TOTAL ANOMALOUS PULMONARY VENOUS CONNECTION (TAPVC)

TAPVC defines 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 (TAPVC) or drain in to right atrium.
A PFO or ASD is present essentially in those who survive after birth
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.

When some or all of the pulmonary veins drain anomalously in to RA or its tributaries without being abnormally connected, the terms partially anomalous pulmonary venous drainage (PAPVD) or totally anomalous pulmonary venous drainage (TAPVD) with normal pulmonary venous connections are used.

1. TOTAL ANOMALOUS
PULMONARY VENOUS
CONNECTION
DR VISHWANATH HESARUR
SENIOR RESIDENT
DEPARTMENT OF CARDIOLOGY
JNMC , BELGAUM

2. DEFINITION
TAPVC defines 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 (TAPVC) or drain in to right atrium.
A PFO or ASD is present essentially in those who
survive after birth

3. HISTORY
Wilson : 1
st
description - case report in
1798.
Friedlowsky : 1
st
description of TAPVC in
1868.
 Muller : 1
st
Successful open repair in
1951.

4. INCIDENCE
0.6 to 1.2 per 10,000 live births

0.7 and 1.5 percent of all CHD

5. A strong male preponderance of 3:1
 
Birth weight was less than 2500 g in 16.2%

Gestational age was less than 38 weeks in 18.9%

Intrauterine growth retardation occurred in 26.8%
68% of these patients were diagnosed as neonates

6. GENETICS
Mechanism of transmission – Not been elucidated
No known genetic pattern of transmission.
Associated with syndromes most notably Asplenia ,
Polysplenia .

8. EMBRYOLOGY

9. PAPVC results from failure to establish a normal
connection between one or more of the pulmonary
veins with the CPV before the connections with the
splanchnic venous system have regressed.

10. TAPVC results from failure to establish a normal
connection between the pulmonary venous plexus
& the CPV before the connections with splanchnic
venous system have regressed .

11.  Atresia of the common pulmonary vein and 
cor triatriatum.

12. CLASSIFICATION OF TAPVC
Smiths classification
Neills classification - Based on embryologic
basis
Burroughs and Edwards classification
Darlings classification

13. Smith classification
Supradiaphragmatic Type: Pulmonary veins are
connected to: the left innominate vein by the
characteristic vertical vein, or the coronary sinus,
the right atrium, or the SVC, without pulmonary
venous obstruction
Infradiaphragmatic Type: Pulmonary veins drain
into the portal vein or hepatic veins, with
pulmonary venous obstruction.
Smith et.al. Am J Dis Child 1961;101:41-51

14. Neills classification
Group having connection to the right atrium or
right common cardinal system (SVC and the
azygous veins)
Connections to the left common cardinal
system(left innominate vein, left SVC or the
coronary sinus)
Connections to the umbilicovitelline system
(portal vein, ductus venosus or hepatic veins)
Neill CA: Pediatrics 18:880,1956

15. Burroughs and Edwards classification
Suggested a classification with prognostic
implications based on the length of of the
anomalous channel (long, intermediate, or short)
However the prognostic implications suggested
are not always true.
Burroughs and Edwards.Am Heart J 1960;59:913-931

16. Darlings classification
The most common classification system was originally
described by Darling et al. in 1957. consists of four
types
Type I (Supracardiac TAPVC ): 45% of cases.
Both right and left pulmonary veins join a common
pulmonary venous confluence behind the heart that
drains via a vertical vein to the undersurface of the left
innominate vein and thence to the Right atrium.
Type II ( Cardiac TAPVC) : 25% of cases.
The pulmonary venous confluence connects to the
coronary sinus, & thence to the RA via the coronary
sinus ostium.

17. Type III (Infracardiac TAPVC) : 21% of cases.
The pulmonary venous confluence drains
inferiorly via a vertical vein to the portal vein
or hepatic veins & thence to the RA.
Type IV (Mixed Type ) : <10% of cases.
Left pulmonary veins drain to the LIV , &
right pulmonary veins to the coronary sinus
Darling RC et.al. Lab Invest.1957;6:44-64

20. THE FREQUENCIES OF THE
VARIOUS SITES OF TAPVC OR
DRAINAGE
More than one-third of the cases had the
anomalous connection to the left innominate
vein (LIV).

