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DGPK guidelineDouble Outlet Right Ventricle
(DORV)
H. Bertram, MHH, HannoverJ. Weil, UKE, Hamburg
J. Sachweh, UKE, Hamburg
DGPK guideline committee
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)
Guideline DORV Definition
Mahle WT et al. Cardiol Young 2008; 18(Suppl. 3): 39–51
> 150 % rule
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
spatial relationship of the semilunar cusps in hearts with DORV
Guideline DORV
Guideline DORV
Mahle WT et al. Cardiol Young 2008; 18(Suppl. 3): 39–51
‚Interventricular communication‘ vs ‚VSD‘
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)
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%)
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
Guideline DORVsubaortic VSD
parasternal long axis
AoAo
LV LA
Guideline DORVsubaortic VSD
Subcostal TEE
Ao
RV
PA
RV
RV
Ao
Ao
subaortic VSD with severe subpulmonary obstruction
Guideline DORVsubaortic VSD
PA
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
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
Guideline DORVsubpulmonary VSD
RV
RV
PA
PA
Guideline DORVsubpulmonary VSD
PA
RV
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
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
Ao
PARV
RV
LV
Guideline DORVnon-committed VSD
Guideline DORVnon-committed VSD
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
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
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
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‘
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
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
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
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
• Life-long follow-up by pediatric cardiologists and subsequently specialists for adult congenital heart disease is mandatory
Guideline DORV
Follow-up