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DR.RAHUL ARORA
1ST YEAR PDT
MANAGEMENT
DIAGNOSIS
TREATMENT
 CLINICAL EXAMINATION
 INVESTIGATIONS
 ECG
 CHEST X RAY
 ECHOCARDIOGRAPHY
 CARDIAC CATHERIZATION
 CARDIAC CT SCAN
...
O/E :
o cyanosis ; clubbing ;
o Arterial pulses …normal in uncomplicated TOF;
o Wide pulse pressure (arterial diastolic ru...
ECG
 QRS axis … same as that of a normal newborn
 RVH…Tall monophasic R in V1 with an abrupt change to an
rS pattern in ...
CXR:
 Normal sized heart; [may be large in PA]
 upturned apex; attenuated & concave left heart border
(infundibular and ...
ECHO :
PLAX :
 VSD; degree of aortic override;
 AR;
 Cranial tilt of the transducer…..infundibulum and the proximal
pul...
 3D transthoracic echo (combined with a semiautomatic
border detection algorithm) for measuring right
ventricular volumes...
64-slice HRCT :
Questions about preoperative pulmonary artery
anatomy, coronary artery anatomy, and systemic or
pulmonary ...
Cardiac Catheterization
 The focus of catheterization has shifted from making the
diagnosis to filling in missing informa...
After surgical repair
 to evaluate hemodynamically significant residual
lesions, right ventricular outflow tract
obstruct...
MANAGEMENT
 What are the factors that decide treatment?
 Pallative treatment
 Types
 Indications
 Complications
 Definitive tre...
 TOF is a progressive disorder
 most infants require surgery
Timing of complete surgical repair is
dependent on numerou...
Medical Management
 ductal-dependent pulmonary blood flow ?......neonates
with critically restricted antegrade blood flow...
TOF WITH PULMONARY STENOSIS
TOF WITH PULMONARY ATRESIA
TOF WITH ABSENT PULMONARY
VALVE
Goal
to increase pulmonary blood flow independent of
ductal patency and to allow pulmonary artery
growth and even total co...
 MODIFIED BLALOCK TAUSSING SHUNT
 CLASSICAL BLALOCK TAUSSING SHUNT
 WATERSTON‟S SHUNT
 POTT‟S SHUNT
 Ascending aorta with right PA
 No longer performed because of high surgical
complication
Potts shunt
•Descending aorta ...
 To maintain the patency of the shunt, initial post
op days use heparin followed by aspirin until
corrective surgery.
 T...
Something for interventionalist……………………
Interventional procedures :
 In patients with severe annular hypoplasia…..
palliation of significant cyanosis by balloon
...
Balloon angioplasty of the pulmonary valve
annulus is preferable to a shunt procedure as it is
 less traumatic,
 it avoi...
 Coil embolization of APCs ….Coiling of vessels that
perfuse pulmonary segments already supplied by
pulmonary arterial fl...
Definitive Surgery aims @
 relieving all possible sources of RVOTO;
 If possible, pulmonary valve function is preserved ...
 To relieve RVOTOpulmonary valvotomy, the
insertion of an outflow tract patch or a transannular
patch are often required...
SURGERY in TOF contd…
VSD closure : Done thru’ RA approach whether or not a trans-
annular patch is used, as this approach...
 Low birth weight
 Pulmonary artery atresia
 Major associated anomalies
 Multiple previous surgeries
 Absent pulmonar...
Contraindications to primary repair in tetralogy
of Fallot include the following:
 The presence of an anomalous coronary ...
Advantages of early total correction
 Prevents cerebral hypoxia, cerebral embolism,
abscess and hematological changes
 D...
TOF
(with favourable
RVOT anatomy)
TOF (with
coronary
anamolies)
B-T Shunt (±)
VSD closure +
RV – PA
conduit(>1yr)
VSD clo...
SURGERY IN TOF + PA :
1. Depends on PA anatomy and collateralization
(cath study) & presence of MAPCAs
2. Stages : complet...
The complexity of pulmonary blood supply determines the extent of
surgical exploration necessary to perform unifocalizatio...
PA anatomy assessment
To quantitate the degree of PA hypoplasia
1) McGoon ratio: (Diameter of RPA/DAo + Diameter of
LPA/DA...
3) Total Neo-Pulmonary Artery Index (TNPAI) = APC
index + Nakata Index
 APC index is the sum of CSA of all usable
APCs/BS...
 Classification of PA - VSD according to the status of
native pulmonary arteries (NPAs), aorto-pulmonary
 collaterals (A...
