1. DGPK guideline Double Outlet Right Ventricle (DORV)
H. Bertram, MHH, Hannover J. Weil, UKE, Hamburg J. Sachweh, UKE, Hamburg DGPK guideline committee

2. Guideline DORV Definition
100 % %
100 % + > 50 %
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
prevalence: • 1,3 % of cardiac defects • 1,1 / live births (PAN)

3. Guideline DORV Definition
> 150 % rule
Mahle WT et al. Cardiol Young 2008; 18(Suppl. 3): 39–51

4. 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

5. spatial relationship of the semilunar cusps in hearts with DORV
Guideline DORV
spatial relationship of the semilunar cusps in hearts with DORV

6. Guideline DORV ‚Interventricular communication‘ vs ‚VSD‘
Mahle WT et al. Cardiol Young 2008; 18(Suppl. 3): 39–51

7. Guideline DORV Diagnostics
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)

8. 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%)

9. Guideline DORV 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

10. Guideline DORV subaortic VSD
parasternal long axis Ao Ao LV LA

11. Guideline DORV subaortic VSD
Subcostal
TEE RV Ao PA

12. Guideline DORV subaortic VSD
subaortic VSD with severe subpulmonary obstruction RV RV Ao PA Ao

13. 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

14. 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

15. Guideline DORV subpulmonary VSDPA RV PA

16. Guideline DORV subpulmonary VSDPA

17. Guideline DORV subpulmonary 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

18. Guideline DORV non-committed VSD
location and size; distance to semilunar valves
presence and severity of a subpulmonary obstruction
subcostal coronal / 4 C views LV Ao

19. Guideline DORV non-committed VSDPA LV Ao RV

20. Guideline DORV non-committed VSD

21. Double Outlet Right Ventricle
Guideline DORV
Double Outlet Right Ventricle
Malposition of the great arteries, which arise completely (100% + 100%) or with the greater part of their circumference (100% + > 50%) from the right ventricle
interventricular communication in relation to the great arteries
subaortic VSD
‚doubly committed‘
subpulmonary VSD
‚non committed‘
AVSD / heterotaxy
right atrial isomerism
TAPVD
l-SVC
subpulmonary obstruction
concommittant malformations
aortic coarctation (in ~ 50 %)
subpulmonary obstruction
valvular / subvalvular PS
valvular / subvalvular PS
clinical symptoms
pulmonary hyperperfusion; congestive heart failure
reduced lung perfusion; mild  severe cyanosis
severe cyanosis; congestive heart failure
clinical symptoms determined by concommittant malformations
clinical subtype
VSD - type
TOF - type
TGA - type
complex DORV
biventricular repair 2-6 years (complex intracardiac tunneling +/- VSD incision / arterial switch) or definitive univentricular palliation
biventricular repair 1-6 mo (VSDclosure)
biventricular repair 4-12 mo (VSD closure + relief of RVOTO)
biventricular repair < 1 mo (VSD-closure + arterial switch)
surgical strategy

22. Guideline DORV medical treatment catheter intervention
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

23. 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

24. Guideline DORV Surgical Repair
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
Subpulmonary VSD with valvular/subvalvular pulm. stenosis
‚Kawashima-OP‘ • ‚Rastelli-OP‘
‚REV-procedure‘ (Reparation a l‘ Etage Ventriculaire)
‚Aortic translocation‘ – ‚Nikaidoh-procedure‘

25. Guideline DORV Surgical Repair
Complex DORV
biventricular repair aged 2-6 years: complex intraventricular baffling (LV  Ao/PA) +/- VSD enlargement +/- arterial switch
definitive functionally univentricular palliation

26. Guideline DORV Surgical Repair
Implications of the 200 % rule (‚true‘ DORV)
Percent of Great Vessels Arising from the RV
DORV
200 %
> 150 %
technical difficulty
‚VSD – type‘ no
yes
simple
‚Fallot – type‘
average
‚TGA – type‘
important
DORV-AVSD
major
DORV ncVSD
Modified from: F. Lacour-Gayet: Intracardiac Repair of Double Outlet Right Ventricle Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann 2008;11:39-43

27. 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

28. Residual lesions requiring reinterventions after surgical repair
Guideline DORV
Residual lesions requiring reinterventions after surgical repair
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

29. Guideline DORV Follow-up
Life-long follow-up by pediatric cardiologists and subsequently specialists for adult congenital heart disease is mandatory