Download - D-TGA
![Page 1: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/1.jpg)
D-TGA
Dr. Tahsin.N
![Page 2: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/2.jpg)
TRANSPOSITION
• Abnormal origin of the Aorta and Pulmonary Artery from the
ventricular complex
• Atrioventricular concordance with ventriculo-arterial
discordance
• Abnormal spatial relationship of the great arteries
• Results in two circulations in parallel
![Page 3: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/3.jpg)
Incidence & Prevalence
• 5% to 7% of all congenital cardiac malformations
• The incidence is reported to range from 20.1 to 30.5/100,000
live births
• strong (60%–70%) male preponderance
![Page 4: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/4.jpg)
Embryology
![Page 5: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/5.jpg)
Embryology
1. Spiral aortico-pulmonary septum forms but does not spiral or
twist during its partitioning of the truncus arteriosus
a. Aorta arises from right ventricle
b. Pulmonary trunk arises from the left ventricle
2. Result is two closed circuits
a. Systemic – unoxygenated – repeatedly re-circulated
b. Pulmonary - oxygenated - repeatedly re-circulated
![Page 6: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/6.jpg)
Embryology
• The normal conus is subpulmonary, left-sided and anterior ; it
prevents fibrous continuity between the pulmonary and
tricuspid valve rings.
• In TGA, the infundibulum is usually subaortic, right-sided and
anterior; it prevents fibrous continuity between the aortic and
tricuspid valve rings and further results in abnormal
pulmonary to mitral valve ring fibrous continuity.
![Page 7: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/7.jpg)
Anatomy
• The common clinical type - situs solitus of the atria,
concordant AV and discordant ventriculoarterial alignments -
complete TGA.
• TGA {S,D,D} - TGA with situs solitus (S) of the atria and viscera,
usual (D) looping of the ventricles and an anterior and
rightward (D) aorta.
![Page 8: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/8.jpg)
Anatomy- Great artery relationship
• Situs solitus and intact ventricular septum - the aortic root is
directly anterior or anterior and to the right of the pulmonary
trunk in a slightly oblique relationship
• Less commonly, the aorta may be positioned anterior and to
the left or, rarely, posterior and to the right of the pulmonary
trunk.
![Page 9: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/9.jpg)
Coronary Anatomy
• The two aortic sinuses of Valsalva adjacent to the
aorticopulmonary septum that “face” the pulmonary artery
contain the ostia of the coronary arteries in more than 99% of
cases
![Page 10: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/10.jpg)
Coronary anatomy
• Usual-66.9
• CX from RCA-16.1
• Single RCA-3.9
• Single LCA-1.7
• Inverted-2.4
• Intramural LCA-2.1
• Other-1.6
![Page 11: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/11.jpg)
SA node artery
• Origin and proximal course of artery may be variable; reaches
the sinus node by the interatrial groove on the anterior
surface of the heart, occasionally with an intramyocardial
course in the anterosuperior rim of the fossa ovalis.
• It can be damaged easily during balloon atrial septostomy,
during surgical septectomy or when this portion of the
septum is widely excised as in the Mustard or Senning atrial
switch operation.
![Page 12: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/12.jpg)
Anatomy - Coexisting Anomalies
• Nearly half of the hearts have no other anomaly except a PFO
or a PDA.
• The VSD is the most frequent coexisting anomaly-40% to 45%.
