ORAL-MAXILLOFACIAL ENT DIVISION

LOGO

DOUBLE OUTLET RIGHT

VENTRICLE (DORV)

ROOM 311

DORV
Both great arteries arise from the morphologic right
ventricle
Results from bulboventricular malformations with
failure of proper alignment of the conotruncus with the
ventricular septum.
Complete characterization of the anatomy will include:
The relationship of the VSD to the great arteries;
The relationship of the great arteries with respect to
one another;
The morphology of the ventricles and their outflow
tracts
The presence of other associated anomalies.
2

CLASSIFICATION
Four different anatomic types of DORV are
defined based on the relationship of the
VSD to the great arteries:
Subaortic VSD with or without pulmonary
stenosis;
Subpulmonary VSD with or without subaortic
stenosis and/or arch obstruction;
doubly committed VSD;
non-committed VSD

PATHOPHYSIOLOGY
Basic physiologic subtypes are tetralogy of Fallot,
large VSD, and transposition of the great arteries.
DORV with pulmonary stenosis resembles the
physiology of tetralogy of Fallot with varying
degrees of cyanosis depending on the severity of
pulmonic stenosis.
The patients have right-to-left shunting across the
VSD and may have hypercyanotic spells,
polycythemia, and failure to thrive.

PATHOPHYSIOLOGY
DORV may be associated with multiple other
anomalies such as multiple VSD, atrioventricular
septal defects, PDA, aortic arch obstruction,
interrupted aortic arch, subaortic stenosis,
hypoplastic ventricle, as well as mitral valvar
abnormalities that may further affect the
physiology

ANESTHETIC MANAGEMENT

Depends on the specific type of DORV and

associated anomalies.
Common to all patients with DORV,
intracardiac shunting must be balanced
with manipulation of PVR and SVR to
optimize systemic CO and oxygen delivery.

Patient No.1
A 3.5 yrs old boy presented with CHD scheduled for
mouth preparation.
Known to have cardiac condition since age 3
months, later diagnosed as DORV. Born in normal
delivery. Active but easily fatigue especially in
exertion, cyanotic in circumoral and fingers.
Underwent PA banding procedure in general
anesthesia (RS Harapan Kita) at age 7 months
Physical examination : alert, BW 10 kg, HR 115 bpm,
RR 26 bpm, SpO2 78-80% room air, systolic murmur
on chest auscultation, circumoral cyanosis (+) esp.
when fatigue, clubbing finger (+)
7

Lab

Result

Hb

15.4

Ht

44

Leu

17300

Tro

392000

PT

17.6

INR

1.36

aPTT

37.4

Na

137

4.3

Ur

17

Cr

0.32

RBG

98

Echocardiography
Situs solitus
VA DORV malpose GA
All PV to LA
Large septectomy ASD, R> L shunt
Mitral atresia, LV hypoplasty
TR (-), good RV function, TAPSE 1.4 cm
VSD large mid muscular balance shunt
Ao right anterior to PA
PA banding tight, RV-PA trans banding gradient 97 mmHg
No left Ao coarctation
9

SVC

IVC

RA

RV

PA banding

PA
(to pulmonal)

PV

LA

LV
(hypoplastic
)
Ao (to systemic)

SpO2 78-80%

Mitral valve
atresia

ANESTHETIC PLAN
Main goal : intracardiac shunting must be
balanced with manipulation of PVR and
SVR to optimize systemic CO and oxygen
delivery.
Avoid increasing in PVR: avoid hypoxemia,
hypercarbia, hypovententilation, avoid
increase intrathoracal pressure
Avoid Decreasing SVR: Avoid hypovolume,
restricted vasodilator agent, adequate pain
management, avoid light anesthesia
11

ANESTHETIC PLAN
Main goal : intracardiac shunting must be
balanced with manipulation of PVR and
SVR to optimize systemic CO and oxygen
delivery.
Avoid increasing in PVR: avoid hypoxemia,
hypercarbia, hypovententilation, avoid
increase intrathoracal pressure
Avoid Decreasing SVR: Avoid hypovolume,
restricted vasodilator agent, adequate pain
management, avoid light anesthesia
12

ANESTHETIC PLAN

Fasting 6 hours pre op

IV fluid with RL 40 cc (20 dpm micro)overnight
Midazolam 1 mg iv prior to OR

INDUCTION :
Preoxygenation with O2 100%
Midazolam 2 mg iv
Ketamin 20 mg iv
Sevoflurane 2vol%, O2:air
Rocuronium 10 mg iv
Intubation with spiral ETT no 4.0
MAINTENANCE
Sevoflurane 2vol%, O2:air, fentanyl iv if needed

Emergence
Extubation fully awake
Post operative analgesia :
Paracetamol 3x200 mg iv

Patient No.2
An 8 yrs old girl presented with CHD scheduled for
mouth preparation.
Known to have cardiac condition since age 9
months, history of unconscious after exertion, later
diagnosed as DORV. Born in normal delivery. Active
but easily fatigue especially in exertion, cyanotic in
circumoral and fingers.
No history of prior operation, no history of allergy.
Physical examination : alert, BW 17 kg, HR 105 bpm,
RR 20 bpm, SpO2 75-76 % room air, systolic murmur
on chest auscultation, circumoral cyanosis (+) esp.
when fatigue, clubbing finger (+)
15

Lab

Result

Hb

21.4

Ht

63

Leu

12400

Tro

330000

PT

18

INR

1.43

aPTT

39.4

Na

136

4.2

Ur

27

Cr

0.39

RBG

85

Echocardiography
Situs solitus
Tricuspid atresia
RV hypoplasia
VSD subaortic
Double outlet right ventricle
Malposision of GA
Pulmonal stenosis infundibular valvular moderate PG 75
mmHg
Stretch PFO

17

SVC

PV

SpO2 78-80%

Stretch PFO

IVC

RA

RV
(hypoplastic)

PA stenosis

PA
(to pulmonal)

LA

LV

Ao (to systemic)

ANESTHETIC PLAN
Main goal : intracardiac shunting must be
balanced with manipulation of PVR and SVR to
optimize systemic CO and oxygen delivery.
Avoid increasing in PVR: avoid hypoxemia,
hypercarbia, hypovententilation, avoid increase
intrathoracal pressure
Avoid Decreasing SVR: Avoid hypovolume,
restricted vasodilator agent, adequate pain
management, avoid light anesthesia
Avoid infundibular spasm

19

ANESTHETIC PLAN

Fasting 6 hours pre op

IV fluid with RL 54 cc (16 dpm macro) overnight
Midazolam 1.5 mg iv prior to OR

INDUCTION :
Preoxygenation with O2 100%
Midazolam 3.5 mg iv, Ketamin 35 mg iv
Sevoflurane 2vol%, O2:air
Rocuronium 15 mg iv
Intubation with spiral ETT no 4.5
MAINTENANCE
Sevoflurane 2vol%, O2:air, fentanyl iv if needed
Vasoconstrictor agent (preferredly Norepinephrine) ready

Emergence
Extubation fully awake
Post operative analgesia :
Paracetamol 3x350 mg iv

Hypercyanotic spell
Management of hypercyanotic spell :
Oxygen 100%

increase SVR
Phenilephrine (5-10 mcg/kg)
NE 0.05-0.5 mcg/kg/min
Reduce PVR and improve PBF
morfin sulfat (0,05-0,1 mg/kg)
Reduce miocard contractility reduce spasme
infundibular with esmolol (0,5 mg/kg continue
with 0,3-0,5 mcg/kg/mnt)
Increase anesthesia depth

Thank you