TOTAL ANOMALOUS PULMONARY VENOUS

CONNECTION
Definition
 TAPVC is the anomaly in which the pulmonary veins have no
connection with the left atrium.Rather,the pulmonary veins
connect directly to one of the systemic veins.A PFO or ASD is
present essentially in those who survive after birth (obligatory
shunt).

 When pulmonary veins drain anomalously into the right

atrium either because of complete absence of the interatrial
septum or malattachment of the septum primum,then it is
known as total anomalous pulmonary venous drainage.
History

 WILSON-first description in 1798.The Philosophical

Transactions of the Royal Society in London published,``A
description of a very unusual formation of the heart“, total
anomalous pulmonary venous connection.
Wilson et.al.Philos.Trans.R.Soc.Lon.88;346,1798

 MULLER-first successful open repair in 1951.

Incidence
 TAPVC occurs in approximately 4-6 per 100,000 live births.
 This anomaly accounts for 1-3 percent of the cases of
congenital heart disease.
Incidence cont.

 Sex ratio with supradiaphragmatic TAPVC is about equal.

 Male predominance (4:1) is seen in infradiaphragmatic

type of TAPVC.
Associated cardiac anomalies

 TGA
 TOF
 Single ventricle
 Truncus arteriosus
 Tricuspid atresia
 HLHS
 CoA
 Asplenia or polysplenia
Embryology
VENOUS SYSTEM OF THE EMBRYO

 VITELLINE VEINS (OMPHALOMESENTERIC VEINS)-

These veins carry blood from the yolk sac to the sinus venosus.

 UMBILICAL VEINS-
Carrying oxygenated blood from chorionic villi to the embryo.

 CARDINAL VEINS-
These veins drain the body of the embryo proper.
Embryology cont.
VENOUS SYSTEM OF THE EMBRYO

The right cardinal venous system develops into the right SVC
whereas the left cardinal venous system mostly disappears and
may potentially develop into left SVC (<1% of individuals).

The umbilicovitelline veins develop into the IVC,portal

venous system and ductus venosus.
Embryology cont.

 In the embryo,the primordia of the lungs,larynx and

tracheobronchial tree are derived from a division of the
foregut.So,during early stage of development,lungs are
enmeshed by the vascular plexus of the foregut (splanchnic
plexus).

 At this stage lungs has no direct connection with the

heart.There are multiple connections with the splanchnic
plexus i.e,umbilicovitelline and cardinal venous systems.

Edwards JE. Mayo Clin Proc 1953;28:441-452

Embryology cont.
 In early stage of embryo,the lung
buds are enmeshed by the
vascular plexus of the foregut
(splanchnic plexus).

 A small evagination arises in the

posterior wall of the left atrium to
the left of the developing septum
secundum.It forms the common
pulmonary vein.

Pediatr Clin North Am 1963;10:781-836

Embryology cont.

 By the end of the 1st month of

gestation common pulmonary vein
establishes connection between
the pulmonary venous plexus and
the sinoatrial portion of the heart.

 Connection b/w pulmonary

venous plexus and the splanchnic
venous plexus are still patent.

Pediatr Clin North Am 1963;10:781-836

 Embryology cont.

 The connection b/w pulmonary venous plexus and splanchnic

venous plexus involutes.
 Common pulmonary vein incorporates into the left atrium so
that individual pulmonary veins connect separately and directly
to the left atrium.

Pediatr Clin North Am 1963;10:781-836

Embryology cont.

TOTALANOMALOUS PULMONARY VENOUS CONNECTIONS

 TAPVC results due to failure to establish connection b/w

pulmonary venous plexus and the common pulmonary vein
before the connections with splanchnic venous system have
regressed.
Edwards JE. Mayo Clin Proc 1953;28:441-452
Embryology cont.

 When there is stenosis of connection between common

pulmonary vein and left atrium,the common pulmonary
vein dilates and k/a COR TRIATRIATUM.
Anomalous connections
1. Connections to right atrium

2. Connections to right common cardinal system (SVC or

azygous vein)

3. Connections to left common cardinal system (left

innominate vein or coronary sinus)

4. Connections to umbilico-vitelline system (portal vein,ductus

venosus or hepatic veins)
 Classification of TAPVC
 Darling`s classification (1957) is the most popular.