- perimembranous (conoventricular 33%)
- AV canal (inlet septum 5%)
- muscular (27%)
- malalignment (30%)
- conal septal hypoplasia type (5%)
![Page 13: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/13.jpg)
VSD
• The subaortic stenosis caused by the anterior malalignment of
the infundibular septum is frequently associated with aortic
arch hypoplasia, coarctation or even complete interruption of
the aortic arch
• Posterior (leftward) malalignment is associated with varying
degrees of LVOTO–subpulmonary stenosis, annular hypoplasia
or even pulmonary valvar atresia
![Page 14: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/14.jpg)
Subpulmonary Stenosis 25% [5%]
• Fixed
-Circumferrential fibrous membrane /diaphragm
- Fibromuscular ridge
- Herniating tricuspid leaflet tissue
- Anomalous MV septal attachments
- Tissue tags from membranous septum
• Dynamic-associated with SAM
![Page 15: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/15.jpg)
Subaortic Obstruction
• Rightward and anterior displacement of the infundibular
septum
• Associated aortic arch anomalies
- hypoplasia
- coarctation
- interruption
Asso. RV hypoplasia & tricuspid valve anomalies
![Page 16: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/16.jpg)
TV anomalies
Nearly 31%
Functionally imp 4%
Ratio of tricuspid to mitral anulus circumference is less
than 1 in almost 50% of cases, whereas in normal hearts this
ratio is always greater than 1
![Page 17: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/17.jpg)
TV anomalies
• Straddling/overriding of chordae
• Overriding of the tricuspid annulus
• Abnormal chordal atatchments
• Dysplasia
• Accessory tissue
• Double orifice
![Page 18: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/18.jpg)
MV anomalies
Nearly 20%
Functionally imp 4%
– Cleft anterior mitral valve leaflet
– anomalous papillary muscles and chordae
– Straddling
– redundant tissue tags
![Page 19: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/19.jpg)
Juxtaposition of atrial appendages
• Both appendages or left + part of right are adjacent
• 2-6%
• Left > right -6x
• Female preponderance
• often additionally associated with major cardiac pathology,
including dextrocardia, VSD, bilateral infundibulum, right
ventricular hypoplasia and tricuspid stenosis or atresia.
• Imp in BAS
![Page 20: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/20.jpg)
Bronchopulmonary Collateral Circulation
• Bronchopulmonary anastomotic channels > 30% of infants
with TGA under 2 years of age
• Persistence of a significant bronchopulmonary collateral
circulation after surgical repair - large enough left-to-right
shunt – CCF - warrant catheter embolization
![Page 21: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/21.jpg)
PBF• 50% of the patients - greater proportion of blood flow to the
right lung than normal due to rightward alignment of MPA
• associated with some degree of hypoplasia of the left
pulmonary arterial vessels and is further manifested in the
occasional reports of unilateral, always left-sided, pulmonary
vein stenosis or hypoplasia.
![Page 22: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/22.jpg)
PBF
![Page 23: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/23.jpg)
Postnatal Physiology of TGA
![Page 24: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/24.jpg)
Determinants of effective gas exchange
• Effective ventilation
• Effective Pulmonary circulation
– Pulmonary blood flow
– Pulmonary vascular resistance
• Existence of a communication between pulmonary and systemic circuits
– Persistent fetal channel – PFO or DA
– Abnormal channels – ASD, VSD
• Effective delivery of oxygenated blood to the tissues
![Page 25: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/25.jpg)
Definition of shunts
• Anatomical shunts
– Left to Right: Blood flowing from left sided chambers to
the right sided chambers
– Right to Left: Blood flowing from right sided chambers to
the left sided chambers
![Page 26: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/26.jpg)
Definition of shunts
• Physiological shunts
– Left to right: The volume of oxygenated pulmonary venous
return recirculated to pulmonary circulation (Qp – Qep)
– Right to left shunt: The volume of systemic venous return
that contributes to cardiac output (reentering the systemic
circulation) without having passed through the pulmonary
circulation (Qs – Qep)
![Page 27: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/27.jpg)
Definition of shunts