 Neonates with adequate-sized confluent pulmonary arteries
may be amenable to primary definitive surgical repair.
 A pal...
 When the pulmonary arteries are hypoplastic, nonconfluent,
and supplied by aortopulmonary collaterals, a multistaged
rep...
For complete repair to be performed in a child
who has undergone palliation:
(1) The central pulmonary arterial area must ...
 Some centers have shifted toward performing a single-
stage repair, wherein all the multiple aortopulmonary
collaterals ...
MAPCAs : management : 2 options
1)Obliterate them by Sx ligation/coil embolisation
2)Surgical unifocalisation : if it is t...
 To assess whether this is likely, surgeons at the
University of California in San Francisco perfuse
blood from the perfu...
Confluent PA
with favourable
PA anatomy
Hypoplastic
PAs
central Shunt RV – PA connection
+ unifocalization +
VSD closure
S...
Post Repair for TOF in general
 Current surgical survival, even for symptomatic
infants <3 months of age, is excellent.
...
Late complications
• Repair
– Pulmonary regurgitation:
 d/t transannular patch repair technique
 well tolerated if mild ...
 – Residual right ventricular outflow tract (RVOT)
obstruction:
 occur at the infundibulum,
 at the level of the pulmon...
– Residual ventricular septal defect (VSD): residual VSDs can be
encountered from either partial patch dehiscence or failu...
Supraventricular arrhythmia: atrial fl utter and atrial fibrillation
are relatively common in the current cohort of adults...
Problems that may develop include
a)Residual VSDs
 Persistent defects at the patch margin or
 Previously unrecognized or...
c)Progressive aortic root dilation &AR :
 Intrinsic dvptal abnormalities of aortic valve/root
 Palliative shunts or sign...
e) LV dysfunction … consequence of adverse
ventricular interaction.
f) Rhythm disturb./SCD : Long-term mortality …. 3%
to ...
Post repair TOF + PA :
 Need for reoperation/ transcatheter balloon dilation
and stenting of an obstructed conduit in RV ...
TOF + AVCD :
 risk for atrioventricular valve incompetence …. TR right
ventricular dilation, and dysfunction.
Congenital Absence of Pulmonary Valve Syndrome
 Chever in 1847
 incompletely formed, rudimentary pulmonary valve
that ty...
 Typically present in the neonatal period, and are
diagnosed based on the presence of their
characteristic murmur and the...
 Some neonates present with severe bronchial obstruction
and require immediate tracheal intubation and mechanical
ventila...
Outcome of TOF + APV depends largely on the severity of
airway disease.
Even after Sx,some pts continue to have bronchial ...
 Surgical repair is only definitive treatment.
 Palliative surgery is supportive.
 Definitive repair to be done as earl...
THANK YOU
PVR : Early PVR in selected patients results in
beneficial remodelling of the right ventricle
Optimal timing is critical f...
Hypoplastic PAs … intervene early …. Encourage
them to grow
 Reconstruction of RVOT with a patch or valveless
conduit
 P...
Surgical management of tetralogy of fallot
Surgical management of tetralogy of fallot
Surgical management of tetralogy of fallot
Surgical management of tetralogy of fallot
Surgical management of tetralogy of fallot
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Surgical management of tetralogy of fallot

include diagnosis and management of tof

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Surgical management of tetralogy of fallot

  1. 1. DR.RAHUL ARORA 1ST YEAR PDT
  2. 2. MANAGEMENT DIAGNOSIS TREATMENT
  3. 3.  CLINICAL EXAMINATION  INVESTIGATIONS  ECG  CHEST X RAY  ECHOCARDIOGRAPHY  CARDIAC CATHERIZATION  CARDIAC CT SCAN  CARDIAC MRI
  4. 4. O/E : o cyanosis ; clubbing ; o Arterial pulses …normal in uncomplicated TOF; o Wide pulse pressure (arterial diastolic runoff)…. aortopulmonary collaterals, palliative surgical shunt or PDA; o Accentuated precordial RV impulse; o LV impulse will not be hyperactive (normal cardiac output); o S2 single& loud (anterior, dextroposed aorta); o S3/S4 are unusual; o Aortic ejection click; o Systolic murmur…crescendo-decrescendo @ LUSB. The intensity of the murmur inversely parallels the degree of pulmonic obstruction; o Diastolic murmurs are unusual. Rarely AR murmur; o TOF with PA…..no harsh, obstructive precordial murmurs; o A harsh diastolic murmur, with a harsh murmur of PS, [harsh sawing, to-and-fro murmur ] ……TOF and APV syndrome; o Continuous murmurs….PDA;aortopulmonary collaterals ;may be best heard in the back.