1. Type I-Supracardiac connections : 45% of TAPVC patients.The

common venous confluence joins SVC.
2. Type II-Cardiac TAPVC :It accounts for 25% of cases.CVC drains into
coronary sinus or directly into RA.
3. Type III-Infracardiac TAPVC: Approx.21% of cases.CVC drains into
hepatic vein,ductus venosus,portal vein or IVC.The common
pulmonary vein penetrates the diaphragm through the esophageal
hiatus.
4. Type IV-Mixed Type: It accounts for <10% of cases.

Darling RC et.al. Lab Invest.1957;6:44-64

Karamlou T et.al. Circulation 2007;115:1591-1598
Classification cont.
 Neill classification (1956)-embryologic basis(not
commonly used)
1. Group having connection to the right atrium or right
common cardinal system(SVC and the azygous veins)

2. Connections to the left common cardinal system(left

innominate vein,left SVC or the coronary sinus)

3. Connections to the umbilicovitelline system(portal

vein,ductus venosus or hepatic veins)

Neill CA:Pediatrics 18:880,1956

Classification cont.

 Smith et.al classification

1. Supradiaphragmatic (without pulmonary venous

obstruction)

2. Infradiaphragmatic (with pulmonary venous

obstruction)

Smith et.al. Am J Dis Child 1961;101:41-51

Classification cont.
Herlong and colleagues suggested complete description
of the TAPVC including-

 Level of connections-supracardiac,cardiac,infracardiac or
mixed.

 Presence or absence of obstruction

 Cause of obstruction-extrinsic,intrinsic or obstructive atrial

septal communication.

Herlong et.al.Ann Thorac Surg 69(suppl):S56,2000

Sites of obstruction

 Obstruction at the Interatrial Septum

The longevity in TAPVC is related to the size of the ASD.
Those patients with large defects survived longer than did
pts.with restricted interatrial openings.
Burroughs and Edwards.Am Heart J 1960;59:913-931
 Obstruction in the anomalous venous channels
Intrinsic narrowing is frequent in the walls of anomalous
venous channels. Extrinsic venous compression may also occur.
Infracardiac type is usually obstructive while
supracardiac and cardiac type are often nonobstructive.
Sites of obstruction
cont.

 Extrinsic pressure may result in narrowing of the venous

structure.e.g when the vertical vein to the innominate vein
passes between the dilated left main pulmonary artery
anteriorly and left bronchus posteriorly
Sites of obstruction cont.
 Anomalous venous connections to SVC may be obstructed
by the right pulmonary artery and trachea.
 Ductus venosus normally undergoes constriction which
results in pulmonary venous obstruction.
 When anomalous connection is to the portal vein,hepatic
sinusoids results in increased resistance to pulmonary
venous return.

 Another factor that may contribute to impedance of

pulmonary venous return is the length of the ascending or
descending vertical venous pathway.
Physiology
 All venous blood returns to the RA. So survival of the child
is dependent on the presence of a right to left intracardiac
shunt either PFO or ASD (obligatory shunt).
 The physiologic features depend on the distribution of mixed
venous blood b/w the pulmonary and systemic circulations.

 The major hemodynamics depend on-

1. Size of interatrial communication
2. Presence or absence of obstruction to PVR and
3. Relative resistance of systemic and pulmonary vascular
bed.
Physiology cont.
 In pts.with a restrictive interatrial communication,the
amount of blood to LA is limited and systemic output is
reduced.

 After birth PVR decreases and systemic demand increases-

pulmonary circulation increases.Pulmonary and systemic
venous return to RA increases RA pressure which results in
elevated pressure and congestion of both venous circuits.

 In presence of wide communication the distribution of

mixed venous blood depends on relative compliance of
atria & ventricles and the relative resistance of pulmonary
& systemic circulations (major determinant is pulmonary
vascular resistance).
Physiology cont.
Hemodynamic of unobstructed TAPVC is similar to ASD
 RA receives the entire venous return from systemic as well as
pulmonary circuits.
 After birth PVR decreases and RV compliance increases so
more blood comes to RV.
 Pulmonary blood flow is 3-5 times more than systemic.It
leads to RV failure in neonates and infants.
 Pressures in RA and LA are equal. Oxygen saturation is equal
in all cardiac chambers(85-90%).
 Later on pulmonary vascular disease develops which leads to
decreased pulm.flow and increases systemic desaturation,so
cyanosis becomes prominent.
Physiology cont.