• Effective pulmonary blood flow (Qep):
– The volume of systemic venous return that is effectively
oxygenated in the lungs
• Effective systemic blood flow (Qes):
– The volume of oxygenated pulmonary venous return that
enters the systemic circulation and perfuses the systemic
capillary bed
![Page 28: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/28.jpg)
= Effective Systemic
Blood Flow
TGA: Atrial and Ventricular level shunts
• From LA to RA / LV to RV
– Anatomically left to
right
– Physiologically, this
volume of oxygenated
blood enters systemic
circulation. Hence,
they contribute to Qes
![Page 29: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/29.jpg)
TGA: Atrial and Ventricular level shunts
• From RA to LA/ RV to LV– Anatomically, right
to left shunt– Physiologically, this
volume of systemic venous blood enters pulmonary circulation. Hence they contribute to Qep
= Effective PBF
![Page 30: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/30.jpg)
Recirculating Oxy
Blood
Recirculating systemic blood
![Page 31: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/31.jpg)
TGA: Shunt at PDA level• Aorta to PA flow:
– Anatomically it is left to right– Here the deoxygenated systemic venous blood enters
pulmonary circulation. Hence, this volume contributes to Qep
• PA to Aorta flow:– Anatomically it is right to left– Here the oxygenated blood enters systemic circulation. Hence,
this volume contributes to Qes
• Thus, the flow across the ductus is functionally opposite to that of flow across ASD or VSD in TGA
![Page 32: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/32.jpg)
Systemic venous
return
Pulmonary venous return
Anat R-L
Anat L-R
Physio R-L
Physio L-R
BODY LUNGS
RIGHT
HEART
LEFT
HEART
![Page 33: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/33.jpg)
Unique feature
• Net inter-circulatory mixing volume is constant: net R-L, L-R, Qep and Qes are equal to each other
• Any major difference in the volumes would result in depletion of blood volume of one circulation at the expense of overloading the other circulation
![Page 34: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/34.jpg)
Precise factors controlling intercirculatory exchange
SPECULATIVE, MULTIPLE
– LOCAL PRESSURE GRADIENTS
• Compliance of the cardiac chambers• Phase of respiratory cycle• Vascular resistances• Heart rate• Volume of blood flow
![Page 35: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/35.jpg)
Flow across the communications“Rules of the Heart”
• With only ASD, the flow has to be bidirectional
• If the flow is only or predominantly left to right across the
ASD, it suggests presence of additional shunt (VSD or PDA)
• Unrestrictive VSD - flow is bidirectional
• Except in the initial few days, PDA flow is always left to right
(Ao to PA).
• Presence of right to left flow across ductus may suggest the
presence of coarctation of aorta
![Page 36: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/36.jpg)
Right to Left Shunt
Systole
Left to Right Shunt
Diastole
![Page 37: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/37.jpg)
• Initially, bidirectional flow across the ductus• Later, once the PVR falls, the flow essentially becomes aorta
to PA• The pulmonary circulation becomes overloaded fast,
especially if the PFO is restrictive
![Page 38: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/38.jpg)
Factors influencing systemic saturation
• Extent of inter-circulatory mixing and Total pulmonary blood
flow
• High PBF results in increased oxygenated blood available in
the left sided chambers for mixing: higher systemic SO2 if
there is good mixing
• Reduced PBF will result in low systemic SO2 in spite of
adequate anatomic shunts
![Page 39: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/39.jpg)
Factors influencing systemic saturation
• If there is delay in the fall of PVR (PPHN), hypoxemia will
persist despite adequate ASD
• Need ECMO or urgent ASO
• Hypoxemia provokes a fall in SVR and increase the
recirculating systemic volume
• Fall in SVR may deplete the pulmonary circulation further
![Page 40: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/40.jpg)
Role of bronchopulmonary collaterals
• Systemic arterial hypoxemia may stimulate development of
bronchpulmonary collaterals
• Usually in TGA with solely a restrictive inter-atrial
communication
• Prolonged survival of such infants may be due to this extra-
cardiac site of shunting/mixing
![Page 41: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/41.jpg)
