TOTAL ANOMALOUS TOTAL ANOMALOUS PULMONARY VENOUS PULMONARY VENOUS

CONNECTIONCONNECTION

CJ JORDAANCJ JORDAAN29/01/0429/01/04

Definition:Definition:

Uncommon congenital cardiac defect (1-Uncommon congenital cardiac defect (1-3%) in which there is no direct 3%) in which there is no direct communication between the pulmonary communication between the pulmonary venous drainage and the Left Atrium.venous drainage and the Left Atrium.

All the pulmonary veins connect/drain to All the pulmonary veins connect/drain to the Right atrium.the Right atrium.

Embryology:Embryology:

Total anomalous pulmonary venous connection Total anomalous pulmonary venous connection (TAPVC) develops when the primordial (TAPVC) develops when the primordial pulmonary vein fails to unite with the plexus of pulmonary vein fails to unite with the plexus of veins surrounding the lung buds.veins surrounding the lung buds.

This results in return of pulmonary venous blood This results in return of pulmonary venous blood to the heart via a systemic vein, and to the heart via a systemic vein, and subsequently to the right atrium. subsequently to the right atrium.

Classification:Classification:

TAPVC is classified according to the site of connection:TAPVC is classified according to the site of connection:

Supra cardiac:Supra cardiac: includes connections to the left Innominate vein, the includes connections to the left Innominate vein, the SVC, or the AzygosSVC, or the Azygos

Cardiac:Cardiac: includes connections to the coronary sinus or directly to includes connections to the coronary sinus or directly to the right atriumthe right atrium

Infra cardiac:Infra cardiac: includes connections below the diaphragm to the includes connections below the diaphragm to the portal vein, hepatic veins, ductus venosus or IVC.portal vein, hepatic veins, ductus venosus or IVC.

Mixed:Mixed: involves connections of two or more of these types; at least involves connections of two or more of these types; at least one of the main lobar pulmonary veins is draining differently from one of the main lobar pulmonary veins is draining differently from the others the others

Each category can be further classified as obstructive or non-Each category can be further classified as obstructive or non-obstructive.obstructive.

1. Supra cardiac TAPVC:1. Supra cardiac TAPVC:

Most common form of TAPVC---45%Most common form of TAPVC---45% Bilat draining of pulmonary veins (PV) to Bilat draining of pulmonary veins (PV) to

Common Pulmonary Venous Sinus (CPVS).Common Pulmonary Venous Sinus (CPVS). This confluence drains via Vertical Vein (VV) to This confluence drains via Vertical Vein (VV) to

Innominate vein, SVC or Azygos.Innominate vein, SVC or Azygos. Stenosis common ( +/- 40-60%):Stenosis common ( +/- 40-60%):

Pulmonary vein systemic connection,Pulmonary vein systemic connection,

Vascular vice orVascular vice or

Long thin VVLong thin VV

-CPVC location…-CPVC location…

Supra cardiac TAPVC Supra cardiac TAPVC - -Sites of stenosisSites of stenosis……

2. Cardiac TAPVC:2. Cardiac TAPVC:

Accounts for Accounts for 25%25% of TAPVC of TAPVC VV drains mostly to Coronary Sinus, seldom directly to VV drains mostly to Coronary Sinus, seldom directly to

Right atrium.Right atrium. Enlarged coronary sinus acts as the CPVS with only a Enlarged coronary sinus acts as the CPVS with only a

thin wall of myocardium separating sinus and the Left thin wall of myocardium separating sinus and the Left atrium.atrium.

Right atrialRight atrial draining: Seen in RA isomerism. Associated draining: Seen in RA isomerism. Associated with absent Coronary sinus, huge or absent intra atrial with absent Coronary sinus, huge or absent intra atrial septum. VV opens as a fibrous midline confluence.septum. VV opens as a fibrous midline confluence.