Hemodynamics of obstructed type is similar to Mitral Stenosis

 When obstruction is there, pulmonary venous pressure

increases,which leads to increased hydrostatic pressure and
interstitial & alveolar edema.

 Infant becomes very sick and die due to pulmonary edema.

Those who survive develop pulmonary hypertension.
Clinical features

TAPVC without Pulmonary Venous Obstruction

 Patients are usually asymptomatic at birth.
 Tachypnea and feeding difficulties are the initial symptoms
usually within first few weeks of life.
 Then infants have recurrent resp.tract infections and failure to
thrive.
 Mild cyanosis is present because of adequate mixing of blood.
 Gradually they develop right heart failure and pulmonary
arterial hypertension.
Clinical features cont.

TAPVC with Pulmonary Venous Obstruction

 Tachypnea,tachycardia and cyanosis within few hours of birth
(usually did not appear in the first 12 hours of life).
D/D Respiratory distress syndrome-symptoms within 12 hours
of life.
 Dyspnea is severe because of marked pulmonary venous
congestion and cyanosis is marked because of reduced
pulmonary flow.
 If left untreated death may occur from pulmonary edema and
RV failure within few days or weeks of life.
Clinical features cont.
 Those who survive their first year almost always have
supradiaphragmatic connections,low pulmonary vascular
resistance and a nonrestrictive atrial septal defect.

 When an infradiaphragmatic venous channel traverses the

esophageal hiatus feeding,crying and straining cause
additional compression that aggravates the dyspnea and
cyanosis. (Lucas et.al. Am J Roentgenol.86;561,1961)

 Newborns with infradiaphragmatic connections and asplenia

may have major esophageal varices.
On examination

TAPVC without Pulmonary Venous Obstruction

 Mild cyanosis with features of CHF.

 Prominant precordium with left lower parasternal heave.
 S1 loud,S2 wide split and fixed with loud P2.RVS3 present.
 ESM 3-4/6 at upper sternal border (↑ pulmonary flow).
 PSM due to TR and MDM due to increased flow across TV may
be there.
On examination cont.

TAPVC with Pulmonary Venous Obstruction

 Clinical condition is grave with minimal cardiac findings.

 Signs of PAH present.
 Apex impulse is of RV type.
 S1 normal,S2 closely split,P2 loud.
 A short systolic murmur due to pulmonary artery dilatation.
 Liver is enlarged and tender.
ECG

 Tall peaked P wave in lead II characteristic of right atrial

enlargement is a constant finding.

 RAD and RVH is usually present (high voltage in right

precordial leads).

 Occasionally an incomplete RBBB pattern present.

 CXR- supracardiac TAPVC

Figure of 8 or snow-man
appearance-
 nonobstructive supracardiac
TAPVC to left innominate vein.
 This diagnostic sign is usually not
present in first few months of life.
CXR- obstructive TAPVC

Ground-glass appearance
 Diffuse reticular pattern
 Cardiac size is normal
 Kerley B lines may be present
 This pattern also seen in other
causes of pulmonary venous
obstruction.
Echocardiography
 2D echo with colour Doppler is the definitive non-invasive
method for diagnosis of TAPVC.

 The reported sensitivity and specificity of echocardiography

was 97% and 99% even before availability of colour doppler.
Huhta JC et al.Br Heart J 1985;53:525-534

 A sensitivity of 100% and specificity of 85% is claimed for

detection of obstruction by 2D echo with colour doppler.
J Am Coll Cardiol 1991;18:1746-1751
Echocardiography cont.
 Goals of echocardiography-
1. Size of pulmonary veins
2. Connection of all 4 major pulmonary veins to confluence and
any additional pulmonary veins.
3. Size of pulmonary venous confluence & its relation with LA.
4. Course of pulmonary venous channel and whether there is
obstruction to its flow.
5. To evaluate interatrial communication for obstruction.
6. Any additional cardiac anonmaly.

Moss and Adams`7th ed. Vol 2 chapter 37

Echocardiography cont.
Features common to all forms of TAPVC are-
 Signs of right ventricular volume overload.
 Inability to image the pulmonary veins entering the LA.

 Size of the individual pulmonary vein at the time of diagnosis

is a strong,independent predictor of survival.
 Smaller pulmonary veins were associated with poorer
prognosis.
Jenkins KJ et al. JACC 1993;22:201-206
Echocardiography cont.

