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Cath data
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OXYGEN SATURATION (%)
% of oxygenated Hb.
Pulm. Ven. Sat. = 98% (100%).Arterial O2 Sat. = PV sat = 98-100%.
Mixed ven. O2 Sat = 75-80% (PA).
A drop of 2% in arterial O2 sat. compared to PV Sat.
= RL shunt
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PRESSURES
RA : a = 2-6; V = 2-4 m = 3 (0-6)
RV : 15-25 edp 0-5.
PA : 15-25; diast. 6-10, m = 10-15.
PAW : a = 6-12, V = 8-14, m 6-10 (12)
LA (PV) : a=6-12, V=8-14, m = 6-10 (12)
LV : 90-120 / 0-10 (12)
SA : 90-120 / 60-75 (70-85).
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8 months old infant,O/E CHF, Cyanosis
Site Pressure(mmHg) Saturation(%)
SVC -- 84
RA a12v7 mean 9 78
RV 74/8 80PA 68/34, mean48 79
LA a4v6 mean 4 76
LV 94/4-6 75
Aorta 94/60 mean 74 76PV mean 8 98
What is the diagnosis?
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Diagnosis
Supracardiac Total anomalous pulmonary venous
connection
Non -Restrictive interatrial communication
Pulmonary arterial hypertension
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TAPVC
Complete Mixing of SV return and PV return atatria level
Classical Supracardiac
Equal Saturation in all cardiac chambers
Infracardiac
Ao Saturation > PA Saturation
Supracardiac PA Saturation > Ao Saturation
Large ASD & VSD No sign. Difference
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Total Anomalous Pulmonary Venous
Connection
DefinitionCardiac malformation in which there is no direct
connection between any pulmonary vein & left atrium,but all the pulmonary veins connect to right atrium
or one of its tributaries. A PFO or an ASD is present
essentially all persons who survive after birth.
HistoryWilson : 1st description in 1798Muller : 1st closed partial approach in 1951
Lewis & Varco : Successful open repair in 1956
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Total Anomalous Pulmonary Venous
Connection
Origin of anomalous connection
1. Drainage to right atrium
2. Drainage to right common cardinal system
(SVC or azygous vein)
3. Drainage to left common cardinal system
(Left innominate vein or coronary sinus)
4. Drainage to umbilical-vitelline system
(Portal vein, ductus venosus, hepatic vein)
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TAPVC
Pathophysiology Entire pulmonary venous return drains into the
right atrium, usually via a common pulmonary vein
confluence, resulting in complete pulmonary andsystemic venous mixing.
Oxygenated blood reaches the left heart via aninter-atrial connection (i.e.,ASD, PFO).
Mechanical or functional obstruction of thepulmonary venous return leads to cyanosis, acidosis,pulmonary hypertension, & congestion.
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TAPVC
1. Pulmonary venous anatomy
1) Type : Supracardiac 45%
Cardiac 25%
Infracardiac 25%
Mixed 5%
2) Pulmonary venous obstruction
. Junction of connecting vein
or compression of long
narrow connect vein
. Functional obstruction
(restrictive PFO)
2. Chamber & septal anatomy
. LA & LV : small
. ASD or PFO : small in 1/2,
rarely no ASD or PFO
3. Pulmonary vasculature
. Increased arterial muscularity
. Structural change
4. Associated condition. PDA : 15%
. VSD : occasionally
. TOF, DORV, IAA : rarely
Morphology
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RV & PA pressure Pulmonary venous obstruction PVR
With obstructed TAPVC PA pressure frequently exceeds Systemic pressure
With no or mild obstruction Mildly to moderately elevated
RVEDP Elevated more with
Pulmonary venous obst
Restrictive PFO LV & SA pressure
Usually normal Decreased with
Restrictive PFO Obstructed TAPVC with R-L duct (very high PAP)
TAPVC
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TAPVC
Types
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TAPVC
Clinical features & diagnosis1. Presentation
. Critically ill infants during 1st few week of life
. Unexplained tachypnea & unimpressive cyanosis
. Metabolic acidosis : pulmonary venous obstruction2. Examination
. Not particularly overactive heart & unimpressive heart sound
3. Chest radiography
. Normal heart size with diffuse haziness or ground glass
if pulmonary venous obstruction. Large heart size with high pulmonary blood flow
. Figure of 8, snowman configuration
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TAPVC: Hemodyanamic Issues
Obstructed
Infradiaphragmatic,
Very early presentation
More cyanosis
Pul edema
Low PV sat
More CHF
PDA: Large
R-L flow
PA decompresses
Less Pul Edema
PDA small:
Pul edema,RV failure
Mild or no obstruction
Good SpO2
High Qp/Qs
Presentation
Infancy
PFO Restriction
Restrict SBF
High PBF
High Qp/Ps
Good SpO2
CHF earlyCongestion of both
syst & pulm venous circ if
Resistance to PBF is high
PAH
Surgical emergency
Surgery at presentation
BAS followed by Surgery
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TAPVC
Natural history1. Incidence
. Relatively uncommon anomaly, 1.5~3% of CHD
2. Survival
. Unfavorable prognosis
50% survival in 3months
20% survival in one year
. Usually have pulmonary venous obstruction due to
long pulmonary venous pathway & a small PFO
. Those who survive the first year of life usually have
large ASD, no pulmonary venous obstruction
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TAPVC
Indications for operation Immediate operation in any neonate or infant
whom are importantly ill with TAPVC Prompt operation in any 6-12 months old infant
Advisable if severe pulmonary vascular disease
has not developed in old patients (under 8 units)
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Surgery:corrective
Supracardiac type:
Side to side anastomosis between commonpulmonary venous sinus & LA.Vertical vein
ligated.ASD closed with cloth patch. To RA: atrial septum excised,patch is sewn in
such a way that the pulmonry venous return is
diverted to LA. To coronary sinus:unroofing of CS,patch
closure of ASD
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Infracardiac:
Vertical anastomosis made between common
pulmonary venous sinus and LA.The common
PV which descends vertically is ligated above
diaphragm
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Sutureless technique is for the relief
of PV stenosis. A, Theincision is made
into the left atrium and extended into
both upper and lower PVostia
separately. B, Suturing is begun in
thepericardium just above the junction
of the superior PV with the left atrium.
