing Malformation)AKA (Taussig-Bing Malformation) Oxygenated Blood from LV Directed to Oxygenated Blood from LV Directed to

PAPA Desaturated Systemic Blood directed to Desaturated Systemic Blood directed to

AortaAorta Severe CyanosisSevere Cyanosis Increased Pulmonary Blood FlowIncreased Pulmonary Blood Flow

Px: Severe Cyanosis, Failure to ThrivePx: Severe Cyanosis, Failure to Thrive

Transposition of the Great ArteriesTransposition of the Great Arteries


Subaortic VSD without Pulmonary Subaortic VSD without Pulmonary StenosisStenosis Pre-Dev. Pulmonary Vascular Obstructive Pre-Dev. Pulmonary Vascular Obstructive

DiseaseDisease Post-Dev. Pulmonary Vascular Obstructive Post-Dev. Pulmonary Vascular Obstructive

DiseaseDisease Subaortic VSD with Pulmonary StenosisSubaortic VSD with Pulmonary Stenosis Subpulmonary VSDSubpulmonary VSD Doubly-Committed or Non-Committed Doubly-Committed or Non-Committed

VSDVSD

Doubly-Committed or Non-Doubly-Committed or Non-Committed VSDCommitted VSD

Mixture of Oxygenated Blood and Mixture of Oxygenated Blood and Desaturated Blood Directed to Both Desaturated Blood Directed to Both Outflow Tracts.Outflow Tracts.

Mild CyanosisMild Cyanosis Increased Pulmonary Blood FlowIncreased Pulmonary Blood Flow

Ventricular Septal DefectVentricular Septal Defect

Doubly-Committed or Non-Doubly-Committed or Non-Committed VSDCommitted VSD

Mixture of Oxygenated Blood and Mixture of Oxygenated Blood and Desaturated Blood Directed to Both Desaturated Blood Directed to Both Outflow Tracts.Outflow Tracts.

Mild CyanosisMild Cyanosis Increased Pulmonary Blood FlowIncreased Pulmonary Blood Flow

Ventricular Septal DefectVentricular Septal Defect

FIESTA Coronal Non-FIESTA Coronal Non-GatedGated










FIESTA Two ChamberFIESTA Two Chamber

Post-Surgical DORVPost-Surgical DORV

Damus-Kaye StanselDamus-Kaye Stansel Modified Fontan Modified Fontan

Further ReadingFurther Reading Moss and Adams' Heart Disease in Infants, Moss and Adams' Heart Disease in Infants,

Children, and Adolescents: Including the Fetus and Children, and Adolescents: Including the Fetus and Young Adult.Young Adult.

Double Outlet Right Ventricle, Normally Related Double Outlet Right Ventricle, Normally Related Great Arteries.Great Arteries.

eMedicine Subspecialties > Pediatrics > CardiologyeMedicine Subspecialties > Pediatrics > Cardiology Double Outlet Right Ventricle, with Transposition.Double Outlet Right Ventricle, with Transposition.

eMedicine Subspecialties > Pediatrics > CardiologyeMedicine Subspecialties > Pediatrics > Cardiology Pediatric Cardiac Surgery.Pediatric Cardiac Surgery. Petiatric Cardiology for Practitioners.Petiatric Cardiology for Practitioners.

ReferencesReferences Allen, Hugh D.; Clark, Edward B.; Gutgesell, Howard P.; Allen, Hugh D.; Clark, Edward B.; Gutgesell, Howard P.;

and Driscoll, David J. and Driscoll, David J. Moss and Adams' Heart Disease Moss and Adams' Heart Disease in Infants, Children, and Adolescents: Including the in Infants, Children, and Adolescents: Including the Fetus and Young AdultFetus and Young Adult. 6. 6thth ed., Philadelphia, Pa, ed., Philadelphia, Pa, Lippincott Williams, & Wilkins, 2001.Lippincott Williams, & Wilkins, 2001.

Mavroudis, Constantine; Barker, Carl L. Mavroudis, Constantine; Barker, Carl L. Pediatric Pediatric Cardiac SurgeryCardiac Surgery. 3. 3rdrd ed., Philadelphia, PA, Mosby, 2003. ed., Philadelphia, PA, Mosby, 2003.

Park, Myung. Park, Myung. Petiatric Cardiology for PractitionersPetiatric Cardiology for Practitioners. . 44thth ed., St. Louis, Mo, Mosby, 2002. ed., St. Louis, Mo, Mosby, 2002.

Donnelly LF, Higgins CB, MR Imaging of Conotruncal Donnelly LF, Higgins CB, MR Imaging of Conotruncal Abnormalities. AJR Am J Roentgenol 1996;166:925-928.Abnormalities. AJR Am J Roentgenol 1996;166:925-928.

Royal Children's Hospital Melbourne, Royal Children's Hospital Melbourne, http://www.rch.org.au/cardiology/defects.cfm?doc_id=508http://www.rch.org.au/cardiology/defects.cfm?doc_id=50877; 4/25/2007.; 4/25/2007.

Lucile Packard Children's Hospital, Lucile Packard Children's Hospital, http://www.lpch.org/diseaseHealthInfo/healthLibrary/cardihttp://www.lpch.org/diseaseHealthInfo/healthLibrary/cardiac/chd.htmlac/chd.html; 4/25/2007.; 4/25/2007.

Documents

Double Outlet Right Ventricle Sireen T. Reddy. Topics of Discussion Spectrum of Disease Spectrum of Disease Definition/Controversy Definition/Controversy