Double outlet right ventricle

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

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 [ T o 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 F allot 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

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