DOUBLE OUTLET RIGHT

VENTRICLE
BY NIKITHA V SHETTY
 DORV
• Definition.
• Pathopysiology
• Morphology
• Signs and symptoms.
• Types.
• Diagnosis.
• Treatment.
• Possible complication.
 DEFINITION

• The term DOUBLE OUTLET RIGHT

VENTRICLE describes heart in which more than
50% of each SEMILUNAR VALVES arises from the
morphologic RIGHT VENTRICLE.
• DOUBLE CONNI
• ABNORMALL CONAL
MORPHOLOGY.
OR
ABSENSE OF
AORTO-MITRAL
CONTINUITY.
 PATHOPHYSIOLOGY
• In normal heart structure, the
aortaconnected to left ventricle, the
chamber that pumps the blood to the
whole body.
• The pulmonary normally connect to the
right ventricle.
In DORV, both arteries flow out of
RIGHT VENTRICLE,this is a
problem because the right ventricle
has poor oxigenated blood, this blood
then circulated into the body.
 People with DORV has another
abnormalities
such as:
• Coarctation of the aorta.
• Mitral valve problem.
• Pulmonary atresia.
• Pulmonary stenosis.
• Right aortic arch.
• Trans position of the great
arteries.
 MORPHOLOGY
• Understanding of the relationship
between the great vessels and the
VSD.
• Anatomy of the Outlets to the great
vessels.
 SIGNS AND SYMPTOMS

• SHORTNESS OF BREATH • CLUBBING (thickness of Nail

bed) on Toes or Fingers
• HEART MURMUR
• SWEATING
• EXTREME TIREDNESS
• CYNOSIS
• WEIGHT LOSS
 DIAGNOSIS/INVESTIGATIONS
• ECG
• ECHO :Most of the ANATOMICAL and PHYSIOLOGICAL changes can
be visualised
• TEE : Complex AV arrangements such as OVERRIDE can be see.
• MRI : to see INTRACARDIAC ANATOMY,GA RELATIONSHIP,AV
VALVES
• CARDIAC CATHETERIZATION: To measure PRESSURE, CORONARY
COURSE
• CHEST XRAY
 TYPES OF DORV
Three main categories of DORV exist:
1. DORV WITH SUBAORTIC VSD

2. DORV WITH SUBPULMONARY VSD

3. DORV WITH NON COMMITTED VSD

4. DORV WITH DOUBLY COMMITTED

VSD.
CONCEPTS OF FLOW.
(Mixing V/s Streaming)
 ECG IN DORV
• ECG in DORV varies with clinical type as
• In DORV as RV is connected to the systemic
pressure,
Hence feature of RIGHT VENTRICULAR
HYPERTROPHY is almost uniformly present in all
types of DORV.
 ECG IN DORV
• As both the great vessels arise from Right
ventricle,a Ventricular septal defect is
obligatory.
• If the Ventricular septal defect is small we
can expect Pressure overloading of left
ventricle.
 ECG IN DORV
• When VSD is large, in absence of
pulmonary stenosis Pulmonary blood
flow is increased And there will be left
ventricular volume overload.
 ECG changes Are:
• PR PROLONGATION
• ATRIAL ELECTRICAL ABNORMALITY
• COUNTER CLOCK LOOP
• CLOCKWISE QRS
• RVH
• RIGHT AXIS DEVIATION
 ECHOCARDIOGRAPHY

• THIS IS THE MAINSTAY OF DIAGNOSIS.

• THE COMMITMENT OF THE SEMILUNAR VALVES TO THE
VENTRICLES IS ASCERTAINED.
• WHEN PRESENT DEVIATION OF THE OUTLET SEPTUM
BENEATH DEVELOPMENT OF THE GREAT VESSELS.
• PREOPERATIVE EVALUATION MUST ALSO TAKE INTO
ACCOUNT POTENTIAL AV VALVE ANOMALIES AND
STRADDLING IN PERTICULAR.
 1 : DORV with SUBAORTIC VSD

Where the aorta and it’s semilunar (aortic valve ) are

closest to or overriding, the trabecular septum.

• ANTERIOR DEVIATION OF THE OUTLET

SEPTUM CAUSES SUBPULMONARY STENOSIS
• THUS THE CLINICAL SCENARIO &
MANAGEMENT ARE SIMILAR OR IDENTICAL
TO THAT OF TOF
IF OUTLET SEPTUM IS DEVIATED
POSTERIORLY ,THERE WILL BE SUBAORTIC
STENOSIS OFTEN CO-EXISISTING
ABNORMALITY OF AORTIC ARCH