  5. 5. ECG  QRS axis … same as that of a normal newborn  RVH…Tall monophasic R in V1 with an abrupt change to an rS pattern in V2 (Tall R extends into adj precordial leads in TOF + APV)  Reduced PBF+ underfilled LVrS in V2-V6  Balanced shunt …qR in V5,V6  L-R shuntQR in V5,V6  LAD with counterclockwise depolarisn TOF+AVSD
  6. 6. CXR:  Normal sized heart; [may be large in PA]  upturned apex; attenuated & concave left heart border (infundibular and PA hypoplasia)….boot-shaped heart, or coeur en sabot…small underfilled LV that lies above horiz IVS, inferior to which is a concentric hypertrophied nondilated RV  Diminished pulmonary vascularity in proportion to the degree of cyanosis.  Absent thymic shadow in the newborn may indicate associated chromosome 22q11.2 microdeletion (DiGeorge syndrome).  RAA in roughly 25%...accompanied by Rt DA on Rt side  In PA..lacy reticular pattern (d/t the anast b/w lobar/segm PAs & CAs)  Syst arterial collaterals rarely cause rib notching as they do not run in intercostal grooves.
  7. 7. ECHO : PLAX :  VSD; degree of aortic override;  AR;  Cranial tilt of the transducer…..infundibulum and the proximal pulmonary arteries. [location and degree of infundibular, valvular, and arterial hypoplasia] Basal SAX:  RVOT and proximal pulmonary arteries…degree of PS….PDA?  VSD [just below the RCC, or at 10 o'clock position]. Tricuspid-aortic continuity confirms the perimembranous nature.  Coronaries…origin and course … echo showed sensitivity of 82%, specificity of 99%, and accuracy of 98.5% in a study by Need et al high parasternal and suprasternal views will provide visualization of the pulmonary arteries and aorta, respectively
  8. 8.  3D transthoracic echo (combined with a semiautomatic border detection algorithm) for measuring right ventricular volumes and ejection fractions has good agreement with MRI. 3D echo has reduced accuracy with larger Right ventricular volumes.  MRI, axial slices offer greater reproducibility for right and left ventricular end-diastolic volumes and end- systolic volumes than do short-axis slices.  Subject to significantly less intraobserver variation.  Postoperative PS and PR and also RV volumes better assessed by MRI than Echo. High quality imaging of MRI can replace angiography.
  9. 9. 64-slice HRCT : Questions about preoperative pulmonary artery anatomy, coronary artery anatomy, and systemic or pulmonary venous anatomy can frequently be resolved even in newborns with 64-slice high-resolution CT scans. Computerized Tomography is infrequently used for evaluation of TOF. Surgical complication like infection or pseudoaneurysm formation can be detected with CT scan. Helical CT scanning can be used to identify airway compression caused by a large ascending aorta of TOF.
  10. 10. Cardiac Catheterization  The focus of catheterization has shifted from making the diagnosis to filling in missing information in the diagnosis such as the hemodynamic data regarding pulmonary blood supply.  Other specific questions unanswered by echocardiography such as: 1) coronary anatomy; 2) aorto-pulmonary collateral arteries (number, size, distribution, any stenosis and blood pressure in each collateral vessel); 3) confirmation of presence or absence of native PAs and a retrograde pulmonary vein wedge injection if needed to identify their presence if not clear on aortography; 4) number of lung segments connected to native Pas; and 5) lung segments with dual blood supply.
  11. 11. After surgical repair  to evaluate hemodynamically significant residual lesions, right ventricular outflow tract obstruction and  residual ventricular septal defects, in patients who are unstable or otherwise unable to wean from support.
