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Total Anomalous Pulmonary Venous Connection

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Title: Total Anomalous Pulmonary Venous Connection


1
Total Anomalous Pulmonary Venous Connection
  • Seoul National University Hospital
  • Department of Thoracic Cardiovascular Surgery

2
Total Anomalous Pulmonary Venous Connection
  • Seoul National University Hospital
  • Department of Thoracic Cardiovascular Surgery

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 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

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
  • 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.
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
  • 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

8
TAPVC
  • Types

9
TAPVC
  • Types

10
Supracardiac TAPVC
Connecting vertical vein
Common vein
11
Cardiac Type TAPVC
Common vein
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, 1.53 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

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

16
TAPVC
  • Supracardiac type

17
TAPVC
  • Cardiac type

18
TAPVC
  • Infracardiac type

19
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.

20
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

21
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

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 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

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 repair of 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.
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