21. ANATOMIC SITES OF OBSTRUCTIO
N TO  PULMONARY VENOUS
DRAINAGE
The presence of an obstructive lesion in the anomalous
 pulmonary venous channel profoundly influences the 
hemodynamic state and clinical features
Obstruction at the Interatrial Septum
Burroughs and Edwards (40) clearly related longevity in
TAPVC to the size of the ASD. 
Those patients with large defects survived longer than
did with restricted interatrial openings.

22. Obstruction in the Anomalous Venous Channel
Intrinsic narrowing in the walls of the anomalous vessels or
Extrinsic pressure results in narrowing of venous structure .

For example, when the vertical vein in TAPVC to the
innominate vein passes between the left main pulmonary
artery and left main bronchus.

23. Similarly, the anomalous pulmonary vein in TAPVC
 to the SVC may be obstructed by the right
pulmonary artery & trachea.. 
Finally, when the anomalous connection is to the 
portal vein or one of its tributaries, the hepatic
sinusoids are interposed in the pulmonary venous
channel and result in increased resistance to
pulmonary venous return.  

24. ASSOCIATED CARDIAC ANOMALI
ES
TAPVC occurs as an isolated anomaly in two third
cases.
It has been reported, however, to be associated wit
h TGA , TOF, PDA, single ventricle , truncus
arteriosus , tricuspid atresia , hypoplastic left heart
syndrome ,pulmonary atresia, multiple small
VSDs , COA , vascular sling, and other anomalies . 
Asplenia & polysplenia syndromes.

25. SK Choudhary, A Bhan, R Sharma, B Airan, V Devagourou, A Saxena,
SS Kothari, Total-Anomalous-Pulmonary-Venous-Connection: Surgical Experience in Indians
Cardiothoracic Centre, All India Institute of Medical Sciences, New Delhi .
Indian Heart J 2001; 53: 754–760)

26. PHYSIOLOGY
All venous blood returns to the RA. 
Communication between the right and left sides of 
the heart - essential for survival . 
Physiologic features depend on the distribution of
this mixed venous blood between the pulmonary
and systemic circulations. 
The state of the interatrial septum is of primary
importance in this distribution. 

27. During fetal life, PBF is small & the combined
systemic and pulmonary venous return to the RA is
only minimally increased.
Hence, the stimulus for the development of a large 
interatrial communication is minimal.
Some degree of restriction to fow across a patent
foramen ovale (found in 70% to 80% of cases) is
common.
 In patients with a restrictive interatrial
communication , the amount of blood reaching the
LA is limited and systemic output is reduced.

28.  As pulmonary vascular resistance gradually
decreases after birth and as demands for SBF
increase with the rapid growth of the infant, massive
pulmonary overcirculation ensues. 
Pulmonary and systemic venous blood return is to
the RA , therefore, increased right atrial pressure
results in pressure elevation and congestion in both
venous circuits.
On the other hand, the presence of a widely patent 
foramen ovale or ASD allows free communication
between the two atria.

29. In this circumstance, the distribution of mixed 
venous blood depends on the relative compliance of
the atria and ventricles and the relative resistance 
imposed by the pulmonary & systemic arterial
circuits.
 
The major variable is the state of the pulmonary
vascular bed , which initially depends on the
presence or absence of pulmonary venous 
obstruction.

30. TAPVC without Pulmonary Venous Obstruction
At birth, the distribution of blood between the 
pulmonary & systemic circuits is approximately
equal because the resistance in these two vascular
beds is nearly equal. 
In the first few weeks of life , maturation of the
pulmonary vascular bed produces a decrease in
pulmonay vascular resistance , & a progressively
larger proportion of the mixed venous blood
traverses the pulmonary circuit. 
PBF is three to five times SBF. SBF is usually
normal.