A. Suprasternal long axis view showing VV,left innominate and right SVC.
B. Phasic pulmonary venous flow in VV and innominate implying absence of
pulmonary venous obstruction.
Echocardiography cont.

Parasternal LAX with dilated Modified A4C view- Pulmonary

coronary sinus bulging into the venous confluence (star) draining
left atrium. into coronary sinus
Echocardiography cont.

Subcostal view showing pulmonary Continuous,turbulant and non-phasic

venous confluence (star) traced to pulmonary venous flow indicating
descending vein (arrow) that pulmonary venous obstruction
drains into portal vein.
CT and MRI

 CT excellently depicts vascular

structures peripheral to heart. With
newer MDCT scanners rapid imaging
is possible without need for
sedation.

 Disadvantage of CT is that it requires

ionizing radiations and IV iodinated
contrast material.

Dillman et al.AJR:192,May 2009

CT and MRI
 MRI is the preferred imaging
technique for evaluation of
pulmonary venous structures after
echocardiography. Lack of ionizing
radiation and need for single IV bolus
gadolinium contrast are advantages of
MRI.

 Long time for acquisition and

susceptibility for metal artifact are
disadvantages.

Dillman et al.AJR:192,May 2009

Cardiac catheterization

 Diagnostic catheterization is rarely performed for TAPVC diagnosis.

 Cardiac cath.is reserved for precise examination of pulmonary veins and their
obstruction.

 The pathognomomic finding is oxygen saturation in all chambers and great

vessels are nearly identical(80-95%).

 When TAPVC is to left innominate vein or right SVC, SVC blood preferentially
flows into tricuspid orifice and IVC blood preferentially shunts into the left
atrium, resulting in a pulmonary artery O2 saturation that may be higher
than that in the systemic artery.
Cardiac catheterisation

Selective pulmonary
arteriography-

 If pulmonary veins cannot be

entered directly then selective
RIGHT pulmonary artery
angiography is done.

 Pulmonary arteriography in
levophase shows the anomalous
venous connections.
Cardiac catheterization
 In infracardiac type, anomalous
connection of pulmonary veins via
descending vertical vein to portal
vein is characteristic and it is
termed as TREE IN WINTER.

 In neonates, umbilical vein

catheterization allows direct
injection of contrast in anomalous
connection in the
infradiaphragmatic type of TAPVC.
Tynan M.Br Heart J.1974;36:115
Catheterization is also helpful in defining pulmonary vein stenosis in
post TAPVC repair patients.
Natural history
 Among patients of TAPVC of all types,50% die at 3 months and
almost 80% die by the age of 1 year.
Keith et al. Am J Med 1954;16:23-38
Burroughs & Edwards,Am Heart J 1960;59:913-931
 When obstruction exists in anomalous venous channels,the
prognosis is grim. Death usually occurs within the first few
weeks of life.

 Patients who survive infancy as a consequence of increased

pulmonary vascular resistance, which is a mixed blessing
and may adversely affect the subsequent attempts of
surgical repair.
Natural history cont.

 Hazelrig and colleagues analysed 183 autopsied cases of

surgically untreated TAPVC.
 Median survival was 2 months (shortest-1 day,longest 49 yrs)
and 90% of deaths were within 1 year of life.
 Pulmonary venous obstruction significantly reduced survival
from 2.5 months in nonobstructive group to 3 weeks in the
obstructive group(p<0.0001).

Hazelrig JB et al.Biometrics 1982;38:1

Complications

1. Congestive cardiac failure

2. Growth and developmental delay
3. Frequent respiratory infections
4. Pulmonary vascular disease
5. Pulmonary edema in obstructive type.
Management

 Corrective surgery is the definitive

treatment.
Management

 Infants presenting with obstructed TAPVC represent surgical

emergency. They need require intentive resuscitation before
going for definitive surgery.

 Nonobstructed TAPVC patient are relatively stable and can be

taken for elective corrective surgery within few days of
diagnosis irrespective of patients age and weight.

Mastery of Cardiothoracic Surgery 2nd ed.

Sabiston & Spencer Surgery of the Chest 7th ed.
Comprehensive surgical management of CHD by Jonas
Emergency medical management

 Immediate endotracheal intubation and hyperventilation with

100% oxygen to a PaCO2 of ˃ 30 mm Hg and correction of pH.