  12. 12. MANAGEMENT
  13. 13.  What are the factors that decide treatment?  Pallative treatment  Types  Indications  Complications  Definitive treatment  Factors  Risk factors  Indication  Types of surgery  Complications  Early  Late  Follow up
  14. 14.  TOF is a progressive disorder  most infants require surgery Timing of complete surgical repair is dependent on numerous variables  symptoms and  any associated lesions (eg, multiple ventricular septal defect [VSD], pulmonary atresia)  Current trend is ----- before the age of 1 year and preferably by the age of 2 years.  Studies have shown that surgery is preferably done at or about 12 months of age.  The majority of patients born with tetralogy of Fallot now thrive well into their adult years.[18]
  15. 15. Medical Management  ductal-dependent pulmonary blood flow ?......neonates with critically restricted antegrade blood flow need to be started on prostaglandin E1and considered for either total repair or a systemic-to-pulmonary shunt.  Most newborns with TOF do not have ductal-dependent pulmonary blood flow and may be followed without specific early intervention.  Parental education for recognition of cyanotic spells [even acyanotic TOF are at risk for the development of spells in the first months of life].  IE Prophylaxis for cyanotic/palliated patients ; patients for 6 months after patch repair surgery, and those with prosthetic valves
  16. 16. TOF WITH PULMONARY STENOSIS TOF WITH PULMONARY ATRESIA TOF WITH ABSENT PULMONARY VALVE
  17. 17. Goal to increase pulmonary blood flow independent of ductal patency and to allow pulmonary artery growth and even total correction. INDICATIONS 1. Neonates with TOF & Pulmonary atresia 2. Children with Hypoplastic pulmonary artery 3. Infants with Hypoplastic pulmonary annulus 4. Age less than 3 months who have medically unmanageable hypoxic spells 5. Infants weighing less than 2.5 kg 6. Abnormal coronary artery anatomy
  18. 18.  MODIFIED BLALOCK TAUSSING SHUNT  CLASSICAL BLALOCK TAUSSING SHUNT  WATERSTON‟S SHUNT  POTT‟S SHUNT
  19. 19.  Ascending aorta with right PA  No longer performed because of high surgical complication Potts shunt •Descending aorta with left PA. •No longer performed
  20. 20.  To maintain the patency of the shunt, initial post op days use heparin followed by aspirin until corrective surgery.  The mortality rate - less than 1%  few complications  acute shunt thrombosis,  hypoplasia of the arm,  digital gangrene,  phrenic nerve injury, and  pulmonary artery stenosis.  The longevity of palliation after shunt placement varies according to the patient's age at the time of surgery and the type of shunt.
  21. 21. Something for interventionalist……………………
  22. 22. Interventional procedures :  In patients with severe annular hypoplasia….. palliation of significant cyanosis by balloon valvuloplasty or RVOT stent placement can be done… Improvement in antegrade flow is thought to simultaneously enhance pulmonary arterial growth by augmenting PBF. A study by Robinson et al found that intraoperative balloon valvuloplasty is associated with significant longitudinal annular growth, with normalization of annular size over time. This technique may be most useful in patients with moderate pulmonary stenosis and moderate pulmonary valve dysplasia.[21]
  23. 23. Balloon angioplasty of the pulmonary valve annulus is preferable to a shunt procedure as it is  less traumatic,  it avoids a thoracotomy,  reduces the likelihood of distortion of the pulmonary arteries  However, this must be done with caution if the outflow obstruction is predominantly at the valve, dilation can result in an acute ventricular left to right shunting pulmonary overcirculation.[36–41]
  24. 24.  Coil embolization of APCs ….Coiling of vessels that perfuse pulmonary segments already supplied by pulmonary arterial flow serves to reduce LV volume loading as well as to eliminate runoff into the pulmonary arterial bed during CPB
  25. 25. Definitive Surgery aims @  relieving all possible sources of RVOTO;  If possible, pulmonary valve function is preserved by avoiding a transannular patch  Closure of VSD (dacron patch) INDICATIONS 1. O2 saturation less than 75-80% 2. Cyanotic spells 3. Mildly cyanotic infants who have had previous shunt surgery mayhave total repair 1-2 yrs after shunt operation TIMING Optimal Today, most centres prefer early repair for PA growth and preservation of right ventricular function
  26. 26.  To relieve RVOTOpulmonary valvotomy, the insertion of an outflow tract patch or a transannular patch are often required.  Surgery during early infancy, when the pulmonary annulus is markedly stenotic, frequently requires the insertion of a long and wide transannular patch.  Consequently, most patients acquire PR as a result of the repair. PR may be well tolerated by many in the early postoperative years, but in the long term chronic PR is associated with reduced exercise capacity, RV dilatation, ventricular arrhythmias, and sudden death.