C, A second inferior suture is started
below theinferior PV and continued inthe same manner to the left atrial
incision to jointhe superior suture line.
Sutureless technique
TAPVC
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Primary Sutureless Repair
Rationale Small size of the pulmonary vein is a major risk factor for
later development of PVS after conventional TAPVDrepair
The acute anatomic benefit for the suturelessrepair isthat each vein is its own native size, without anysuturematerial to cause an excessive inflammatory reactionorluminal compromise
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TAPVC
1. Operative mortality:
5-10% in non-obstructive type
20% in obstructive type
2. Modes of death
. Hypertensive crisis
. Pulmonary venous stenosis
3. Incremental risk factors
for death
. Infracardiac drainage
. Pulmonary venous obstruction
. Poor preoperative state
. Small size of pulmonary vein
. Increased PVR
. Small left ventricle
4. Cardiac rhythm:atrial
arrhythmias
5. Reoperation
. Anastomotic stricture
(5~10%)
. Pulmonary vein stenosis
Surgical results
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1 day old ,TAPVC to Hepatic vein
SVC 32
IVC 38
HV 65 RA 44 m12
RV 42 110/16
PA 42 115/80
DAo 46 80/55
LA 48 m8
LV 48 82/10
O2 sat Pressure mmHgSite
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32 yrs old /F presented with recent onset of
palpitations
Site Pressure(mmHg) Saturation(%)
SVC -- 64
RA a10v7 mean 6 84
RV 34/8 80
PA 32/18, mean 24 79
LA a11v8 mean 10 96
LV 120/10 94
Aorta 126/70 mean 94 96
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There is significant step-up in Oxygen
saturation from SVC-RA
Mildly elevated PA pressure
Diagnosis could be
ASD
PAPVC to RA
Coronary AV fistula to RA
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8 yrs old child with mild cyanosis
Site Pressure(mmHg) Saturation(%)
SVC -- 60.7
RA a6v3 mean 4 64.6
RV 128/6 62.6
PA 16/8, mean 12 66
LA a5v8 mean 4 96
LV 124/8 86
Aorta 126/70 mean 94 87
Diagnosis?
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Data suggests
Nonrestrictive VSD
Severe PS
Tetralogy of Fallot
Cyanosis is due to rightleft ventricular
shunt
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TOF
Systolic pr in RV=LV=Ao
Step down at LA-LV level
Acyanotic /pink TOF-inc PA pr
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15 yrs old child with H/O exertional dyspnea
Site Pressure(mmHg) Saturation(%)
SVC -- 66
RA a12v7 mean 10 67
RV 124/10 65
PA 120/70, mean 88 72
LA a11v8 mean 10 96
LV 120/10 94
Desc Ao 126/74 mean 92 86
Diagnosis?
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Data shows:
Systemic RV and PA pressure
Presence of desaturation in aorta
Differential Diagnosis Large ductus arteriosus
Large aorto-pulmonary window
With severe PAH
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5 yrs old child referred for bradycardia
Site Pressure(mmHg) Saturation(%)
SVC -- 66
RA a10v6 mean 6 67
RV 124/8 96
PA 20/10, mean 14 72
LA a6v10 mean 8 96
LV 24/8 70Ao 126/74 mean 92 96
Diagnosis?
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This hemodyanamic data shows
AV & VA discordance
Congenitally corrected transposition of greatvessels
Complete Heart block is common (2% per
year) in patients with corrected TGA Cause of bradycardia