Total Anomalous Pulmonary Venous Connection

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Total Anomalous Pulmonary Venous Connection

• Seoul National University Hospital
• Department of Thoracic Cardiovascular Surgery

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Total Anomalous Pulmonary Venous Connection

• Seoul National University Hospital
• Department of Thoracic Cardiovascular Surgery

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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 its 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

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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, and so on)

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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.
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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 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.

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TAPVC

• Morphology

• 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

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TAPVC

• Types

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TAPVC

• Types

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Supracardiac TAPVC
Connecting vertical vein
Common vein
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Cardiac Type TAPVC
Common vein
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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

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TAPVC

• Natural history
• 1. Incidence
• . Relatively uncommon anomaly, 1.53 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
• 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)

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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

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TAPVC

• Supracardiac type

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TAPVC

• Cardiac type

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TAPVC

• Infracardiac type

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Sutureless technique
TAPVC

• 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.

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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 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

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TAPVC

• Surgical results

• 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
• (510)
• . Pulmonary vein stenosis

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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

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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

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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)

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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

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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 repair of 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
  

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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 repair of acquired PV stenosis have
  also been less than optimal due to the problem of
  restenosis

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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.

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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

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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.