THE PRESENTATION AND MANAGEMENT

OF THIS VARIATION ARE ENTIRELY
DIFFERENT.
 ECG IN DORV WITH
SUBAORTIC VSD
• In Tetrology like DORV which has
SUBAORTIC VSD deffect with
PULMONARY STENOSIS the
ECG shows RIGHT AXIS
DEVIATION
ECG IN DORV WITH SUBAORTIC
VSD
• VSD like DORV with SUBAORTIC VSD with
NO PULMONARY STENOSIS
• This condition has LEFT VENTRICULAR
VOLUME OVERLOAD On ECG and LEFT
AXIS DEVIATION
SUBAORTIC VSD
 2.DORV with SUBPULMONARY VSD
OR
TAUSSING-BING ANOMOLY
• Can be considered along with TGA.
• This is because USUAL POSITION OF PULMONARY
ARTERY IS POSTERIOR AND LEFTWARD OF AORTA
• MEANS that streaming of Deoxygenated and Oxygenated
blood is similar to that of TGA even though most of the
PULMONARY VALVE IS CONNECTED TO RV.
• ANTERIOR DEVIATION of the
OUTLET SEPTUM causes SUBAORTIC
STENOSIS and AORTIC ANOMALIES.
• POSTERIOR DEVIATION of the
OUTLET SEPTUM causes
SUBPULMONARY STENOSIS and
LIMITS PULMONARY BLOOD FLOW
 ECG IN DORV WITH
SUBPULMONARY VSD
• TGA like DORV has SUBPULMONIC VSD
with out PULMONARY STENOSIS
• Here ECG shows Left Ventricular volume
overload and Right axis deviation.
• Left Ventricular volume overload manifest tall
R waves and Deep narrow Q waves in Lateral
Leads.
SUB PULMONIC VSD
(TAUSSINGBING)
DORV with DOUBLY COMMITTED VSD

• THIS MEANS THAT THERE

ARE 2 VSD’s ONE BELOW THE
AORTA AND ONE BELOW THE
PULMONARY ARTERY .
D
3.DORV WITH NON COMMITTED VSD

• THIS DEFINES HEARTS IN WHICH

THE VSD IS REMOTE FROM THE
OUTLETS.
• THE SURGICAL MANAGEMENT IN
THIS CASE IS PARTICULARLY
DIFFICULT.
 VAN PRAAGH CLASSIFICATION OF
DORV
• TYPE I DORV
- ISOLATED CONOTRUNCAL ANOMOLY.
• TYPE II DORV
-CONOTRUNCALL ANOMALY WITH MALFORMATIONS OF
AV VALVE & VENTRICLES
• TYPE III DORV
-HETROTAXY SYNDROMES
 ASSOCIATED LESIONS

• More than half of pacients with DORV have

associated anomalies of AV valves.
1. Mitral valve stenosis or Atresia associated
with hypoplastic LV (common)
2. Ebsteins Anomoly of Tricuspid valve.
 3. Complete AV Septal Defect.

4. Overriding Or Straddling of either AV

valve may occur.
 INDICATIONS FOR INTERVENTION

• The Goal of operative Treatment are to

ESTABLISH CONTINUITY BETWEEN THE
LEFT VENTRICLE and AORTA , CREATE
ADEQUATE RIGHT VENTRICLE TO
PULMONARY CONTINUITY AND REPAIR
ASSOCIATED LESION.
 In most cases

• COMPLETE REPAIR is performed as PRIMARY

PROCEDURE.
• In DORV with SUBAORTIC VSD REPAIR can be
accomplished by creating an INTRACELLULAR
BAFFLE that conducts LEFT VENTRICULAR
BLOOD into AORTA OR INTRAVENTRICULAR
TUNNEL REPAIR (VSD TO AORTA).
INTRAVENTRICULAR TUNNEL
REPAIR
• When the DORV with
SUBPULMONARY VSD without
SUBPULMONARY STENOSIS , Repair
is Accomplished by CLOSURE OF VSD
and ATRIAL SWITCH PROCEDURE.
 RASTELLI PROCEDURE

• In this cases ,THE AORTA IS

CONNECTED TO LV USING AN
INTRAVENTRICULAR BAFFLE AND A
RIGHT VENTRICLE TO PULMONARY
ARTERY CONDUIT IS PLACED TO
COMPLETE THE REPAIR.
 AFTER SURGERY.
• SUBAORTIC OBSTRUCTION
• SUBPULMONARY OBSTRUCTION
• AV VALVE REGURGITATION
• RHYTHM PROBLEMS SUCH AS HEART BLOCK
• ATRIAL ARRHYTHMIA
• VENTRICULAR ARRHYTHMIA
• SUDDEN DEATH
• ENDOCARDITIS
 POSSIBLE COMPLICATIONS.
• CONGESTIVE HEART FAILURE
(CHF)
• HIGH BLOOD PRESSURE IN LUNGS
• IRREVERSIBLE DAMAGE TO LUNGS
DUE TO UNTREATED HIGH BP.
 FOLLOW UP

• ALL OF THESE PATIENTS

REQUIRE AT LEAST ANNUAL
REVIEW BY CONGENITAL
CARDIOLOGIST.
THANK YOU