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Venous Connection
Seoul National University Hospital
Department of Thoracic & Cardiovascular Surgery
Total Anomalous Pulmonary
Venous Connection
Seoul National University Hospital
Department of Thoracic & Cardiovascular Surgery
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
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)
Pulmonary Vein
Development
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.
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.
TAPVC
Morphology
1. Pulmonary venous anatomy 2. Chamber & septal anatomy
1) Type : Supracardiac 45% . LA & LV : small
Cardiac 25% . ASD or PFO : small in 1/2,
Infracardiac 25% rarely no ASD or PFO
Mixed 5% 3. Pulmonary vasculature
2) Pulmonary venous obstruction . Increased arterial muscularity
. Junction of connecting vein . Structural change
or compression, or long 4. Associated condition
narrow connect vein . PDA : 15%
. Functional obstruction . VSD : occasionally
(restrictive PFO) . TOF, DORV, IAA : rarely
TAPVC
Types
TAPVC
Types
Supracardiac
Connecting vertical vein
TAPVC
Common vein
Cardiac Type
TAPVC
Common vein
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
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
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)
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
TAPVC
Supracardiac type
TAPVC
Cardiac type
TAPVC
Infracardiac type
TAPVC
Sutureless technique
• 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.
Primary Sutureless Repair
Rationale
• Small size of the pulmonary vein is a major risk factor
for later 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
TAPVC
Surgical results
1. Survival
. Small left ventricle
2. Modes of death
4. Functional status
. Hypertensive crisis
5. Hemodynamic result
. Pulmonary venous stenosis
6. Cardiac rhythm
3. Incremental risk factors
7. Reoperation
for death
. Anastomotic stricture
. Infracardiac drainage
(5~10%)
. Pulmonary venous obstruction . Pulmonary vein stenosis
. Poor preoperative state
. Small size of pulmonary vein
. Increased PVR
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
Residual TAPVC
PVD in remained anomalous veins
• Possible pressure-sensitive receptors at