31. Progressive dilation and hypertrophy of RV and
dilation of the pulmonary artery usually occur.
Pulmonary artery pressure in infants ranges from 
slightly elevated to systemic. 
The state of the interatrial communication in 
patients with TAPVC without pulmonary venous
obstruction has a major impact on PBF , pressure
and resistance. 
In the few patients who survive to older childhood 
or early adulthood , PA pressure is only slightly
elevated. 

32. As time goes on, medial hypertrophy and 
intimal proliferation occur in the pulmonary
arterioles, resulting in more severe pulmonary
hypertension in the third and fourth decades.

33. TAPVC with Pulmonary Venous Obstruction
Elevated pressure in the pulmonary venous 
channels is transmitted to the pulmonary capillary 
bed - pulmonary edema.
The right ventricular volume and pressure overload
  result in a leftward shift of the interventricular
septum that, together with the decreased inflow
from the LA, lead to decrease in
left ventricular volume. 
Systemic output usually is low because of the
inadequate filling volume.

34. CLINICAL MANIFESTATIONS
The signs and symptoms in TAPVC are variable, 
depending on the underlying hemodynamics. 
When the interatrial communication is inadequate ,
symptoms occur at birth or shortly thereafter.
The hemodynamic consequences of inadequate 
interatrial communication include pulmonary 
venous obstruction. 
The presence of intrinsic or extrinsic narrowing in 
the connecting  vein also produces pulmonary
 venous obstruction. 

35. Thus, the manifestations may be divided 
according to whether pulmonary venous
obstruction is absent or present.

36. CLINICAL FEATURES
TAPVC without Pulmonary Venous  Obstruction
Asymptomatic at birth.
Tachypnea and feeding difficulties - within
first few weeks of life.
Have recurrent resp.tract infections and
failure to thrive.
Mild cyanosis
Gradually they develop right heart failure
and pulmonary arterial hypertension

37. A prominent right ventricular heave . 
A characteristic feature - multiple cardiac
sounds. 
S1is Loud and often is often followed by a
systolic ejection click. 
S2 widely split & does not vary with respiration,
The pulmonary component of the second sound
 is  accentuated. 
S3 maximal at apex almost always ,is present.
S4 is frequently heard in older patients.
Characteristically, a grade 2/6 soft , blowing ,
systolic ejection murmur is heard in pulmonary
area .

38. When the anomalous connection is to the LIV, a
venous hum at the left or right base may be heard.
 
 Hepatomegaly & peripheral edema
Clubbing occasionally is seen in the patient who 
survives infancy.

39. TAPVC with Pulmonary Venous Obstruction
Tachypnea, tachycardia and cyanosis within few
hours of birth.

Dyspnea - pulmonary venous congestion and
cyanosis - reduced pulmonary flow.
If left untreated death may occur from pulmonary
edema and RV failure within few days or weeks of
life.

40. Once symptoms began- rapid progression to
dyspnea , feeding difficulties and cardiorespiratory
failure.
Age at death ranged from 2 days to 4.5 months .
When the anomalous connection is below the
diaphragm , cyanosis & dyspnea may be
accentuated by straining & swallowing as a
consequence of interference of pulmonary venous
outflow by increased intra-abdominal pressure or
impingement of the esophagus on the CPV as it
exits through the esophageal hiatus.

41. The clinical course in patients with severely
obstructed infradiaphragmatic TAPVC might be
stormy with rapid development of severe respiratory
 distress and acidosis in the first hours of life.
Despite the alarming symptoms, the cardiovascular 
findings may be minimal. 
No significant right ventricular heave. 
S1 loud, S2 split, P2 loud  
A cardiac murmur often is absent, but, when 
present, it is usually a soft blowing systoloic
ejection murmur in the pulmonary area. 
Moist rales - lung bases. 
Hepatomegaly and peripheral edema.