  27. 27. SURGERY in TOF contd… VSD closure : Done thru’ RA approach whether or not a trans- annular patch is used, as this approach allows to minimise the length of the right ventriculotomy (length only necessary to relieve the RVOT obstruction and not for the VSD exposure). Efficacy of the RA approach ….  Preserves the right ventricular function, …..  Resultant PR after limited transannular patching is less severe than that which occurs after transventricular repair  Less incidence of ventricular/atrial arrhythmias  Easier to preserve the integrity & function of the tricuspid valve. Concerns of right ventriculotomy (classical RV approach)  Low cardiac output in the early postoperative period,  a higher incidence of arrhythmias,  Risk of late sudden death
  28. 28.  Low birth weight  Pulmonary artery atresia  Major associated anomalies  Multiple previous surgeries  Absent pulmonary valve syndrome  Young or old age  Severe annular hypoplasia  Small pulmonary arteries  High peak RV–to–left ventricular pressure ratio  Multiple VSDs  Coexisting cardiac anomalies
  29. 29. Contraindications to primary repair in tetralogy of Fallot include the following:  The presence of an anomalous coronary artery  Very low birth weight  Small pulmonary arteries  Multiple VSDs  Multiple coexisting intracardiac malformations
  30. 30. Advantages of early total correction  Prevents cerebral hypoxia, cerebral embolism, abscess and hematological changes  Decrease RVPprevent persistent myocardial hypertrophy and probably reduce the risk of fibrosis of the RV  Providing adequate PBF will optimize the opportunity for normal growth of the main and branch PAs…also normal pulmonary circulation may be important for lung development.  Tendency for progressive hypertrophy of the RV infundibular region is largely abolished by early repair.
  31. 31. TOF (with favourable RVOT anatomy) TOF (with coronary anamolies) B-T Shunt (±) VSD closure + RV – PA conduit(>1yr) VSD closure (±) transannular patch ( as early as 3-4 mo; & by 1-2 yr ) VSD closure + Widening of RVOT + Small RV- PA conduit(>1yr) TETRALOGY OF FALLOT B-T Shunt (±)
  32. 32. SURGERY IN TOF + PA : 1. Depends on PA anatomy and collateralization (cath study) & presence of MAPCAs 2. Stages : complete repair or staged operation 3. If PA anatomy appears amenable to reconstruction, procedures leading to complete repair are indicated. 4. Palliative procedures…PA Anatomy unfavourable… Central shunt vs reconstruction of RVOT using a patch/conduit while leaving open the VSD 5. If there is a connection between RV & PT  correction can be achieved with a patch reconstruction
  33. 33. The complexity of pulmonary blood supply determines the extent of surgical exploration necessary to perform unifocalization. Eligibility for complete repair is dependent on this since the RV-PA conduit needs to be placed to the vessel which is connected to maximum possible pulmonary vascular bed. Furthermore, closing the VSD at the time of placement of RV – PA conduit needs to be determined. Adequacy of the pulmonary vascular bed and the pulmonary vascular resistance are the determinants of postoperative RV pressure which in turn has been closely correlated with surgical outcome. At least 10 – 16 lung segments need to be connected to the RV-PA conduit in order to have satisfactory hemodynamic result after complete repair9. If the central native PAs were not identified on echo, it is prudent to demonstrate them angiographically. Furthermore, a simultaneous contrast injection into the proximal stump of the pulmonary artery and the pulmonary vein wedge injection will help to define the length of discontinuity that need to be “bridged” surgically during repair10. Preoperative evaluation of adequacy of pulmonary artery size is difficult because of under filling of PAs and therefore, the potential size of these PAs after surgical repair is unpredictable.
  34. 34. PA anatomy assessment To quantitate the degree of PA hypoplasia 1) McGoon ratio: (Diameter of RPA/DAo + Diameter of LPA/DAo)  Normal 2.1  Adequate for VSD closure 1.2  Inadequate <0.8 for VSD closure 2) Nakata Index:(CSA of RPA + CSA of LPA)/BSA  Normal value > 200 mm2/m2 > 150 mm2/m2 is adequate. (Not usable preoperatively when MAPCAs are the major source of PBF & one-stage unifocalization + full repair is planned).