STENOSISSTENOSIS: Rare. May occur where the CPVS joins the : Rare. May occur where the CPVS joins the Coronary sinus, or at the mouth of the coronary sinus Coronary sinus, or at the mouth of the coronary sinus (persisting Thebesian valve)(persisting Thebesian valve)

3. Infra cardiac TAPVC:3. Infra cardiac TAPVC:

Accounts for Accounts for 25%25% of TAPVC of TAPVC The common pulmonary vein drains through the The common pulmonary vein drains through the

diaphragm to the portal vein, ductus venosus or diaphragm to the portal vein, ductus venosus or seldom to the IVC.seldom to the IVC.

Has the greatest propensity forHas the greatest propensity for STENOSIS STENOSIS::

-May be compressed where it penetrates the Diaphragm,-May be compressed where it penetrates the Diaphragm,-Connecting vein is narrowed at its junction with the portal -Connecting vein is narrowed at its junction with the portal vein,vein,-VV has a thickened wall with intimal proliferation,-VV has a thickened wall with intimal proliferation,-portal sinusoids (Liver) offer additional obstruction to -portal sinusoids (Liver) offer additional obstruction to venous returnvenous return

Infra cardiac TAPVC:Infra cardiac TAPVC:

4. Mixed APVC4. Mixed APVC

Involves a combination Involves a combination of connections of two of connections of two or more of the or more of the subtypes (at least one subtypes (at least one of the main lobar of the main lobar pulmonary veins is pulmonary veins is draining differently draining differently from the others.)from the others.)

AnatomyAnatomy

RA:RA: Enlarged and thick walled. Decreased Enlarged and thick walled. Decreased compliance.compliance.

LA:LA: Volume 53% less than predicted. LA auricle Volume 53% less than predicted. LA auricle is normal in size, decrease in LA can be is normal in size, decrease in LA can be explained by the absence of the pulmonary vein explained by the absence of the pulmonary vein component.component.

ASD:ASD: ASD or PFO must exist for survival. ASD or PFO must exist for survival. Usually of adequate size and not obstructive. Usually of adequate size and not obstructive. Obstruction leads to a decreased R to L shunt Obstruction leads to a decreased R to L shunt with pulmonary venous obstruction and Pulm. with pulmonary venous obstruction and Pulm. Hypertension. Presents as a severely sick Hypertension. Presents as a severely sick neonate.neonate.

AnatomyAnatomy

LV:LV: Normal in size, wall thickness and mass, but decreased Normal in size, wall thickness and mass, but decreased LV cavity ( due to leftward displacement of septum LV cavity ( due to leftward displacement of septum secondary to right ventricle pressure-volume overload.)secondary to right ventricle pressure-volume overload.)

RV:RV: Varies in size, depends on magnitude of pulmonary Varies in size, depends on magnitude of pulmonary blood flow, pulmonary venous stenosis, point of PV blood flow, pulmonary venous stenosis, point of PV connection. (Infra cardiac connection- RV not dilated or connection. (Infra cardiac connection- RV not dilated or hypertrophied)hypertrophied)

PA: PA: Most infants have marked pulmonary hypertension. Most infants have marked pulmonary hypertension. Structural changes are usually found in the lungs even in the Structural changes are usually found in the lungs even in the youngest infants dying of TAPVC. Increase in pulm. Arterial youngest infants dying of TAPVC. Increase in pulm. Arterial muscularity-increase in wall thickness and extension of muscularity-increase in wall thickness and extension of muscle into smaller and peripheral arteries. Vein wall muscle into smaller and peripheral arteries. Vein wall thickness is also increased.thickness is also increased.

Pathophysiology:Pathophysiology:

All pulmonary venous All pulmonary venous blood returns to the right blood returns to the right atrium. (common mixing atrium. (common mixing chamber)chamber)

A A right-to-leftright-to-left shunt at the shunt at the atrial level (-RV compl, atrial level (-RV compl, ASD size, Rp)ASD size, Rp)

Increased pulmonary Increased pulmonary blood flow and pulmonary blood flow and pulmonary venous obstruction will venous obstruction will eventually result in eventually result in pulmonary hypertension.pulmonary hypertension.

Infra diaphragmatic Infra diaphragmatic draining….draining….