42. ELECTROCARDIOGRAPHIC FEATUR
ES
TAPVC without Pulmonary Venous  Obstruction
A tall peaked P wave in lead II or the right 
precordial leads characteristic of RA enlargement.
Right-axis deviation.
 
Right ventricular hypertrophy –high voltage in the
right precordial leads

Occasionally as an incomplete RBBB pattern.

43. TAPVC with Pulmonary Venous  Obstruction
Right ventricular hypertrophy is invariably 
present.
 
Unlike TAPVC without obstruction ,
however RA enlargement is not a usual
feature.

44. CRITERIA OF RVH IN NEWBORNS
Pure R wave 10 mm (with no S waves )in V1.
˃
R wave in V1 25 mm or R wave in aVR 8 mm.
˃ ˃
S wave in lead I 12 mm or greater.
A qR pattern in V1.(also seen in10% of normal
newborns).
Extreme RAD.
Upright T waves in V1 after 1 week of age.

Normally T wave upright until 4 to 7 days of
age. Between 1 week to adolescence it is negative
and then reverts to upright.

45. RADIOLOGIC FEATURES
TAPVC without Pulmonary Venous  Obstruction
The RA and RV are enlarged, and the pulmonary
artery segment is prominent. 
The left-sided chambers are not enlarged.
A figure-of-8 or snowman appearance of the cardiac
shadow is seen in patients with TAPVC to the LIV . 

46.  “FIGURE OF EIGHT” OR “SNOWMAN'S”
APPEARANCE.

47. Ground-glass
appearance
Diffuse reticular pattern
Cardiac size is normal
Kerley B lines may be
present
TAPVC with Pulmonary Venous  Obstruction

48. ECHOCARDIOGRAPHIC FEATURE
S
Goals of the echocardiographic examination  
To establish the diagnosis; 
To image and determine the size of the individual
pulmonary veins ; 
To ascertain that all 4 pulmonary veins join the
pulmonary venous confluence and no additional
pulmonary veins drain separately ; 
To image and determine the size of the pulmonary
venous confluence and its relation to the LA; 

49. To image the course of the pulmonary venous 
channel (usually the vertical vein ), its connection
with systemic vein & its relation to neighboring
structures (i.e., pulmonary arteries and airways); 
To determine whether there is obstruction to
pulmonary venous flow ; 
To evaluate the interatrial communication for 
obstruction; and 
To perform a complete anatomic and functional 
survey of all cardiovascular structures & to exclude
additional structural cardiac anomalies. 
These goals are achieved by performing
a complete step by step
echocardiographic examination from multiple
windows.

50. The features common to all forms of TAPVC are
 
Signs of Rright ventricular volume overload. 
The right-sided heart structures are dilated. 
The RA is enlarged, and the atrial septum
bows toward the left. 
The right ventricle appears to compress
the left ventricle, the interventricular septum 
deviates leftward,and left ventricular volume is 
decreased. 
The IVS septum may move paradoxically. 
The pulmonary arteries are dilated.

51. Features of right ventricular volume overload, the 
first echocardiographic suspicion that supports the
diagnosis of TAPVC is the inability to image the
pulmonary veins entering the LA & LA is small. 
The pulmonary venous confluence - echo free space
behind the LA.
The individual pulmonary veins - parasternal ,
subclavicular & suprasternal notch views mostly
used.

52. Once the pulmonary venous confluence is characte
rized, the venous channel that connects with the
systemic vein is followed by 2-D imaging
and color Doppler flow mapping. 
The venous channel in 
Supracardiac TAPVC - precordial windows
Infradaiphragmatic TAPVC - subcostal view.
 In supracardiac TAPVC, the venous channel
should be examined for its relation with the branch
 pulmonary arteries and the bronchi.

54. In TAPVC to coronary sinus, the sinus is
dilated and bulges anterosuperiorly into the
LA.
Imaging of the pulmonary veins draining into
the coronary sinus is important for diagnosis
because CS may be dilated in other conditions
also like persistent LSVC to CS.
Descending anomalous vein is characterized
by the venous flow pattern and direction of
flow is away from the heart towards abdomen.

