A ppt presentation of two rare CHDs-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

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

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

12.  Physical Examination
 CXR
 ECG
 Echo
 Cardiac Cath

13.  Cyanosis
 Active precordium
 Regurgitant holosystolic murmur
 Widely split S2
 Gallop rhythm

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

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

23. Critically ill neonates Non critical older children
 Aortopulmonary Shunt  ASD closure
 Palliative Sx
 ECMO  Tricuspid Valvuloplasty
 /Replacement
Ortho topic Cardiac
 Fontan Operation
Transplantation

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)

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

29.  Low CO
 Pulmonary insufficiency
 Residual TR
 Dysrhythmias-PSVT, VT,VF, Heart Block

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.

33. Truncal valve
VSD

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

45. Pulmonary Overcirculation
Progressive Pulmonary Vascular Disease
Lethal Increase in PVR as early as 3rd mth of age

46. Physical Examination
 S/s of CHF : tachycardia, tachypnea, irritability,
poor feeding, recurrent pulmonary infections
 Jerky collapsing arterial pulse
 Systolic thrill over LSB
 Loud Ejection Click & holosystolic murmur
 Early diastolic murmur – if truncal valve
insufficiency present

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

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

55. Thanks