dgpk guideline double outlet right ventricle (dorv) h. bertram, mhh, hannover j. weil, uke, hamburg...
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DGPK guidelineDouble Outlet Right Ventricle
(DORV)
H. Bertram, MHH, HannoverJ. Weil, UKE, Hamburg
J. Sachweh, UKE, Hamburg
DGPK guideline committee
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Guideline DORV Definition
Double Outlet Right Ventricle (DORV) represents a spectrum of congenitally malformed hearts
in which the circumference of both arterial valves, or the greater part of both circumferences,
are supported by the right ventricle
100 % + 100 % 100 % + > 50 %
prevalence: • 1,3 % of cardiac defects • 1,1 / 10.000 live births (PAN)
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Guideline DORV Definition
Mahle WT et al. Cardiol Young 2008; 18(Suppl. 3): 39–51
> 150 % rule
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Guideline DORV Definition
Double Outlet Right Ventricle (DORV) represents a spectrum of congenitally malformed hearts
in which the circumference of both arterial valves, or the greater part of both circumferences,
are supported by the right ventricle
• same ventriculo-arterial connection, but variations in - infundibular morphology - arterial interrelationship - coronary arterial anatomy
• any arrangement of the atrial appendages, or situs
• any atrioventricular connection
• multiple combinations of associated malformations
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spatial relationship of the semilunar cusps in hearts with DORV
Guideline DORV
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Guideline DORV
Mahle WT et al. Cardiol Young 2008; 18(Suppl. 3): 39–51
‚Interventricular communication‘ vs ‚VSD‘
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Guideline DORVDiagnostics
Goal: displaying cardiac anatomy with emphasis on potential surgical biventricular repair (feasibility of tunneling the interventricular communication to one or other arterial trunk)
• position, size, interrelationship, course of the great arteries• morphology and size of the interventicular communication / the VSD in relation to diameter of the aortic valve• location and severity of a subpulmonary or subaortic obstruction• morphology and size of both ventricles and AV-valves
Methods:
• Echocardiography • Angiography (Cath./MRT/CT)
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Guideline DORV
Classification of the interventricular communication / VSD according to its location in relation to the great arteries
• subaortic (65%)
• subpulmonary (20-25%)
• doubly committed (3%)
• non committed (7%)
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subaortic VSD• size in relation to the diameter of the aorta• distance between VSD and aortic valve• presence and severity of a subpulmonary obstruction• subcostal coronal and sagittal planes; parasternal long axis
Guideline DORVsubaortic VSD
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Guideline DORVsubaortic VSD
parasternal long axis
AoAo
LV LA
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Guideline DORVsubaortic VSD
Subcostal TEE
Ao
RV
PA
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RV
RV
Ao
Ao
subaortic VSD with severe subpulmonary obstruction
Guideline DORVsubaortic VSD
PA
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Guideline DORV
doubly committed VSD• size and distance of the VSD to the aorta / pulmonary artery• presence and severity of a subpulmonary obstruction• subcostal coronal and sagittal planes
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Guideline DORV
subpulmonary VSD• size in relation to the diameter of the pulmonary artery• presence and severity of a subpulmonary obstruction• subcostal coronal and parasternal long axis planes
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Guideline DORVsubpulmonary VSD
RV
RV
PA
PA
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Guideline DORVsubpulmonary VSD
PA
RV
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Guideline DORVsubpulmonary VSD
Taussig-Bing malformation:
• DORV with subpulmonary VSD
• semilunar valves side-by-side
• no subpulmonary obstruction
• semilunar valves and AV-valves separated by conal septum
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Guideline DORV
non-committed VSD• location and size; distance to semilunar valves• presence and severity of a subpulmonary obstruction• subcostal coronal / 4 C views
LVAo
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Ao
PARV
RV
LV
Guideline DORVnon-committed VSD
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Guideline DORVnon-committed VSD