  35. 35. 3) Total Neo-Pulmonary Artery Index (TNPAI) = APC index + Nakata Index  APC index is the sum of CSA of all usable APCs/BSA >250 - suitable for one-stage repair including VSD closure (These pts. have low RV/LV pressure ratio postoperatively). Critique of all these indices: These indices consider only the size of proximal vessel and not consider the condition of distal parts of the vessels (which may be stenosed). Recently the value of all these has been questioned
  36. 36.  Classification of PA - VSD according to the status of native pulmonary arteries (NPAs), aorto-pulmonary  collaterals (APCs) and patent ductus arteriosus (PDA).  Type A: Native pulmonary arteries present, no APCs.  Type B: Native pulmonary arteries and APCs present.  Type C: No native pulmonary arteries, only APCs maintain pulmonary blood flow
  37. 37.  Neonates with adequate-sized confluent pulmonary arteries may be amenable to primary definitive surgical repair.  A palliative procedure with a systemic–to–pulmonary artery shunt may be performed while awaiting complete repair at a later date.  The ultimate surgical goals are: (1) to incorporate as many pulmonary artery segments as possible into a pulmonary artery confluence, (2) to place a conduit from the right ventricle to the pulmonary artery confluence, and (3) to close the ventricular septal defect (VSD).  While the primary intervention in the majority of patients is surgical, selected cases may be amenable to transcatheter perforation of the right ventricular outflow tract followed by balloon dilation.[20]
  38. 38.  When the pulmonary arteries are hypoplastic, nonconfluent, and supplied by aortopulmonary collaterals, a multistaged repair is often required.[21]  Hypoplastic pulmonary arteries generally require palliative shunting to induce enlargement and growth of these vessels so they can be successfully incorporated into the complete repair.  The shunts used may be modified Blalock-Taussig or central shunts and may be unilateral or bilateral.  Another important strategy to maximize the long-term outcome in this group of patients is the early unifocalization of as many of the aortopulmonary collaterals as possible into a central pulmonary artery confluence.[22, 23, 24, 25] This maximizes the recruitment of lung segments and increases the likelihood of performing the definitive repair.
  39. 39. For complete repair to be performed in a child who has undergone palliation: (1) The central pulmonary arterial area must be greater than 50% of normal; (2) predominantly left-to-right intracardiac shunting must be present; (3) the equivalent of an entire lung must be supplied by the central pulmonary artery confluence; and (4) stenotic lesions in the pulmonary artery outflow must be addressed.
  40. 40.  Some centers have shifted toward performing a single- stage repair, wherein all the multiple aortopulmonary collaterals (MAPCAs) are ligated at the aorta.[31, 32]These MAPCAs are then mobilized toward the posterior mediastinum to construct a pulmonary artery confluence, followed by insertion of a pulmonary allograft to establish continuity between these neopulmonary arteries and the right ventricle. The ventricular septal defect (VSD) is closed.  good results.  Infants with postunifocalization pulmonary arteries that, combined, are only mildly hypoplastic (> 200 mm2/m2) have a lower mortality rate and acceptable right ventricular pressures.  require repeat catheterizations for balloon dilation or stent placements in stenotic pulmonary artery segments to alleviate elevated right ventricular pressures.[33]
  41. 41. MAPCAs : management : 2 options 1)Obliterate them by Sx ligation/coil embolisation 2)Surgical unifocalisation : if it is the sole supply to many segments …..connecting all MAPCAs, as well as the small native pulmonary arteries, to one source of blood flow from the RV [ie to a central PA confluence or prosthetic PA confluence]….. If performed early,it avoids the dvpt of PVOD changes, as well as stenoses in the MAPCAs. If the pulmonary vascular morphology and resistance are such that adequate PBF can be accommodated…then RVP after correction will be low enough to close the VSD.
  42. 42.  To assess whether this is likely, surgeons at the University of California in San Francisco perfuse blood from the perfusion system through the pulmonary artery at a rate of 2.5 L/min per m. If PA mean pressure is <25 mmHg, it is considered safe to close the VSD. If it is not closed, the patient is followed; the PVR may decrease over time, allowing later closure. Also, if stenoses in pulmonary vessels are noted, relief by balloon angioplasty, with stenting if necessary, may permit RVP to fall and permit closure of the defect.  Post surgery if the RVP/LVP is >0.75….RVF may result…avoid closure VSD / fenestrated patch closure
  43. 43. Confluent PA with favourable PA anatomy Hypoplastic PAs central Shunt RV – PA connection + unifocalization + VSD closure Single stage repair VSD closure + RV to unifocalized PA connection RV- PA connection unifocalization VSD closure VSD closureunifocalizationRV- PA conduit Non confluent Pas + MAPCAs TETRALOGY OF FALLOT WITH PULMONARYATRESIA
  44. 44. Post Repair for TOF in general  Current surgical survival, even for symptomatic infants <3 months of age, is excellent.  Hospital and 1-month survival rates of 100% have been reported.  Earlier age at repair (<1 year of age) did not adversely affect the rate of reintervention; so primary repair should be regarded as the preferred management strategy.  Twenty-year survival for hospital survivors, irrespective of management strategy, was 98% for patients who have TOF with PS and slightly lower for patients with PA, reflecting the overall excellent long- term survival of these patients.
