Double outlet right ventricleHallmark: Aortomitral discontuity and 2 coni

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

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”.

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

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 ]

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.

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.

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.

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

Physiology

• Simple VSD

• Tetralogy of Fallot

• Transposition

• Single-ventricle type physiology

Location of VSD

VSD in DORV

Double-outlet right ventricle (DORV) with subaortic VSDInfundibular septum (IS) is attached to the anterior limb of the trabeculae septomarginalis(TSM)

DORV with subpulmonary VSDIS is attached to the posterior limb (PL) of the TSM

DORV with doubly committed VSDAbsent or virtually absent IS

DORV with remote VSDHere the VSD is in the inlet portion of the septum;remote VSDs also occur as muscular VSDs unrelated to either great vessel

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

AL, anterior limb of TSM; Ao, aorta; PA, pulmonary artery;SVC, superior vena cava; TV,

tricuspid valve; VIF,ventricular infundibular fold (same as conus).

"Taussig Bing" anomaly”?

4 major clinical patterns of DORV

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.

Investigation

• TTEMost of the anatomic and physiologic

• TEEComplex 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

Electrocardiogram

• NSR

• PR Prolongation

• Atrial electrical abnormality

• Counter clock loop

• Clockwise QRS

• RVH

• Right axis deviation

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

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,highpulmonary 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.

Surgery

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

Subpulmonary Ventricular Septal Defect

Arterial switch

Kawashima repair(tunnelling)

Doubly Committed Ventricular Septal Defect

• 4C repair may be attempted but at the cost of future intraventricularobstruction

Right Atrioventricular Valve Overridingand Straddling• Palliation

Remote Ventricular Septal Defect

• Choice-1Single-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

DORV with Transposition-like Physiology and Pulmonary Stenosis

• Rastelli repair

• REV procedureThe 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 repairThe 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].

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

Arrhythmias and Sudden Cardiac Death

• That is always there with or without operation

Endocarditis Prophylaxis

• Should be

Pregnancy

• If Biventricular repair is successful

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