History
• M:F – 4:1;unless juxtaposition of atrial appendages
• Usually in multigravida-2X increase in > 3 pregnancies
• Familial recurrence-monogenic inheritance
![Page 42: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/42.jpg)
Cyanosis
• As early as day 1 in pts with IVS(1st hr-56%;1st day-90%)
• More intense if associated PS/atresia
• Mild if associated non restrictive VSD
• PS often responsible for hypercyanotic spells-intense cyanosis,
tachypnea, extreme irritability and hypothermia
• Squatting is rare
• Reverse differrential cyanosis
![Page 43: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/43.jpg)
CHF
• In patients with a large PDA• Large VSD
![Page 44: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/44.jpg)
Mortality
• 1st week-30%
• 1st month-50%
• 1st year-90%
• Depends on the degree of shunting
• Moderate PS improves survival
• Predilection for brain abscess but rare < 2 years
![Page 45: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/45.jpg)
Appearance
• Birth weight greater than normal
• Reverse differential cyanosis
• Varicosities of scalp and arms
![Page 46: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/46.jpg)
Arterial Pulse
• Bounding pulse - due to large volume of highly unsaturated blood - Not due to PDA-since only systolic shunt from aorta to
PA
• Diminished femoral pulses - CoA - Subaortic stenosis-anterior and rightward
displacement of septum
![Page 47: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/47.jpg)
Palpation
• Nomal in neonates
• RV impulse in patients with CHF• LV impulse – non restrictive VSD with low PVR
• Palpable S2 A2
![Page 48: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/48.jpg)
Auscultation
• Loud A2
• LV S3-mildly cyanosed patients,increased PBF,LV failure
• RV S3-deeply cyanosed patients, increased systemic flow, RV
failure
![Page 49: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/49.jpg)
Auscultation
• Ejection click-pulmonary;does not decrease with inspiration
• Aortic-subaortic stenosisdilated aortic root
• MSM-aortic:hypervolemic and hyperkinetic circulation
• Pulmonary: valvular- after few weeks of birth, progressively
increases
• Subvalvar dynamic obstruction-3rd LICS and radiates to the
right
![Page 50: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/50.jpg)
Auscultation
• VSD: absentholosystolicshortensabolished
• PDA:
Systolic if large PDA since high PVR curtails diastolic flow
Continuous if restrictive PDA
• Continuous murmurs may arise in large systemic arterial
collaterals but rare
• MDM may be heard across AV valves
![Page 51: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/51.jpg)
ECG
• Normal in first few days of life
• RAE-increased pressure(CHF)/volume (hypervolemic systemic
circulation)
• LAE-large ASD,increased PBF
• RAD-occurs when LV volume overload is curtailed by
pulmonary vascular disease or PS
![Page 52: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/52.jpg)
ECG
• RVH - NR VSD +high PVR/PS
• BVH - NR VSD + low PVR
• Right precordial T waves not inverted but rather distinctly
taller than the left sided T waves
![Page 53: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/53.jpg)
![Page 54: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/54.jpg)
CXR
• Absent thymic shadow after 12 hours of life
• Narrow vascular pedicle bcoz - AP orientation of great vessels
• Right aoric arch -11-16%
• Egg on side appearance
• Juxtaposition-localised bulge along the mid left cardiac border
which represents contiguous mass of the 2 appendages together
• PBF & Heart size inversely proportional
![Page 55: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/55.jpg)
![Page 56: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/56.jpg)
ECHO
![Page 57: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/57.jpg)
• Diagnosis• Detection & quantitation of shunt• Detection of outfow obstructions• Asso anomalies• Coronary Anatomy• Post op Detection of Complications
![Page 58: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/58.jpg)
![Page 59: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/59.jpg)
Cardiac catheterization in TGA
![Page 60: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/60.jpg)
Fallacies in application of Fick’s Principle in calculating shunts and flows in TGA
• Oxygen consumption is not normal, so assumed values are
unreliable
• Arteriovenous oxygen differences may be very small, so
magnitudes of errors in calculated values would be very large.
