3. Total Anomalous Pulmonary
Venous Connection
Definition
Cardiac 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 it’s tributaries. A PFO or an ASD is present
essentially all persons who survive after birth.
History
Wilson : 1st description in 1798
Muller : 1st closed partial approach in 1951
Lewis & Varco : Successful open repair in 1956
4. 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, and so on)
5. Pulmonary Vein
Splanchnic plexus provides drainage of the lung buds into cardinal &
umbilicovitelline venous system. Common pulmonary vein evaginates
from the left atrium and merges with the splanchnic plexus.
Connections of pulmonary drainage to systemic venous system regress.
Development
6. TAPVC
Pathophysiology
• Entire pulmonary venous return drains into the
right atrium, usually via a common pulmonary
vein confluence, resulting in complete pulmonary
and systemic venous mixing.
• Oxygenated blood reaches the left heart via an
inter-atrial connection (i.e.,ASD, PFO).
• Mechanical or functional obstruction of the
pulmonary venous return leads to cyanosis,
acidosis, pulmonary hypertension, & congestion.
7. 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, or 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
12. TAPVC
Clinical features & diagnosis
1. Presentation
. Critically ill infants during 1st few week of life
. Unexplained tachypnea & unimpressive cyanosis
. Metabolic acidosis : pulmonary venous obstruction
2. Examination
. No 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
4. Echocardiography
5. Cardiac catheterization & cineangiography
13. TAPVC
Natural history
1. 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
14. TAPVC
Indications for operation
• Investigation must be undertaken promptly in any
neonate or infant, no matter how young, who develops
signs or symptoms suggestive of TAPVC
• 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)
15. TAPVC
Operative techniques
• Operation should be undertaken as an emergency after
diagnosis by echocardiography who enter the hospital
critically ill. Preoperative preparation & stabilization
is contraindicated.
1. TAPVR to Lt. innominate vein
2. TAPVR to SVC
3. TAPVR to coronary sinus
4. TAPVR to right atrium
5. TAPVR to infradiaphragmatic vein
19. • Suturelesstechnique 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 in
the same manner to the left atrial
incision to jointhe superior suture line.
Sutureless technique
TAPVC
20. Primary Sutureless Repair
Rationale
• Small size of the pulmonary vein is a major risk factor
forlater development of PVS after conventional
TAPVD repair and that high mortality of right atrial
isomerism is related, at least in part, to intrinsically
small pulmonary veins.
• Furthermore, most of the patients with RAI are not
anatomic candidates for biventricular repair. PVS is a
risk factor for poor Fontan operation outcome
• The acute anatomic benefit for the sutureless repair is
that each vein is its own native size, without any suture
material to cause an excessive inflammatory reaction or
luminal compromise
21. TAPVC
1. Survival
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. Functional status
5. Hemodynamic result
6. Cardiac rhythm
7. Reoperation
. Anastomotic stricture
(5~10%)
. Pulmonary vein stenosis
Surgical results
22. TAPVC
Special situation & controversies
1. Delayed operation
In critical patients with obstruction at atrial level,
balloon dilation and 1-2 days later operation
2. Mixed total anomalous venous connection
3. Operative exposure
4. Surgical enlargement of left atrium
Decrease in atrial volume of more than 50%
result in reduction in cardiac output ?
5. Pulmonary vein stenosis
23. Residual TAPVC
PVD in remained anomalous veins
• Possible pressure-sensitive receptors at
the anomalous vein-vena cava junction
• Axon reflex triggered by right atrial
distention
• Results of the increased blood flow
24. Pulmonary Vein Stenosis
Etiology
1. Low grade venous obstruction presents at the
end of procedure results in reactive fibrosis
( diffuse fibrosis & thickening of vein )
2. Self perpetuating stenosis
3. Intraatrial thickening
4. Diffuse pulmonary vein stenosis
5. Congenital nature ( hypoplasia, focal stenosis,
discrete ostial stenosis)
25. Pulmonary Vein Stenosis
Factors of development
1. Small confluent pulmonary vein
2. Suture material
3. TAPVC type?
4. Undue trauma toward pulmonary
vein ostium and tension
5. Steroid therapy
26. Congenital PV Stenosis
Clinical features
• Occur in about 0.4% of congenital heart defects and
one or multiple veins may be affected.
• Histologically, the lesion is characterized by fibrous
intimal thickening in most cases and medial
hypertrophy in many
• The first surgical repairof congenital PV stenosis was
reported by Kawashima and colleagues in 1971 and
surgical approaches have evolved over the years, but
results have been generally disappointing.
• Diffuse restenosis has been documented as a significant
cause of late mortality after repair
27. Acquired PV Stenosis
Characteristics
• Anatomically localized to the anastomosis, and the
natural history is more favorable, or the stenosis may
extend diffusely into the branch pulmonary veins.
• It can sometimes be difficult to distinguish these forms of
acquired PV stenosis at the time of presentation.
• Acquired PV stenosis occurs in approximately 7% to
11% of early survivors after total anomalous pulmonary
venous connection repair
• Results of repairof acquired PV stenosis have also been
less than optimal due to the problem of restenosis
28. Acquired PV Stenosis
Anatomic features
• Post-repair pulmonary vein stenosis appears to have
three basic subtypes.
• The most minimal form of the disease is limited to the
anastomotic area with sparing of the pulmonary veins
and confluence, suggesting a technical error or
imperfection at the time of initial repair.
• The intermediate form is limited to the pulmonary
venous confluence in addition to the anastomotic area.
• The most extensive form of the disease includes a
fibrous reaction extending retrograde deep into the
lung parenchyma.
29. Pulmonary Vein Stenosis
Strategy for treatment
1. Minimize trauma at suture line
Suture line (tension or inflexibility, deformation)
Suture material
Handling the vein tissue
2. Avoid postoperative turbulence
Constraints imposed by restrictive characteristics
3. Surgical methods
Operative patch venoplasty
Sutureless pericardial marsupialization
Catheter dilation
Stent placement and combination
30. Complex TAPVC
Etiology of high mortality
1. The interplay of systemic shunt with abnormal
pulmonary vasculature contributes to difficulty in
maintaining postoperative pulmonary to systemic
flow ratio.
2. To limit excessive pulmonary blood flow with banding,
or augmentation with shunt, the end result is similar.
3. The static matching of this resistance to the cardiac
output in face of abnormal pulmonary vasculature may
not allow appropriate regulation of pulmonary blood
flow during dynamic changes.