Pathophysiology:Pathophysiology:

Pulmonary vein stenosis:Pulmonary vein stenosis: Obstruction to pulmonary draining with increase in pulm. Obstruction to pulmonary draining with increase in pulm.

venous pressure.venous pressure. Capillary leak with interstitial edema.Capillary leak with interstitial edema. Reflex pulm. vaso constriction and progressive increase Reflex pulm. vaso constriction and progressive increase

in Rp =pulmonary hypertension.in Rp =pulmonary hypertension. Increased PAP leads to increased RV pressures Increased PAP leads to increased RV pressures

(sometimes supra systemic) with RV failure, decreased (sometimes supra systemic) with RV failure, decreased pulm. blood flow, decreased Qp:Qs, decreased systemic pulm. blood flow, decreased Qp:Qs, decreased systemic SAT, peripheral hypoxia and metabolic acidosis with SAT, peripheral hypoxia and metabolic acidosis with multi organ failure.multi organ failure.

PFO obstructionPFO obstruction: Increased LAP, impedes pulm. venous : Increased LAP, impedes pulm. venous return, producing pulm. hypertension. return, producing pulm. hypertension.

Burchell principle:Burchell principle:

A direct relationship exists between the A direct relationship exists between the magnitude of pulm. blood flow and systemic magnitude of pulm. blood flow and systemic saturation.saturation.

The Qp:Qs is determined by magnitude of The Qp:Qs is determined by magnitude of pulmonary blood flow, pulm. blood flow is pulmonary blood flow, pulm. blood flow is inversely related to Rinversely related to Rp. p.

Thus an increase in PAP and Rp leads to a Thus an increase in PAP and Rp leads to a decrease in pulm blood flow with a decreased decrease in pulm blood flow with a decreased systemic SAT.systemic SAT.

SAT less than 80%: Qp:Qs likely to be < 1.4 and SAT less than 80%: Qp:Qs likely to be < 1.4 and Rp > 10 !!!!!!!Rp > 10 !!!!!!!

Clinical features:Clinical features:

Clinical features are determined by the degree of pulmonary Clinical features are determined by the degree of pulmonary venous obstruction.venous obstruction.

If If obstruction is severeobstruction is severe, infant will be critically ill with tachypnea, , infant will be critically ill with tachypnea, hypoxemia, and metabolic acidosis. Cyanosis can be hypoxemia, and metabolic acidosis. Cyanosis can be unimpressive to severe, this is a surgical emergency.unimpressive to severe, this is a surgical emergency.

Prognosis: 50% die within 3 weeks, 75% at 5 weeks and 100% Prognosis: 50% die within 3 weeks, 75% at 5 weeks and 100% at 8-12 weeksat 8-12 weeks

No obstruction:No obstruction: Not so critically ill. May present later with Not so critically ill. May present later with pulmonary hypertension, cardiomegaly, large pulm blood flow. pulmonary hypertension, cardiomegaly, large pulm blood flow. Patients surviving first year of life present with failure to thrive, Patients surviving first year of life present with failure to thrive, severe growth retardation, mild cyanosis. Stable hemodynamic severe growth retardation, mild cyanosis. Stable hemodynamic state with progressive pulmonary hypertension and development state with progressive pulmonary hypertension and development of Eisenmenger in their twenties.of Eisenmenger in their twenties.

Diagnosis:Diagnosis:

CXR:CXR: Shows ground glass (diffuse alveolar Shows ground glass (diffuse alveolar

pattern) or "snowman" appearance from pattern) or "snowman" appearance from persistent vertical vein, small heart = pulm persistent vertical vein, small heart = pulm stenosis.stenosis.

No stenosis: Large heart with increased No stenosis: Large heart with increased pulm blood flow.pulm blood flow.

There is cardiomegaly There is cardiomegaly with increased pulmonary with increased pulmonary arterial markings. There arterial markings. There is dilation of both the left is dilation of both the left and right innominate and right innominate veins and the right veins and the right superior vena cava superior vena cava producing the classical producing the classical "snowman" or "figure of "snowman" or "figure of 8" appearance. The 8" appearance. The superior mediastinum is superior mediastinum is enlarged secondary to enlarged secondary to dilation of the right vena dilation of the right vena cava, innominate vein cava, innominate vein and ascending vertical and ascending vertical vein. vein.