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TOF - type
subaortic VSD
Double Outlet Right Ventricle
valvular / subvalvular PS
biventricular repair 1-6 mo(VSDclosure)
Malposition of the great arteries, which arise completely (100% + 100%) or with the greater part of their circumference (100% + > 50%) from the right ventricle
‚doubly committed‘ ‚non committed‘subpulmonary VSD AVSD / heterotaxy
pulmonary hyperperfusion;congestive heart failure
reduced lung perfusion;mild severe cyanosis
TGA - typeVSD - type
severe cyanosis; congestive heart failure
biventricular repair < 1 mo(VSD-closure + arterial switch)
biventricular repair 4-12 mo(VSD closure + relief of RVOTO)
complex DORV
clinical symptoms determined by concommittant malformations
interventricular communication in relation to the great arteries
clinical symptoms
clinical subtype
biventricular repair 2-6 years(complex intracardiac tunneling
+/- VSD incision / arterial switch) or
definitive univentricular palliation
surgical strategy
concommittant malformations
aortic coarctation (in ~ 50 %)
• right atrial isomerism• TAPVD • l-SVC• subpulmonary obstruction
subpulmonary obstructionvalvular / subvalvular PS
Guideline DORV
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Guideline DORV
medical treatment• PG E in duct dependent pts with severe subpulmonary obstruction
• diuretics, ß-blockers, … in pts with pulmonary hypercirculation and congestive heart failure
catheter intervention• TOF-type: balloon valvuloplasty; ductal stent; RVOT stent
• TGA-type: BAS
surgical palliation• TOF-type: modified BT-shunt if primary repair is not suitable or considered ‚high risk‘
• PAB in ncVSD to delay complex intraventricular repair
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Guideline DORV Surgical Repair
VSD - type
• VSD closure in the age of 1 to 6 months - some pts need enlargement of the VSD (> 4/5 aortic annulus); cave: AV-Block
TOF - type
• VSD closure and relief of subpulmonary obstruction in the age of 4 to 12 months +/- muscular and transjunctional incision or patch enlargement
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TGA - type
• neonatal corrective surgery with arterial switch, VSD closure +/- resection of aortic coarctation / aortic arch reconstruction +/- resection of subaortic infundibulum cave: coronary artery anomalies
• alternatively ‚Rastelli - type repair‘: baffling of the left ventricle to both arterial valves and placement of a conduit from RV to the pulmonary trunk
Guideline DORV Surgical Repair
Subpulmonary VSD with valvular/subvalvular pulm. stenosis
• ‚Kawashima-OP‘ • ‚Rastelli-OP‘
• ‚REV-procedure‘ (Reparation a l‘ Etage Ventriculaire)
• ‚Aortic translocation‘ – ‚Nikaidoh-procedure‘
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Complex DORV
• biventricular repair aged 2-6 years: complex intraventricular baffling (LV Ao/PA) +/- VSD enlargement +/- arterial switch
• definitive functionally univentricular palliation
Guideline DORV Surgical Repair
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Guideline DORV Surgical Repair
Percent of Great Vessels Arising from the RV
DORV 200 % > 150 % technical difficulty
‚VSD – type‘ no yes simple
‚Fallot – type‘ no yes average
‚TGA – type‘ no yes important
DORV-AVSD yes yes major
DORV ncVSD yes yes major
Implications of the 200 % rule (‚true‘ DORV)
Modified from: F. Lacour-Gayet: Intracardiac Repair of Double Outlet Right Ventricle Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann 2008;11:39-43
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Guideline DORV
prognosis
• biventricular repair achievable in most pts
• increased operative risk determined by concommittant malformations: • aortic arch obstructions • AV-valve anomalies • coronary arterial anomalies • LV hypoplasia • multiple VSDs
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Residual lesions requiring reinterventions after surgical repair
Guideline DORV
• Depending on different morphology and type of previous surgical repair
• TOF – type: - pulmonary valve incompetence - residual subpulmonary obstruction
• RV-PA-conduit: definitive reoperation for conduit replacement (stenosis, incompetence, size-mismatch in growing children)
• complex intracardiac baffling: subaortic obstruction biventrcular surgical repair has a much higher rate of reintervention than a strategy of functionally univentricular palliation
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• Life-long follow-up by pediatric cardiologists and subsequently specialists for adult congenital heart disease is mandatory
Guideline DORV
Follow-up