 Induced respiratory alkalosis decreases pulmonary vascular

resistance and improves oxygenation.

 Metabolic acidosis should be treated with NaHCO3 or

Tromethamine (THAM) infusions.
 ECMO may be used in infants with severe pulmonary
hypertension or refractory cardiac failure.

 Balloon or blade atrial septostomy may be used as a palliative

procedure. It is not appropriate because it delays the definitive
procedure and is of no value in obstructed venous channel.
Surgery

 The goal of the surgery is to create an unobstructed egress of

blood from pulmonary veins into the left atrium.

 various approaches for surgery-

1. Posterior approach
2. Right atrial approach
3. Superior approach
De leval Surgery for CHD 3rd ed.
Kirklin and Barratt-Boyes Cardiac Surgery 3rd ed.
Sabiston & Spencer Surgery of the Chest 7th ed.
Posterior approach

 The pulmonary venous confluence is seen in the posterior

pericardium by retracting the heart anteriorly and to the right.

 Because of lack of pulmonary vein attachment to the left

atrium,the heart is unusually mobile and pulmonary venous
confluence can be easily exposed.

 A longitudinal incision is made in the venous confluence to

match a corresponding incision in the posterior left atrium.
Posterior approach

 Posterior approach is applicable to all types of TAPVC.

Posterior approach

 It is challenging to orient left atrial and pulmonary venous

confluence incisions while simultaneously retracting the heart.

 Retraction may place tension on the suture line of anastomosis.

 Limitation to rightward extension of the anastomosis. And more

so when the venous confluence is oriented rightward.
Right atrial approach

 A atriotomy incision extended across the right atrium and then

across the atrial septum.

 It allows incision of the posterior wall of the left atrium

precisely over the pulmonary venous confluence.

 In patients with small left atria and rightward pulmonary

venous confluence,this approach allows patch augmentation
of the left atrium when reconstructing the atrial septum and
right atrial incision.
Right atrial approach
Superior approach
 This approach is specially used in patients in whom the
pulmonary venous confluence drains to the SVC-RA junction.

 Aorta and SVC are retracted laterally exposing left atrium and
pulmonary venous confluence.

Anomalous vertical vein is routinally ligated in supracardiac

TAPVC but it is controversial in infracardiac type.
Repair of Cardiac type TAPVC
 Right atrial atriotomy done.

 Unroofing of the coronary sinus with incision b/w CS and

foramen ovale.

 Pericardial patch closure of the atrial septum done leaving the

pulmonary venous drainage to left atrium through unroofed
coronary sinus.
Repair of Cardiac type TAPVC
Infracardiac TAPVC

 The pulmonary venous confluence is more vertically oriented

so the incision in the left atrium is vertical or Y shaped to
maximise the size of the anastomosis.

 Vertical vein is left intact to provide pressure relief “POP OFF”

if left atrial pressure is high in early postoperative period b/c
of small left atrial size and poor ventricular compliance.
Cope JT et al.Ann Thorac Surg 1997;64(1):23-28
Surgical outcomes

 Surgical mortality has decreased from approx.50% in 1960 to

˃5% recently.

 Risk factors are-young age at operation,pulmonary venous

obstruction,infracardiac TAPVC,emergent operations,refractory
pulmonary hypertension and cardiac failure.
Complications of surgery
EARLY-
 Pulmonary edema
 Pulmonary hypertensive crisis
 Phrenic nerve damage
 Rhythm disorders

LATE-
 Pulmonary venous obstruction
 Anastomotic stricture
 Pulmonary venous stenosis
 Pulmonary edema- noncompliant left heart and increased left
atrial pressure leads to pulmonary arteriolar vasoconstriction.
Diuretics are useful for treatment.

 Pulmonary hypertensive crisis-hyperventilation with 100%

oxygen and inhaled nitric oxide is the treatment of choice.
Infusion of prostacyclin may also be useful.

 Rhythm disorders- junctional rhythms and various types of

heart blocks are common in cardiac type TAPVC repair.
Conclusion
 TAPVC is a rare congenital heart anomaly but presents as a
surgical emergency in neonatal periods.

 Echocardiography is the diagnostic modality of choice.

 Cardiac catheterization is rarely needed for diagnosis.

 Surgical correction is the definitive treatment.

Thanks