56. An increased flow velocity, turbulent flow pattern
and loss of phasic variations characterize
obstructed pulmonary venous flow. (normal venous
flow is low velocity, phasic laminar pattern with
brief flow reversal during atrial systole)

58. MRI AND CT IMAGING

62. CARDIAC CATHETERIZATION
TAPVC without Pulmonary Venous  Obstruction
The venous site of anomalous connection may be 
identified if highly saturated blood is obtained from
LIV, right SVC, or CS. 
In TAPVC, the oxygen saturation in the RA- usually
ranges between 80% and 95%, and saturations in 
the RA, RV, PA, LA, LV and systemic arteries are
nearly identical.
Pressure in RV & PA ranges from slightly elevated to
equal or higher than systemic pressure.

63.  
Interpretation of atrial pressures – adequacy of
the interatrial communication, is difficult.
 
The presence of equal pressures in the two atria -
nonobstructive intera-trial communication. 
A RA pressure >2 mm Hg in excess of LA pressure -
restrictive interatrial communication.

64. TAPVC with Pulmonary Venous  Obstruction
Difficult in patients with obstructed TAPVC
Must be avoided – May aggravate already
compromised clinical condition of these patients
and delay operation.
Right ventricular pressures usually are systemic or 
higher.
Pressures in the RA usually are normal.
LA pressure is normal.

65. Selective pulmonary arteriography - in levophase shows the
anomalous venous connections.

66. THE TREE IN WINTER –
INFRACARDIAC TYPE

68. NATURAL HISTORY
Among patients of TAPVC of all types, 50% die at 3
months and almost 80% die by the age of 1 year
Asymptomatic at birth
56% symptomatic at 1
st
month of life
Failure at 6 months of age
Severe obstruction pulmonary edema 1
st
few hrs
Cyanosis mild, more with failure & pulm vascular
changes

69. Death – 1
st
few wks/months of life in most neonates
80% death – 1
st
year of life
Survivors of 1
st
few wks of life – ↑pulm bld flow mild
cyanosis, PHTN

50% survive beyond 3 months
Median survival 3wks (obst) & 2.5 months in (non
obstr)
Infracardiac- worse prognosis- 3wks survival

70. MANAGEMENT
Corrective surgery - definitive treatment.
Infants presenting with obstructed TAPVC
represent surgical emergency. They need require
intensive 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.

71. EMERGENCY MEDICAL
MANAGEMENT
Mechanical ventilation.
Correction of Metabolic acidosis.
Inotropic support
Prostaglandin therapy (PGE1)

72. ADDITIONAL INTERVENTIONS INCLUDE
Extracorporeal membrane oxygenation (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.

73. SURGERY
The goal of the surgery is
To create a communication between LA and
the pulmonary venous.
Closure of the anomalous pulmonary
venous connections to systemic circulation
Closure of ASD

74. The surgical approach is via a median sternotomy
and is performed under cardiopulmonary bypass
with circulatory arrest. The surgical procedure
varies depending upon the anatomy of the TAPVC
lesion.
In supra- and infracardiac TAPVC with a common
vertical vein, a normal pulmonary venous
pathway is created by opening and forming an
anastomosis between the pulmonary venous
confluence and the left atrium. The vertical vein is
then ligated and divided.

75. SUPRACARDIAC TAPVC:

76. INFRACARDIAC TAPVC:

77. CARDIAC TAPVC:
In intracardiac TAPVC to the coronary sinus, the
sinus and the partition between the sinus and right
atrium are incised, and connected to the left
atrium.

78. MIXED TYPE TAPVC
The repair of mixed type TAPVC involves a
combination of the above approaches as
dictated by the specific anatomy of the
lesion

79. Recurrent
pulmonary venous
obstruction

80. SURGICAL OUTCOMES
Surgical mortality has decreased from approx. 50%
in 1960 to 5% recently.
˂
 Risk factors for mortality included earlier age at
surgery, hypoplastic/stenotic pulmonary veins,
associated cardiac lesions, postoperative pulmonary
hypertension, postoperative PVO , Small pulmonary
vein and confluence size & Increased total bypass
and circulatory arrest time .