Neonate –no stenosis Neonate with stenosis

Diagnosis:Diagnosis:

Echo:Echo:

Diagnostic in most Diagnostic in most infants.infants.

Evaluation of cardiac Evaluation of cardiac chambers, Echo free chambers, Echo free space posterior to LA space posterior to LA with abnormal with abnormal drainagedrainage

Diagnosis:Diagnosis: AngiographyAngiography

Diagnosis:Diagnosis: MRI angiogram:MRI angiogram:

TREATMENT:TREATMENT:

Patients with TAPVC should undergo operative Patients with TAPVC should undergo operative repair when the diagnosis is maderepair when the diagnosis is made

Obstructed TAPVC is a surgical emergencyObstructed TAPVC is a surgical emergency Non-obstructed TAPVC should have prompt Non-obstructed TAPVC should have prompt

repair as well, as the clinical status of these repair as well, as the clinical status of these patients can deteriorate rapidlypatients can deteriorate rapidly

Early repair of non-obstructed TAPVC also Early repair of non-obstructed TAPVC also prevents the adverse squeal of cyanosis and prevents the adverse squeal of cyanosis and volume overload of the heart and lungs volume overload of the heart and lungs

Treatment:Treatment:

Pre operatively:Pre operatively: Admit in PICUAdmit in PICU Intubation and ventilationIntubation and ventilation Tolazoline, PGE1 IVITolazoline, PGE1 IVI Stabilize, correct metabolic acidosis, Stabilize, correct metabolic acidosis,

confirm diagnosisconfirm diagnosis Prepare for emergent theatre.Prepare for emergent theatre.

Operative TechniqueOperative Technique

Cardiopulmonary bypass with hypothermic Cardiopulmonary bypass with hypothermic circulatory arrest is the preferred approach in circulatory arrest is the preferred approach in critically ill infantscritically ill infants

Surgical goals are: eliminating all anomalous Surgical goals are: eliminating all anomalous connections, draining the pulmonary veins into connections, draining the pulmonary veins into the left atrium, and closing intracardiac shunts, the left atrium, and closing intracardiac shunts, small PFO is left for RV decompressionsmall PFO is left for RV decompression

The PV-LA anastomosis must be large and The PV-LA anastomosis must be large and undistorted .undistorted .

Problems:Problems: Small aorta Small aorta Hypothermic CPB responseHypothermic CPB response

Left SVC connectionLeft SVC connection· Ligate left-sided vertical vein at junction with · Ligate left-sided vertical vein at junction with Innominate veinInnominate vein· Open left atrium and incise the posterior wall· Open left atrium and incise the posterior wall· Find and incise the anterior wall of the confluence· Find and incise the anterior wall of the confluence·· Anastomose the pulmonary venous confluence to the the pulmonary venous confluence to the left atriumleft atrium· Close PFO or ASD through left atrium or through · Close PFO or ASD through left atrium or through separate right atrial incision separate right atrial incision

Right SVC connectionRight SVC connection· Expose pulmonary venous confluence and anastomose · Expose pulmonary venous confluence and anastomose to left atrium as aboveto left atrium as above· Ligate anomalous connections or patch from within · Ligate anomalous connections or patch from within SVCSVC· A baffle may be used instead to channel flow from the · A baffle may be used instead to channel flow from the right SVC through an enlarged interatrial connection right SVC through an enlarged interatrial connection

Azygos connectionAzygos connection· Ligate anomalous connection· Ligate anomalous connection· Anastomose confluence to left atrium as above· Anastomose confluence to left atrium as above

2. Cardiac Type2. Cardiac Type Coronary sinus connectionCoronary sinus connectionClassic repair:Classic repair:

· Create common large interatrial connection by incising · Create common large interatrial connection by incising coronary sinus septum and septum primumcoronary sinus septum and septum primum· Close this new defect with a single patch; all pulmonary · Close this new defect with a single patch; all pulmonary venous return and coronary sinus return now drains into venous return and coronary sinus return now drains into the left atriumthe left atrium