  45. 45. Late complications • Repair – Pulmonary regurgitation:  d/t transannular patch repair technique  well tolerated if mild to moderate.  Severe chronic pulmonary regurgitation, however, may lead to symptomatic RV dilatation and dysfunction.  The severity of pulmonary regurgitation, and its deleterious long- term effects, are augmented by coexisting proximal or distal pulmonary artery stenosis, or pulmonary artery hypertension (uncommon). – Right ventricular (RV) dilatation:  residual longstanding free PR +/– RVOT obstruction or  as a consequence of RV surgical scar (transventricular approach for repair, now abandoned).  Significant tricuspid regurgitation (TR) may occur as a consequence of RV dilatation, which begets more RV dilatation.
  46. 46.  – Residual right ventricular outflow tract (RVOT) obstruction:  occur at the infundibulum,  at the level of the pulmonary valve and main pulmonary trunk,  distally, beyond the bifurcation and occasionally into the branches of the left and right pulmonary arteries.  – Aneurysmal dilatation of the RVOT:  relatively common in patients with previous transannular patch repair and significant pulmonary regurgitation.  Aneurysmal dilatation of the RVOT can be associated with regional RV hypokinesis.  Swirling of blood can be inferred from color flow Doppler signals in the aneurysmal right ventricular outflow tract regions. To date, no episodes ofsudden rupture of these regions have been reported. Furthermore, this area can be the focus of sustained ventricular tachycardia.
  47. 47. – Residual ventricular septal defect (VSD): residual VSDs can be encountered from either partial patch dehiscence or failure of complete closure at the time of surgery. – Aortic regurgitation (AR) with or without aortic root dilatation: AR can be due to damage to the aortic valve during VSD closure or secondary to an intrinsic aortic root abnormality (more common in patients with pulmonary atresia and systemic to pulmonary artery collaterals). The pathologic substrate for aortic root dilatation seems to be „cystic medial necrosis‟. – Left ventricular dysfunction: occasionally, left ventricular dysfunction can be seen from a variety of factors, including inadequate myocardial protection during previous repair(s), chronic LV volume overload due to longstanding palliative arterial shunts and/or residual VSD, injury to anomalous coronary artery (uncommon) or longstanding cyanosis before repair. – Endocarditis: residual lesions leading to turbulent fl ow (residual VSD patch leak, RVOT obstruction, PR, TR) encountered in most patients after initial repair can serve as substrate for endocarditis. –
  48. 48. Supraventricular arrhythmia: atrial fl utter and atrial fibrillation are relatively common in the current cohort of adults with previous tetralogy repair. Atrial tachyarrhythmia occurs in about one-third of adult patients and contributes to late morbidity and even mortality. – Ventricular tachycardia (VT): sustained monomorphic ventricular tachycardia is relatively uncommon. The usual arrhythmia focus is in the RVOT in the area of the previous infundibulectomy or VSD closure during tetralogy repair. Right ventricular dilatation from impaired hemodynamics is also contributory to the creation of re-entry circuits within the RV. The QRS duration from the standard surface EKG has been shown to correlate well with RV size in these patients. A maximum QRS duration of 180 ms or more is a highly sensitive marker for sustained VT and sudden cardiac death in adult patients with previous repair of tetralogy. – Sudden cardiac death (SCD): the reported incidence, presumably arrhythmic, in late follow-up series varies between 0.5–6% over 30 years, accounting for approximately one-third to one-half of late deaths.
  49. 49. Problems that may develop include a)Residual VSDs  Persistent defects at the patch margin or  Previously unrecognized or underestimated additional defects in the muscular septum.  Partial patch dehiscence b)Recurrent RVOTO :  Muscle bundles obstruct the os infundibulum in 3%  Nontransannular patches…annulus becomes restrictive as child grows  Transannular patch…restriction at the distal insertion of the patch into the branch pulmonary arteries.  Stenosis owing to compression from an aneurysmal RVOT patch …. should be approached surgically with revision of the patch and repair of the proximal obstruction.  may develop additional sites of peripheral PS over time.
  50. 50. c)Progressive aortic root dilation &AR :  Intrinsic dvptal abnormalities of aortic valve/root  Palliative shunts or significant APCs left-to-right shunt volume contribute to aortic dilation. Progressive dilation of aorta correlate with longer time between palliation & repair ; Pulmonary atresia ; right AA & male gender. d) RV dilation;syst/diast dysfunction later RHF  excess volume load from pulmonary regurgitation  pressure load from outflow obstruction,  Hypoxemic coronary perfusion,  surgical incision, patch and scarring, and post– cardiopulmonary bypass ischemia reperfusion injury— possibly superimposed on tetralogy associated congenital abnormalities of the myocardium.
