Double outlet right ventricle

1. Double outlet right ventricle
Hallmark: Aortomitral discontuity and 2 coni

2. DORV is cyanotic or acyanotic CHD?
Transposition of the great arteries Ebstein anomaly
Tetralogy of Fallot Eisenmenger physiology
Tricuspid atresia Critical pulmonary stenosis or atresia
Truncus arteriosus Functionally single ventricle
Total anomalous pulmonary venous return

3. For a layman
“Double outlet right ventricle is a
congenital heart defect. When a
baby is still in the womb,
something goes wrong during the
formation of the heart and both
great arteries are attached to the
right ventricle. No arteries, or only
a part of the aorta, are attached
to the left ventricle”.

4. Album
1703 • The earliest report in French
1793
• John Abernathy,2 an assistant surgeon at St Bartholomew’s Hospital in London, described
“partial transposition”
1898
• Karl von Vierordt3 called double outlet right ventricle partial transposition to signify that
the aorta was transposed but the pulmonary trunk was normally aligned
1957
• Witham4 introduced double outlet right ventricle as a diagnostic term for a
partial transposition complex

5. Definition
• 50% Rule(At least 50% each GA’s from RV)
• 200% rule(Both the GA’s completely from RV)
• Aortomitral discontinuity is must
[ To differentiate from TOF with significant aortic override but maintained aortomitral
continuity ]

6. References for definitions
1.Walters III HL, Mavroudis C, Tchervenkov CI, et al. Congenital Heart Surgery Nomenclature and Database Project: double outlet right
ventricle. Ann Thorac Surg 2000;69(Suppl. 4):63.
2. Becker AE, Anderson RH. Double outlet ventricles. In: Becker AE,Anderson RH, editors. Pathology of Congenital Heart Disease.London:
Butterworths; 1981. p. 297–307.
3. Howell CE, Ho SY, Anderson RH, Elliott MJ. Variations within the fibrous skeleton and ventricular outflow tracts in tetralogy of Fallot. Ann
Thorac Surg 1990;50:450–457.
4. Lev M, Bharati S, Meng CC, et al. A concept of double-outlet right ventricle. J Thorac Cardiovasc Surg 1972;64:271–281.
5. Van Praagh R. What is the Taussig-Bing malformation? Circulation 1968;38:445–449.
6. Bacha EA. Ventricular septal defect and double-outlet right ventricle. In: Sellke FW, del Nido PJ, Swanton SJ, editors. Sabiston & Spencer
Surgery of the Chest. 7th ed. Philadelphia: Elsevier Saunders; 2005. p. 1981–1997.
7. Jonas RA. Double outlet right ventricle. In: Jonas RA, DiNardo J,Laussen PC, et al., editors. Comprehensive Surgical Management of
Congenital Heart Disease. London: Arnold; 2004. p. 413–428.
8. Anderson RH, McCarthy K, Cook AC. Continuing medical education: double outlet right ventricle. Cardiol Young 2001;11:329–344.
9. Lacour-Gayet F. Intracardiac repair of double outlet right ventricle. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2008;39–43.

7. Epidemiology
 Isolated /With extracardiac anomalies
 Incidence:0.03 to 0.14 per 1000 live births
 1% of all CHD
 Association: coarctation, aortic arch hypoplasia, or interrupted aortic
arch—particularly at the transposition end of the spectrum, right
atrial isomerism
 Chromosomal abnormalities :Trisomy 13,trisomy 18, 22q11 deletion.

8. Where from 2 coni came?: Embryology window
Common arterial trunk arises from the RV-as the common trunk separates
into the two great vessels, they both arise from the RV for a period of time.
Regression of muscle between the aorta and the mitral valve results in the
aorta arising from the left ventricle in fibrous continuity with the mitral
valve. In some situations the muscle between the mitral and aortic valve
does not regress, resulting in what is known as a persistent left
ventriculoinfundibular fold (VIF). An alternative term is persistent left-sided
conus. A persistent left VIF can be, but is not necessarily,associated with
DORV.

9. Decision making for Rx
 Diagnosis is DORV
 Differentiate from TOF
 Location of the VSD
 Disposition of great arteries
 PS or no PS
 Size of VSD

10. Physiology
• Simple VSD
• Tetralogy of Fallot
• Transposition
• Single-ventricle type physiology

11. Location of VSD

12. VSD in DORV
 Double-outlet right ventricle (DORV) with subaortic VSD
Infundibular septum (IS) is attached to the anterior limb of the trabeculae septomarginalis
(TSM)
 DORV with subpulmonary VSD
IS is attached to the posterior limb (PL) of the TSM
 DORV with doubly committed VSD
Absent or virtually absent IS
 DORV with remote VSD
Here the VSD is in the inlet portion of the septum;remote VSDs also occur as muscular
VSDs unrelated to either great vessel

13. AL, anterior limb of TSM; Ao, aorta; PA, pulmonary artery;SVC, superior vena cava; TV, tricuspid
valve; VIF,ventricular infundibular fold (same as conus).

