double outlet right ventricle (dorv) documents...•double outlet right ventricle is a spectrum of...
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Vita Zīdere, MD FRCPConsultant Paediatric and Fetal Cardiologist
Double outlet right ventricle(DORV)
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• Complex lesion, represents 3 % of CHD seen in fetus
• Both Great Arteries arise completely or predominantly from the morphological right ventricle
• Associates with increased NT, genetic and extracardiac anomalies
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• Double outlet right ventricle is a spectrum of abnormalities
• almost always has a VSD
• heterogeneous with respect to size and position of VSD and relationship of GA
• individual anatomy dictates surgical approach and outcome
• It poses a number of challenges
• Definition
• Anatomic Variability (incl. normal or abnormal AV connections)
• Surgical options
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DORV
“Simple” DORV
Always VSD:
subaortic,
subpulmonary or uncommitted; +/-PS
Complex DORV
Unbalanced Ventricles AVSD (often RAI or LAI)
MV atresia
Coarctation, IAA
Discordant AV connections Criss-cross heart
TOF type DORV
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Normal atrial situs Patent, concordant AV connections
DORV
Both GA (parallel) from RV
Subpulmonary, subaortic or uncommitted VSD
Repair depends from GA relationship with VSD
LV to Ao baffle ASO with baffling VSD to neo-Ao
Rarely BV repair is unfeasible
Overriding aorta
“50 % rule”
TOF type DORV
Anatomical repair-
VSD closure & PS release
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• True override is independent of septal axis• postnatally is assessed relative to chord of circle in short axis*
G P
rice
20
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*BR Wilcox, AC Cook, RH Anderson. Surgical anatomy of the heart,2004
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Double Outlet Right ventricle v. Tetralogy of Fallot
L R
• Perimembranous VSD, overriding aorta• Pulmonary artery < aorta (PA st)
R
L
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This is not a single diagnosis but a description of many different ones !
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• VSD closer to the aorta
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• Subpulmonary VSD
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• ?Noncommitted VSD
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• 2D echocardiography
• Echocardiographic “sweeps” used to determine
• Position of VSD
• Position of great arteries
• Straddling valves
• Outflow tract obstruction
Traditional approach
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Feature Normal heart DORV
4-chamber view Normal Normal/ VSD/straddling AV valves
LVOT Aorta directed towards right shoulder Highly variable
Relationship of GA Aorta and PA cross each other with PA anterior Highly variable: normally related, transposed
Size of GA Aorta similar to PA Highly variable: equal sized, Aorta larger than PA or vice versa
3-VTV Aortic arch and ductal arch meet in a “V” shape Aortic arch left or right sided
Echocardiographic features of DORV with normal atrioventricular connections
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AO PA
LV
MV
RV
Courtesy of JM Simpson
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AO
PA
RV
Courtesy of JM Simpson
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AO
PA
Courtesy of JM Simpson
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AO PA
Courtesy of JM Simpson
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Echocardiography 2013
UOG 2013
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Three-dimensional fetal echocardiography for prediction of postnatal surgical approach in double outlet right ventricle: a pilot study. Zidere et al, 2013 Ultrasound Obstet Gynecol
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Straddling mitral valve
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Courtesy of JM Simpson
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R R LLL
Vigneswaran TV, Jones CB, Zidere V, Charakida M, Miller OI, Simpson JM, Sharland GK. Effect of Prenatal Laterality Disturbance and Its Accompanying Anomalies on Survival. Am J Cardiol. 2018
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Associations
• Extracardiac and genetic anomalies
• 22q11.2 deletion
• aneuploidies
• e.g T13, T18
• VACTREL and CHARGE
Postnatal management according to anatomy
• Baffling of LV to aorta using a patch which also effects closure of VSD• enlargement of VSD may be needed
• Arterial switch operation and VSD closure
• Not possible to fully repair • Anatomy of VSD
• Size of ventricles and function of AV valves
• Position of great arteries
• Prognostication difficult
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• Systematic, thorough approach to all segments essential
• Understand the surgical considerations
• Some of the newer modalities may assist in difficult cases
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Published in October 2018