• Effect / contribution of Bronchopulmonary collaterals to PBF –
can result in overestimation.
![Page 61: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/61.jpg)
TGA and PVOD
![Page 62: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/62.jpg)
Changes in Pulmonary Vascular Resistance
• Accelerated PVD is common
• With unrestrictive VSD, Grade 3 or 4 changes seen in 20%
before 2 months and in 80% by 1 year
• Without VSD or PDA, it is seen in 6%, progression is slower
than with VSD
• The number of intra acinar pulmonary arteries are also shown
to be decreased
![Page 63: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/63.jpg)
• In TGA/ASD, regression of PVR occurs as in simple ASD but
subsequently PVOD may develop rapidly.
• Reduced saturation Increased hematocrit Increased
shear stress PVOD
• Bronchial artery collaterals bring poorly saturated blood to
pulmonary vessels
• Short MPA
![Page 64: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/64.jpg)
Metabolism in TGA physiology
• Oxygen demands are high while delivery and uptake is poor –
at baseline
• Metabolic acidemia, lactic acidosis
• HYPOTHERMIA can KILL – EXAGGERATING TISSUE HYPOXIA
AND METABOLIC ACIDEMIA
• Hypoglycemia
• Hyperinsulinemia
![Page 65: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/65.jpg)
Management
![Page 66: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/66.jpg)
Definitive Repair
at three levels: • the atrial level : Senning or Mustard Sx• ventricular level : Rastelli operation• great artery level : arterial switch operation or Jatene
operation
• Damus-Kaye-Stansel operation in conjunction with the Rastelli operation can be used in patients with VSD and subaortic stenosis
• Lecompte Operation-VSD+subpulmonary stenosis
![Page 67: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/67.jpg)
Arterial switch operation (Jatene operation)
Advantages • physiologic correction• fewer long-term complications
– Arrhythmias
– RV dysfunction
– baffle stenosis
– tricuspid regurgitation (TR).
![Page 68: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/68.jpg)
Arterial switch operation (or Jatene operation)
![Page 69: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/69.jpg)
Pre requisite • An LV that can support the systemic circulation after surgery
• The LV pressure should be near systemic levels at the time of surgery, or the switch should be performed shortly after birth (i.e., before 2 weeks of age).
• In patients whose LV pressure is low, it can be raised by PA banding, either with or without a shunt, for 7 to 10 days (in cases of a rapid, two-stage switch operation) or for 5 to 9 months before undertaking the switch operation.
• LV pressure >85% and LV posterior wall thickness >4.5 mm appear to be satisfactory.
![Page 70: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/70.jpg)
Pre-op
• Coronary artery pattern amenable to transfer to the neoaorta without distortion or kinking.
• Risk is high when the left main or LAD coronary artery passes anteriorly between the aorta and the PA.
![Page 71: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/71.jpg)
Pre-op
• The left ventricular inflow and outflow tracts must be free of significant structural abnormality.
• The right ventricular outflow tract should be free of significant stenosis.
![Page 72: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/72.jpg)
Anatomic variants that may impact operative mortality include
– An intramural course of a coronary artery – A retropulmonary course of the left coronary artery– Multiple VSDs– Coexisting abnormalities of the aortic – Straddling AV valves – Longer duration of global myocardial ischemic (cross-clamp)– prolonged circulatory arrest times
![Page 73: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/73.jpg)
Complications
• PA stenosis at the site of reconstruction - 5% to 10% • complete heart block - 5% to 10%.
• Aortic regurgitation (AR)
– late complication > 20% of patients especially PA banding – An important cause of AR may be unequal size of the pulmonary cusps
that leads to eccentric coaptation
• Coronary artery obstruction– myocardial ischemia, infarction, and even death.