Van Praag Van Praag

Right atrium connectionRight atrium connection· Enlarge interatrial connection· Enlarge interatrial connection· Create baffle to direct flow from pulmonary venous · Create baffle to direct flow from pulmonary venous opening across interatrial connectionopening across interatrial connection

3. Infracardiac Type3. Infracardiac Type

· Ligate PDA once CPB is established· Ligate PDA once CPB is established· Identify and ligate anomalous descending · Identify and ligate anomalous descending vertical vein at the diaphragmvertical vein at the diaphragm· Initiate circulatory arrest· Initiate circulatory arrest· Open left atrium and incise the posterior wall· Open left atrium and incise the posterior wall· Find and incise the anterior wall of the · Find and incise the anterior wall of the confluenceconfluence· Anastomose the pulmonary venous confluence · Anastomose the pulmonary venous confluence to the left atriumto the left atrium· Close the interatrial communication· Close the interatrial communication

4. Mixed Type4. Mixed Type

Problems with Mixed Type:Problems with Mixed Type:· No pulmonary venous confluence· No pulmonary venous confluence· Requires 2 or more anastomosis· Requires 2 or more anastomosis· Smaller anastomosis predispose to · Smaller anastomosis predispose to pulmonary venous obstruction pulmonary venous obstruction · Risk of mortality and late pulmonary · Risk of mortality and late pulmonary venous obstruction is increased venous obstruction is increased · Operative repair probably not curative · Operative repair probably not curative

Post operative :Post operative :Pulmonary hypertensive crisis….Pulmonary hypertensive crisis….1.1. Sedation, ventilation, Fentanyl analgesiaSedation, ventilation, Fentanyl analgesia2.2. Pco2 (<30mmHg) and high PO2Pco2 (<30mmHg) and high PO23.3. TNT, PGE1, Dobutamine, Tolazoline, Adrenaline etc…..TNT, PGE1, Dobutamine, Tolazoline, Adrenaline etc…..

ResultsResults· Early (hospital) mortality ranges from 2-20%· Early (hospital) mortality ranges from 2-20%· 90% of patients are cured· 90% of patients are cured· 5-10% have late failure of repair due to pulmonary · 5-10% have late failure of repair due to pulmonary venous obstructionvenous obstruction

LA size adequate due to incorporation of PVC into LALA size adequate due to incorporation of PVC into LA

Post operative :Post operative :

Risk Factors:Risk Factors:· Poor pre-operative status (acidosis)· Poor pre-operative status (acidosis)· Pulmonary venous obstruction, small · Pulmonary venous obstruction, small PFO PFO

· High pulmonary vascular resistance, pre · High pulmonary vascular resistance, pre operative SAT <80%operative SAT <80%· Young age· Young age· Small left ventricle· Small left ventricle· Major associated anomalies· Major associated anomalies· Infracardiac or mixed type· Infracardiac or mixed type

Literature:Literature:

Bu’Lock FA et al. Balloon dilatation of vein stenosis in obstructedBu’Lock FA et al. Balloon dilatation of vein stenosis in obstructed TAPVCTAPVC. Ped Cardio 1998;15:78. Ped Cardio 1998;15:78

Balloon atrial septostomy: Risk factor for death, not an ameliorating Balloon atrial septostomy: Risk factor for death, not an ameliorating

factor. Balloon dilatation of left vertical vein in obstructive factor. Balloon dilatation of left vertical vein in obstructive supracardiac TAPVC may provide important clinical improvement supracardiac TAPVC may provide important clinical improvement and short term hemodinamic stability before operationand short term hemodinamic stability before operation

Kiziltepe U et al. Acute pulm hypertensive crisis after TAPVC repair…Kiziltepe U et al. Acute pulm hypertensive crisis after TAPVC repair…treatment. treatment. Internat J of Cardio 2002:87;107-109Internat J of Cardio 2002:87;107-109

Treatment of recurrent severe pulm hypertension refractive to medical Treatment of recurrent severe pulm hypertension refractive to medical treatment can be dealt with inflow occlusion and creation of large treatment can be dealt with inflow occlusion and creation of large intra atrial connection. Chest left open post operatively. intra atrial connection. Chest left open post operatively.