  51. 51. e) LV dysfunction … consequence of adverse ventricular interaction. f) Rhythm disturb./SCD : Long-term mortality …. 3% to 6%. A review of 125 adult patients with TOF suggested that a greater degree of PR, a history of sustained VT, QRS duration >180 ms, or LV dysfunction was a predictor of sudden death g) Lower IQ h) Life time increased risk for IE i)Membranous subaortic stenosis …..may be seen years after the initial repair in a few patients…may require surgical excision. j)Small coronary-to-RV fistulas, thought to relate to the excision of muscle in the right ventricular outflow tract.
  52. 52. Post repair TOF + PA :  Need for reoperation/ transcatheter balloon dilation and stenting of an obstructed conduit in RV to PA conduit surgery patients.  Ongoing risk for recurrent peripheral PS…serial catheterizations are indicated for ongoing pulmonary artery rehabilitation  higher risk for progressive aortic root dilation and AR
  53. 53. TOF + AVCD :  risk for atrioventricular valve incompetence …. TR right ventricular dilation, and dysfunction.
  54. 54. Congenital Absence of Pulmonary Valve Syndrome  Chever in 1847  incompletely formed, rudimentary pulmonary valve that typically is both stenotic and regurgitant ; aneurysmally dilated PA, and a large malaligned outlet VSD.  PDA is always absent….has been postulated as being responsible for its pathogenesis and PA dysplasia.  Other clinical features fairly typical in this disorder include the common association of airway abnormalities that may lead to severe respiratory failure .  The conal septal abnormalities and infundibular obstruction, however, in distinction to typical tetralogy, are less severe or absent, and much of the PS results from annular hypoplasia.
  55. 55.  Typically present in the neonatal period, and are diagnosed based on the presence of their characteristic murmur and the presence of cyanosis.  A significant proportion of patients will present with associated respiratory distress or frank respiratory failure, often requiring mechanical ventilation.  harsh to-and-fro murmur of PS followed by the diastolic murmur of PR….sound of ’sawing wood’  CXR…Massively dilated Pas ; Infundibular dilation project leftward as a hump shaped shadow ; Pulmonary vascularity is normal
  56. 56.  Some neonates present with severe bronchial obstruction and require immediate tracheal intubation and mechanical ventilation, followed by early surgical repair.  Moderate resp obstruction … lying prone may help … by relieving the anterior vascular compression of bronchi  Mild or no airway obstruction may require no additional support in the neonatal period and go on to elective repair later in infancy.  The surgical repair….. in addition to ventricular septal defect closure and right ventricular outflow reconstruction, involves reduction of the aneurysmal mediastinal pulmonary arteries to relieve bronchial compression.
  57. 57. Outcome of TOF + APV depends largely on the severity of airway disease. Even after Sx,some pts continue to have bronchial obstruction  Residual airway hypoplasia and deformity  Abnormally branching segmental pulmonary arteries compressing the intraparenchymal bronchi As these patients grow, however, pulmonary function tends generally to improve as PAP fall and the maturing tracheobronchial tree develops less compressible walls and larger caliber.
  58. 58.  Surgical repair is only definitive treatment.  Palliative surgery is supportive.  Definitive repair to be done as early as possible .  TOF+PA requires indivualization of treatment dependiing on pulmonary anatomy.
  59. 59. THANK YOU
  60. 60. PVR : Early PVR in selected patients results in beneficial remodelling of the right ventricle Optimal timing is critical for preserving RV function (not too late) and avoiding the need for early re-operation (not too early). Amelioration of RV function following PVR has to be weighed against the risk of subsequent re- operation for homograft failure. Studies…RV end-diastolic volume may become a helpful indicator for defining both a lower limit (150 mL/m2) and an upper limit for re-intervention (200 mL/m2).[normal 60-100] CMR is the gold standard for evaluation of RV volumes and quantification of the degree of PR & TR.
  61. 61. Hypoplastic PAs … intervene early …. Encourage them to grow  Reconstruction of RVOT with a patch or valveless conduit  Placement of a central AP shunt  If MPA,RPA & LPA are present, even though very small (diameter 3 mm), they are capable of considerable enlargement if blood flow through them is increased. Creating an AP window early in infancy sufficient enlargement of the pulmonary arterial tree to later perform successful repair using the normal pulmonary arteries and a unifocalization procedure can be avoided.

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