![Page 74: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/74.jpg)
Atrial level SurgeryMustard operation: This oldest surgical technique redirects the
pulmonary and systemic venous return at the atrial level by using either a pericardial or a prosthetic baffle.
![Page 75: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/75.jpg)
• Senning operation: This is a modification of the Mustard
operation. It uses the atrial septal flap and the RA free wall to
redirect the pulmonary and systemic venous return
![Page 76: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/76.jpg)
![Page 77: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/77.jpg)
Complications
a.Obstruction to the pulmonary venous return (<5% of all cases)
b.Obstruction to the systemic venous return (<5% of all cases)
c.Residual intra-atrial baffle shunt (=20% of all cases)
d.Tricuspid valve regurgitation (rare)
e.Absence of sinus rhythm (>50% of all cases) and frequent supraventricular arrhythmias
f.Depressed RV (i.e., systemic ventricular) function during exercise
g.Sudden death attributable to arrhythmias (3% of survivors)
h.Pulmonary vascular obstructive disease
![Page 78: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/78.jpg)
Rastelli operation
• In patients with VSD and severe PS
• The LV is directed to the aorta by creating an intraventricular tunnel between the VSD and the aortic valve.
• A conduit is placed between the RV and the PA
![Page 79: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/79.jpg)
Rastelli operation
![Page 80: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/80.jpg)
Complications
• conduit obstruction (especially in those containing porcine heterograft valves)
• complete heart block (rarely occurs).
• This conduit needs to be replaced as the child grows.
![Page 81: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/81.jpg)
Medical
• Prostaglandin E1 infusion should be started to improve arterial oxygen saturation by reopening the ductus. This should be continued throughout the cardiac catheterization and until the time of surgery.
• Oxygen should be administered for severe hypoxia. Oxygen may help lower pulmonary vascular resistance and increase PBF, resulting in increased systemic arterial oxygen saturation.
![Page 82: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/82.jpg)
Role of PGE1 in TGA
• Considerable benefit in first few days till PVR is elevated,
especially if PFO is small
• Enables bidirectional shunting, improves mixing
• If valve of FO is competent, it would result in increased LA
pressure and pulmonary edema
![Page 83: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/83.jpg)
Atrial Septostomy
• Before surgery, cardiac catheterization and a balloon atrial septostomy (i.e., the Rashkind procedure) are often carried out to have some flexibility in planning surgery.
• a balloon-tipped catheter is advanced into the left atrium (LA) through the PFO. The balloon is inflated with diluted radiopaque dye and abruptly with-drawn to the right atrium (RA) under fluoroscopic or echo monitoring.
![Page 84: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/84.jpg)
![Page 85: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/85.jpg)
Atrial Septostomy
• For older infants and those for whom the initial balloon atrial
septostomy was only temporarily successful, blade atrial
septostomy may be performed.
• Following this, the balloon procedure can be repeated for a
better result.
![Page 86: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/86.jpg)
Pulmonary Artery Banding
Transposition associated with large VSD without LVOTO
To prevent
Heart failure
Pulmonary vascular disease
Present Indications
Presence of complex/multiple VSDs
Coexisting medical conditions that cause a delay in surgery
To train LV before switch in TGA/IVS
![Page 87: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/87.jpg)
Systemic-Pulmonary Anastomosis
TGA/VSD and severe LVOTO
Operative mortality - as low as 5%.
Reassessment of pulmonary vascular resistance
![Page 88: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/88.jpg)
![Page 89: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/89.jpg)
• D-TGA (Complete Transposition of great arteries)
• Severe cyanosis in a large newborn
• Male preponderance (3:1)
• Single S2
• Signs of CHF (±)
• Usually no heart murmur
• “Egg-shaped” heart with narrow waist (on x-ray film)
• ECG: Normal or RVH
![Page 90: D-TGA](https://reader038.vdocument.in/reader038/viewer/2022110103/5681432c550346895daf9d01/html5/thumbnails/90.jpg)
THANK U