Post operative PV stenosis:Post operative PV stenosis:Stark J, De Leval MR. Management of pulm venous obstruction after Stark J, De Leval MR. Management of pulm venous obstruction after

TAPVC repair. Eur J Cardio-Thorac Surg. 2003:24:28-36 TAPVC repair. Eur J Cardio-Thorac Surg. 2003:24:28-36

Complicates surgery in 5-10%Complicates surgery in 5-10% Classification of PV stenosis:Classification of PV stenosis:

Discrete stenosisDiscrete stenosis

Tubular focal stenosisTubular focal stenosis

Diffuse hypoplasiaDiffuse hypoplasia May be severe anastomotic stenosis or May be severe anastomotic stenosis or

pulmonary vein stenosis.pulmonary vein stenosis.

Post operative PV stenosis:Post operative PV stenosis:

Predominate after correction of Infracardiac and cardiac Predominate after correction of Infracardiac and cardiac TAPVC.TAPVC.

Vary from Fibrosis and neo-intimal proliferation at the Vary from Fibrosis and neo-intimal proliferation at the anastomotic site, to segmental or diffuse intimal anastomotic site, to segmental or diffuse intimal hyperplasia with extension to the individual pulm veinshyperplasia with extension to the individual pulm veins

Abnormal architecture of pulm venous wall leads to Abnormal architecture of pulm venous wall leads to increased tendency to neo intimal proliferation with increased tendency to neo intimal proliferation with increase in intimal and muscular media.increase in intimal and muscular media.

Obstruction involving individual PV occurring early post Obstruction involving individual PV occurring early post op – Very difficult to treat with high re-stenosis rate. op – Very difficult to treat with high re-stenosis rate.

Poor prognosis. Poor prognosis. 66% early mortality.66% early mortality.

Post operative PV stenosisPost operative PV stenosis Risk factors:Risk factors:

Use of Gore-tex patchesUse of Gore-tex patchesEarly presentationEarly presentationdifficult anastomosisdifficult anastomosissmall pulmonary venous confluencesmall pulmonary venous confluence

Diagnosis:Diagnosis:1.1. Pulm venous flow patterns which does not reach baseline throughout the cardiac Pulm venous flow patterns which does not reach baseline throughout the cardiac

and resp cyclesand resp cycles2.2. Absence of pulm venous return from one lungAbsence of pulm venous return from one lung3.3. TR jet velocity > 3m per sec.TR jet velocity > 3m per sec.4.4. RV pressure >40 mmHgRV pressure >40 mmHg5.5. Increase in pulm blood flow>1.8 m per secondIncrease in pulm blood flow>1.8 m per second

Scrupulous and atraumatic technique still remain the best option Scrupulous and atraumatic technique still remain the best option for success. Use of absorbable sutures controversial.for success. Use of absorbable sutures controversial.

Post operative PV stenosis:Post operative PV stenosis:

Repair:Repair:100% success rate achievable in anastomotic stenosis. 100% success rate achievable in anastomotic stenosis.

Patch enlargement relieves stenosis and re stenosis is Patch enlargement relieves stenosis and re stenosis is uncommon,uncommon,

No surgical approach including: patch augmentation, No surgical approach including: patch augmentation, longitudinal incision with transverse plication, balloon longitudinal incision with transverse plication, balloon dilatation or placement of stents has shown any dilatation or placement of stents has shown any improvement in patient outcome.improvement in patient outcome.

Goldenar syndrome: Goldenar syndrome: (Infracardiac TAPVC, R lung (Infracardiac TAPVC, R lung hypoplasia, sub arterial VSD and ost secundum ASD) hypoplasia, sub arterial VSD and ost secundum ASD) has a particular tendency to PV stenosis irrespective of has a particular tendency to PV stenosis irrespective of type of repair. End with near atresia of PV’s type of repair. End with near atresia of PV’s