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Ebstein’s anomaly & Truncus Arteriosus
1. Dr Tarun Bhatnagar
Consultant Anaesthesiologist
Narayana Hrudayalaya, Ahmedabad
dr.tarunbhatnagar@yahoo.co.in
2. First described by Dr
William Ebstein in 1866
Rare disease, accounts for
0.5% of cases of CHDs
Ch by maldevelopment of
of tricuspid valve
Aetiology ?? maternal
Lithium ingestion
3. Cyanotic congenital heart diseases
Atrial septal defect (ASD)
Cases of severe right heart failure
Isolated severe tricuspid regurgitation
L-transposition of the great vessels
WPW /Preexcitation Syndrome
4. Wide spectrum of anatomic malformation
Wide spectrum of clinical presentation
Fetus- Hydrops
Neonate- Severe cyanosis with circulatory collapse
Child- Mild cyanosis
Adult- Minimal or no symptoms
5. Maldevelopment of Tricuspid Valve
Embryologically, TV is normally formed by
delamination or exfoliation of inner layer of RV
myocardium up towards the tricuspid annulus
If this process is arrested or incomplete, the
attachments of the leaflets are apically displaced
6.
7. Characteristic features
The septal & posterior leaflets are displaced
downwards or apically into the RV and are dysplastic
Anterior leaflet is, large, redundant (sail-like),
undisplaced & attached to the ventricular free wall
rather than to the papillary muscles
Atrialized RV (ARV)- proximal inlet portion of the RV
that is above the displaced leaflets but below the
annulus
Functional RV (FRV)- remaining part of RV that lies
below the displaced TV & is much smaller than usual
8.
9.
10. The predominant clinical finding is tricuspid insufficiency or
regurgitation that leads to Severe cyanosis due to
Dilated RA with R→L shunting thru an interatrial communication
(ASD or PFO)
Inadequate function of Distal RV - In severe cases the RV can’t
develop adequate force to open the pulmonary valve (Functional
Pulmonary Atresia)
Compromised LV filling by the dilated RV
Other causes of cyanosis due to ↓ Antegrade PBF
Anatomic pulmonary stenosis or atresia
Subpulmonary Obstruction due to abnormal TV tissue
Elevated PVR of neonatal period
11. Neonates
Cyanosis is the most common presentation in infancy
Cyanosis alone can improve by normal postnatal ↓ in PVR or by
conservative management
Neonates with severe TR- present with Cyanosis ,CHF , Metabolic
acidosis or Supraventricular tachyarrythmias
High Mortality rate in the neonatal group (20%)
Older children
DOE, fatigueability consistent with CHF
Supraventricular Tachyarrythmias
Neurological complications- CVA , Brain abscess
Infective Endocarditis
LV Dysfunction secondary to RV dilatation
14. Massive Cardiomegaly
due to enlarged right
atrium
Decreased pulmonary
vasculature
Small aortic root and
main pulmonary artery
shadow
15. RAD +Abnormal largeP waves consistent with right atrial
enlargement – “Himalayan P waves”
PR interval
◦ Commonly prolonged
◦ May be normal or short in patients with WPW syndrome
QRS complex
◦ RBBB
Rhythm
◦ Paroxysmal SVT, atrial flutter, atrial fibrillation, ventricular
tachycardia
16. Two-dimensional
◦ Displaced Septal & Posterior leaflet & the redundant
anterior leaflet are easily seen
◦ Apical displacement of the septal leaflet of greater than 8
mm/m2 - Most specific sign.
◦ Eccentric leaflet coaptation.
◦ Dilated right atrium.
◦ Delayed closure of tricuspid valve leaflet.
◦ Various left heart structural abnormalities
Color flow Doppler studies
◦ Varying degrees of tricuspid regurgitation
◦ R-L atrial shunting
◦ Status of pulmonary blood flow
17.
18. No longer required to make/confirm the diagnosis
The most diagnostic characteristic- Typical
atrial pressure & ventricular intracardiac ECG in
the atrialized portion of the RV
Elevated RAP
R-L atrial shunting with systemic desaturation
Elevated RVEDP
19. Hypoxia & Acidosis cause pulmonary
vasoconstriction !!
while
Hyperoxia & Alkosis causes pulmonary
vasodilatation !!
20. Measures to reduce PVR
-Mechanical Ventilation -High FiO2/ Hyperventillation /PEEP
-Sedation +Paralysis –avoids reflex ↑ in PVR secondary to noxious
stimuli
-Alkalosis-pulmonary vasodilator /pH7.50-7.60/ sodabicarb or
THAM
-Inhaled NO-starting dose 5-40ppm
PGE1 infusion- to keep the ductus arteriosus patent & maintain
the pulmonary blood flow
Correction of Metabolic Acidosis –improves myocardial function
Inotropes- Avoid Adrenaline as far as possible as it ↑es PVR
Avoid Overzealous Volume Infusions-can cause further annular
dilatation –worsen the TR
21. Indications
Severe cyanosis(spO2< 80%)
CHF
Intractable Arrythmias due to accessory AV
pathway
Cardiomegaly (CTR ratio>0.65 on CXR)
Paradoxical embolism
24. In neonates with severe Ebstein anomaly, the functional RV is
hypoplastic, and the patient is usually best treated by closing the
TVand creating a tricuspid atresia physiology
(Starnes procedure)
This strategy commits the neonates for future single ventricle
palliations like BDG or Fontan
Plication of Rt atrial tissue
+
Atrial Septectomy
+
Patch closure of tricuspid annulus (surgically creating tricuspid
atresia)
+
Insertion of Aortopulmonary shunt
25. Indication: Mild- Moderate Tricuspid insufficiency where
chordae & papillary muscles are intact
Median sternotomy/CPB
Surgery
-Patch closure of ASD/PFO +
-Plication of Atrialized RV +
-Plastic repair of TV+
-Posterior tricuspid annuloplasty+
-Rt atrial redundant tissue excision+
-Correction of any associated anomalies(PS / division of
accessory conduction pathways)
26.