14. AL, anterior limb of TSM; Ao, aorta; PA, pulmonary artery;SVC, superior vena cava; TV,
tricuspid valve; VIF,ventricular infundibular fold (same as conus).

15. "Taussig Bing" anomaly”?

16. 4 major clinical patterns of DORV

17. Conduction Tissue
• AV node and bundle of His pathways follow the normal pathways for
• specific AV connections. The VSD in DORV is frequently in the
perimembranous location
• position and thereby is in jeopardy at the time of surgical
• repair at the margin of the tricuspid annulus and VSD closest to the
• crux of the heart. DORV associated with AV discordance has
conduction
• pathways that match the AV discordance, that is, anterior to the
• typical VSD where it is associated with the PA.

18. Investigation
• TTE
Most of the anatomic and physiologic
• TEE
Complex AV arrangements such as straddle or override.
• Cyanosis
? Decreased pulmonary blood flow
?Eisenmenger complex
 MRI
 Intracardiac anatomy
 GA’s relationship
 AV valves
 Cardiac catheterization
 PVD
 Coronary course

19. Electrocardiogram
• NSR
• PR Prolongation
• Atrial electrical abnormality
• Counter clock loop
• Clockwise QRS
• RVH
• Right axis deviation

20. Clinical classification
• The More Common Types
• A. Subaortic ventricular septal defect without pulmonary
• stenosis:
• a. Low pulmonary vascular resistance
• b. High pulmonary vascular resistance
• B. Subpulmonary ventricular septal defect without
• pulmonary stenosis:
• a. Low pulmonary vascular resistance
• b. High pulmonary vascular resistance
• The Less Common Types
• A. Doubly committed ventricular septal defect
• B. Uncommitted ventricular septal defect
• C. Intact ventricular septum

21. Common clinical presentation
• Subaortic ventricular septal defect, no pulmonary stenosis, low
pulmonary vascular resistance: resembles non-restrictive
perimembranous ventricular septal defect.
• Subaortic ventricular septal defect, no pulmonary stenosis,high
pulmonary vascular resistance: resembles Eisenmenger’s syndrome.
• Subaortic ventricular septal defect with pulmonary
stenosis:resembles Fallot’s tetralogy.
• Subpulmonary ventricular septal defect with no pulmonary stenosis:
resembles complete transposition of the great arteries with
nonrestrictive ventricular septal defect.

22. Surgery

23. Subaortic Ventricular Septal Defect
• Intraventricular tunnel repair (VSD to aorta)
• If VSD is more anterior-cephalward like TOF then
• TOF like repair can be done

24. Subpulmonary Ventricular Septal Defect
 Arterial switch
 Kawashima repair(tunnelling)

25. Doubly Committed Ventricular Septal Defect
• 4C repair may be attempted but at the cost of future intraventricular
obstruction

26. Right Atrioventricular Valve Overriding
and Straddling
• Palliation

27. Remote Ventricular Septal Defect
• Choice-1
Single-ventricle palliation for children with remote VSDs typically involve a PA
band as a neonate followed by a bidirectional cavopulmonary connection at
around 6 months of age. A Fontan procedure is typically performed between 18
months and 4 years of age.
Choice-2
Long intraventricular conduit into aorta at the cost of frequent
obstraction

28. DORV with Transposition-like Physiology and
Pulmonary Stenosis
• Rastelli repair
• REV procedure
The REV procedure (réparation à l’étage ventriculaire) entails division of the
main PA with extensive mobilization,translocation of the PA anterior to aorta
(Lecompte maneuver),and direct connection of the PA to the RV, thus
eliminating the use of prosthetic materials]
• Nikaidoh repair
The Nikaidoh procedure is an aortic root translocation procedure into the
enlarged pulmonary root position. A right ventricle-to-pulmonary artery (RV-PA)
conduit is then placed].

29. After surgery
• Subaortic obstruction
• Subpulmonary obstruction
• AV valve regurgitation
• Conduit failure (with stenosis and or regurgitation)
• Neoaortic regurgitation (following arterial switch procedures)
• Coarctation or recoarctation of the aorta
• Small or dysfunctional RV (may relate to complex intracardiac repair)
• Rhythm problems such as heart block, atrial arrhythmia, and
• ventricular arrhythmia
• Sudden death
• Endocarditis
• Thromboembolic phenomena

30. Arrhythmias and Sudden Cardiac Death
• That is always there with or without operation

31. Endocarditis Prophylaxis
• Should be

32. Pregnancy
• If Biventricular repair is
successful

33. “The future of pediatrics will be forged by thinking differently,
breaking paradigms and joining together in a shared vision of
tackling the toughest challenges before us.”
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