Double Outlet Right Ventricle Sireen T. Reddy. Topics of Discussion Spectrum of Disease Spectrum of...
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Transcript of Double Outlet Right Ventricle Sireen T. Reddy. Topics of Discussion Spectrum of Disease Spectrum of...
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
Spectrum of DiseaseSpectrum of Disease
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
Spectrum of DiseaseSpectrum of Disease
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
Spectrum of DiseaseSpectrum of Disease
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
Possible VariationsPossible Variations
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
Classification: Classification: ManifestationManifestation
Subaortic VSD without Subaortic VSD without Pulmonary StenosisPulmonary Stenosis
Classification: Classification: ManifestationManifestation
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
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
Subaortic VSD without Pulmonary Subaortic VSD without Pulmonary StenosisStenosis
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 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)
Subaortic VSD without Pulmonary Subaortic VSD without Pulmonary StenosisStenosis
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)
Classification: Classification: ManifestationManifestation
Subaortic VSD without Pulmonary Subaortic VSD without Pulmonary StenosisStenosis Pre-Dev. Pulmonary Vascular Pre-Dev. Pulmonary Vascular
Obstructive DiseaseObstructive Disease Post-Dev. Pulmonary Vascular Post-Dev. Pulmonary Vascular
Obstructive DiseaseObstructive Disease Subaortic VSD with Pulmonary Subaortic VSD with Pulmonary
StenosisStenosis
Subaortic VSD with Pulmonary Subaortic VSD with Pulmonary StenosisStenosis
Oxygenated Blood from LV Directed to Oxygenated Blood from LV Directed to AortaAorta
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
Oxygenated Blood from LV Directed to Oxygenated Blood from LV Directed to AortaAorta
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
Classification: Classification: ManifestationManifestation
Subaortic VSD without Pulmonary Subaortic VSD without Pulmonary StenosisStenosis Pre-Dev. Pulmonary Vascular Pre-Dev. Pulmonary Vascular
Obstructive DiseaseObstructive Disease Post-Dev. Pulmonary Vascular Post-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
Classification: Classification: ManifestationManifestation
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 Coronal Non-FIESTA Coronal Non-GatedGated
FIESTA Coronal Non-FIESTA Coronal Non-GatedGated
FIESTA Coronal Non-FIESTA Coronal Non-GatedGated
FIESTA Coronal Non-FIESTA Coronal Non-GatedGated
FIESTA Coronal Non-FIESTA Coronal Non-GatedGated
FIESTA Coronal Non-FIESTA Coronal Non-GatedGated
FIESTA Coronal Non-FIESTA Coronal Non-GatedGated
FIESTA Coronal Non-FIESTA Coronal Non-GatedGated
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.