81. SURGICAL OUTCOMES RISK FACTORS

377 patients of operated TAPVC were followed
retrospectively.pulmonary venous obstruction was in 48% of
patients.

83. SURGICAL OUTCOMES IN NEONATES
112 patient were followed retrospectively who were
operated for simple TAPVC in first month of life
from 1973 to 2008.
Preoperative pulmonary venous obstruction in 89
pts (80%).

84. There were 12 (10.7%) early deaths. Significant risk
factors were bypass time 65 minutes and emergent
˃
surgery.
Survival at 20 years was 83.4%.Risk factors for late
death were operative weight 2.5 kg or less and
postoperative pulmonary hypertensive crisis.
Re-operation for recurrent PVO was in 13 patients
(11.9%).

85. SURGICAL OUTCOMES IN INDIA
73 pts, were operated on for TAPVC from Jan 1987 to Oct
1997.
35 patients had obstructed drainage.
Operative mortality was 23.3% (17 out of 73).

87. COMPLICATIONS OF SURGERY
EARLY-
Pulmonary edema
Pulmonary hypertensive crisis
Phrenic nerve damage
Rhythm disorders
LATE-
Pulmonary venous obstruction
Anastomotic stricture
Pulmonary venous stenosis

88. 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.

89. Pulmonary venous obstruction
This is most significant cause of late morbidity and
mortality after corrective surgery.
It develops in 5-15% of patients within first
postoperative year where TAPVC is corrected using
standard technique.
Anastomotic fibrotic strictures, intimal proliferation
and diffuse fibrosis are pathogenic mechanisms.

90. REOPERATION
 Based upon a number of case series, the rate
for reoperation is between 10 and 15 percent
in patients with isolated TAPVC (11 percent) .
 Stenosis of individual pulmonary vein and
surgical anastomosis are the primary reasons
for reoperation.
If restenosis does not occur within one year
after surgical repair, then reoperation is rarely
required.

91. MORBIDITY
Although long-term follow-up data are limited, one
case series reported a poorer perception of health
and school performance in survivors of TAPVC
correction.
 In addition, several case series noted an increased
risk of arrhythmias, especially sinus node
dysfunction .
This may be due to disruption of the conduction
system by the atrial incision used to repair TAPVC.

92. Children with TAPVC should undergo appropriate
surveillance, screening, and or referral for
neurodevelopmental impairment as recommended
in a 2012 scientific statement from the American
Heart Association.

93. follow-up care should be individually planned through
the primary caregiver in collaboration with a pediatric
cardiologist
In the absence of residual pulmonary vein stenosis or
pulmonary hypertension, exercise tolerance is generally
normal in children with repaired TAPVC regardless of
the anatomic subtype.

Physical activities and sports participation should not be
restricted. Infective endocarditis prophylaxis precautions
should be considered within the first six months after
surgical repair, after which it is no longer required.
FOLLOW-UP CARE

94. Periodic screening for arrhythmias is not routinely
recommended in asymptomatic children with
repaired TAPVC.
Because of the known association between
intracardiac surgical repairs and atrial arrhythmias,
however, periodic Holter monitoring may be
considered in asymptomatic adolescents

95. 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.

96. Improved surgical techniques and hospital care
have led to significantly better outcomes of TAPVC
surgery.
Suturless repair is safe and effective method to deal
with post operative pulmonary venous obstruction.

97. DIFFERENTIAL DIAGNOSIS
Non obstructive TAPVC -
Conditions producing high pulmonary flow
with cyanosis like TGA ,Taussig Bing anomaly,
persistent truncus arteriosus and common atrium.
Obstructive type of TAPVC-
Conditions producing PAH without shunt
lesion like congenital mitral stenosis, cor-
triatriatum, pulmonary venous stenosis and
persistent fetal circulation.

98. THANKS