Ebstein’s anomaly & Truncus Arteriosus


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Ebstein’s anomaly & Truncus Arteriosus

  1. 1. Dr Tarun Bhatnagar Consultant AnaesthesiologistNarayana Hrudayalaya, Ahmedabad dr.tarunbhatnagar@yahoo.co.in
  2. 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. 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. 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. 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. 6. 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
  7. 7. 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
  8. 8. 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
  9. 9.  Physical Examination CXR ECG Echo Cardiac Cath
  10. 10.  Cyanosis Active precordium Regurgitant holosystolic murmur Widely split S2 Gallop rhythm
  11. 11.  Massive Cardiomegaly due to enlarged right atrium Decreased pulmonary vasculature Small aortic root and main pulmonary artery shadow
  12. 12.  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
  13. 13.  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
  14. 14.  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
  15. 15.  Hypoxia & Acidosis cause pulmonary vasoconstriction !! while Hyperoxia & Alkosis causes pulmonary vasodilatation !!
  16. 16.  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
  17. 17. Indications Severe cyanosis(spO2< 80%) CHF Intractable Arrythmias due to accessory AV pathway Cardiomegaly (CTR ratio>0.65 on CXR) Paradoxical embolism
  18. 18. Critically ill neonates Non critical older children Aortopulmonary Shunt  ASD closure Palliative Sx ECMO  Tricuspid Valvuloplasty /Replacement Ortho topic Cardiac  Fontan Operation Transplantation
  19. 19.  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
  20. 20.  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)
  21. 21.  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)
  22. 22.  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
  23. 23.  Low CO Pulmonary insufficiency Residual TR Dysrhythmias-PSVT, VT,VF, Heart Block
  24. 24.  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.
  25. 25. Truncal valveVSD
  26. 26.  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
  27. 27.  Aortic Coarctation Type B interrupted Aortic Arch Persistent LSVC ASDs Digeorge Syndrome
  28. 28. Pulmonary Over circulation Coronary IschemiaAfter 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
  29. 29. Pulmonary Overcirculation Progressive Pulmonary Vascular DiseaseLethal Increase in PVR as early as 3rd mth of age
  30. 30. 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
  31. 31.  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
  32. 32.  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
  33. 33.  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
  34. 34.  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
  35. 35.  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
  36. 36.  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
  37. 37. Thanks