27. Indication
Complete failure of
formation of TV with no
chordae or papillary
muscles
Disadvantage
-Higher incidence of
complications
-High chances of Redo
TVR (pts growth)
28. Major Concerns
↓CO, R→L shunting with cyanosis, Atrial tachyarrythmias
Anaesthetic Goals
Minimizing PVR, Optimizing RV preload, Maintain RV contractility to
optimize PBF
Continue PGE1 infusion untill the completion of Aortopulmonary shunt
Induction
Primary Narcotic based technique+ Pancuronium
Atrial tachyarrythmias Rx
External Cardioversion-0.5J/kg in hemodynamically unstable pts
Adenosine 0.1mg/kg for PSVT
Monitoring
Invasive Arterial & CVP
Atrial & Ventricular pacing wires
32. Uncommon lesion, 1.4% CHDs
Ch by single arterial trunk arising from both
ventricles due to the failure of truncus
arteriosus to divide into the Aorta & PA
Commonly assoc with a VSD & Coronary
anomalies
Most infants present with CHF during the first 2
weeks; 85% of untreated children die by 1 year
of age.
42. Truncus
-Longer than normal aorta
-Overrides the ventricular septum more towards RV
-The Coronaries & one or two PAs arise from it
-Branch PA stenosis when present protect pul vasculature from
pulmonary overcirculation & pulmonary vascular disease
Truncal Valve
-Dysmorphic, stenotic, regurgitant
-Tricuspid(60-70%), quadrcuspid(25%), bicuspid(5%)
Coronary artery Anomalies(50%)
Clinically imp variations are
-High origin of LCA: makes it vulnerable to surgical injury when explanting
the PAs)
- Large infundibulae or anterior descending artery crossing the RVOT
making it vulnerable at the time of RVOT conduit placement
VSD
- large, lies immediately beneath the truncal valve
43. Aortic Coarctation
Type B interrupted Aortic Arch
Persistent LSVC
ASDs
Digeorge Syndrome
44. Pulmonary Over circulation Coronary Ischemia
After the 1st/2nd wk of life PVR falls Pulmonary Circulation Runoff
→Qp/Qs >1
during diastole
↓ ↓
Pulmonary Overcirculation Low systemic diastolic pressure
↓ ↓
Volume Overload (LV) + Pressure Low coronary perfusion
Overload (RV)
pressure
↓ ↓
CHF Coronary Ischemia & Impaired
myocardial function
↑
Truncal Valve Insufficiency
47. CXR
Cardiomegaly, ↑ Pulmonary vascular markings
ECG
BVH
Echo- diagnostic
-Single great artery with a semilunar valve that overrides the
ventricular septum & is continuity with the mitral valve
-Enlarged LA
-Origins of PAs
-Conotruncal VSD
-Competence of truncal valve
- Caliber of aortic arch
Cardiac Cath
- Indicated in older infants with significant PVD
-Type IV truncus to delineate MAPCAs & PA anatomy
- SaO2<84% indicates significant PVD the child may not
tolerate the operative correction
48. Sx is recommended within the first 2 mths of life as PVD
develops by 3-6 mths of age
Infants with CHF are mx by fluid restriction, diuretics ,
digitalis & afterload reduction
Infants with persistent severe CHF- Complete repair
immediately
Surgery is not recommended
-Fixed PVD with PVR > 8 Woodunits/m2
Surgery is recommended
-Reactive PVD responding to O2 & hyperventillation
49. Airway abnormalities
-Small mouth, micrognathia: Difficult Intubation
CHF
-occur due to torrential PBF that can be exacerbated by hyperoxia &
hypocapnia
-FiO2 of 0.21 + Ventilatory adjustment to maintain SaO2 of 85-95% &
pCO2 of 40mmHg is desirable
-Afterload reduction (Milrinone) may improve Systemic CO & reduce
PBF
Myocardial Ischemia
- can occur before & after induction of anaesthesia
-↑ myocardial wall tension on LV due to volume overload
↑ diastolic runoff to pulmonary circulation
- Mx by Temporary PA banding followed by Complete repair once BP &
ECG settles
50. CPB-Deep Hypothermia-Cardioplegic arrest
The PAs are removed from the truncus- site is oversewn
with a running suture
VSD is closed allowing the LV to eject thru the truncal
valve to the aorta
A valved conduit (cryopreserved valved pulmonary or
aortic homograft) is placed from the RV into the distal
main PA
Moderate truncal insufficiency –Valve repair+
commissural annuloplasty
Severe truncal insufficiency- Valve replacement
51.
52.
53. Pulmonary Hypertensive Crisis
-Commonly occur in 3-6 mths old infants after truncus repair
-Ch by hypotension, bradycardia & cyanosis
-Triggered by hypoxia , hypercapnia, acidosis, pain airway stimulation
,LVF
- Rule out Large residual VSD before medical mx
- Mx by Mechanical ventilation/NMB/Sedation
- Refactory cases-ECMO
Low Cardiac Output
Causes: RV failure (m/c) due to Rt ventriculotomy & wide swings in
PVR
Others -Truncal valve insufficiency, inadequate myocardial protection ,
Coronary artery compression
- Mx by optimizing preload, Adjusting ventilation to reduce airway
pressures, Inotropes/Inodilator
Major Late Complication – Obstruction or Stenosis of Conduit
54. Early Truncus repair (within 6 wks) at University of
California Sanfrancisco (UCFS) have achieved
86% survival in their series of 244 pts since1975
All children were followed carefully to watch for
the development of complications