This document provides information on total anomalous pulmonary venous connection (TAPVC), including its definition, history, classification, embryology, pathophysiology, clinical manifestations, and findings. TAPVC is an anomaly where the pulmonary veins do not connect to the left atrium and instead connect directly to systemic veins. It can be classified based on the site of connection and presence of obstruction. Clinical presentation depends on whether there is pulmonary venous obstruction, ranging from asymptomatic to respiratory distress and death in infants.

1. Dr. Rezaul Hayat
Phase B Resident
Pediatric Cardiology, BSMMU
Total Anomalous Pulmonary
Venous Connection

2. TAPVC
 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 into
the right atrium (TAPVD)

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

4. Incidence
 The reported incidence for TAPVC ranges from
0.4 to 2 percent
 few studies have demonstrated higher incidence
in males especially in the infracardiac type
 There is a marked male preponderance in TAPVC
to the portal vein
 no sex prevalence in the other sites of connection

5. Genetics
 there is no known genetic pattern of transmission
of TAPVC
 a possible association with exposure to lead,
paint or paint-stripping chemicals, and pesticides

6. Associations
 Often TAPVC occurs as an isolated lesion except for
the associated inter-atrial septal defect
 The presence of an interatrial communication is
necessary to sustain life; therefore, an ASD or patent
foramen ovale is considered part of the complex. Also,
the young age of the patients makes the presence of a
PDA usual, and this is not considered a complicating
defect
 The non-cardiac conditions associated with TAPVC
are asplenia or polysplenia heterotaxy syndromes
 However, it is known to be associated with other
cardiac malformations like transposition of the great
arteries, pulmonary atresia, truncus arteriosus,
atrioventricular septal defect and single ventricle

7. Embryology

8. Embryology
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

9. Embryology
 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. Embryology
 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. Embryology
 Atresia of the common pulmonary vein andcor
triatriatum.

12. Anomalous Connection
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)

13. Classification
A number of classification schemes for describing
the different types of TAPVC have been proposed
1. Darlings classification
2. Smiths classification
3. Burroughs and Edwards classification
4. Neills classification - Based on
embryologic
basis

14. Smith et.al classification
1. Supradiaphragmatic (without pulmonary
venous
obstruction)
2. Infradiaphragmatic (with pulmonary venous
obstruction)

15. Burroughs and Edwards
classification with prognostic implications based on
the length of the anomalous channel
1. Long
2. Intermediate
3. short

16. Darling et al. classification (1957)
• Type 1 - Supracardiac type
• Type 2 - Cardiac type
• Type 3 - Infracardiac type
• Type 4 - Mixed type

17. Darling et al. classification (1957)

18. Comparison of Anatomic Site of Connection of
TAPVC and TAPVD in Three Autopsy Series

19. Anatomic Sites of Obstruction
The presence of an obstructive lesion in the
anomalous
pulmonary venous channel profoundly influences
the
hemodynamic state and clinical features
1. Obstruction at the Interatrial Septum
Burroughs and Edwards clearly related
longevity in TAPVC to the size of the ASD.
Those patients with large defects survived
longer than did with restricted interatrial openings.

20. 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.

21. Obstruction in the Anomalous Venous
Channel
 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

22. Pathophysiology
 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

23. Pathophysiology
 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

24. Pathophysiology
 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

25. Pathophysiology
 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.

26. 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.

27. TAPVC without Pulmonary Venous Obstruction
 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.

28. TAPVC without Pulmonary Venous Obstruction
 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.

29. 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.

30. Clinical Menifestations
 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.
 Thus, the manifestations may be divided
according to whether pulmonary venous
obstruction is absent or present.

31. 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

32. Clinical Findings
APVC without Pulmonary Venous Obstruction
 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 .

33. TAPVC without Pulmonary Venous Obstruction
 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.

34. 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.
 Once symptoms began- rapid progression to
dyspnea , feeding difficulties and
cardiorespiratory failure.
 Age at death ranged from 2 days to 4.5 months .

35. TAPVC with Pulmonary Venous
Obstruction
 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 itexits through the esophageal hiatus

36. Clinical Findings
TAPVC with Pulmonary Venous Obstruction
 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.

37. ECG
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.

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

39. 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.

40. X RayTAPVC 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

41. X ray
TAPVC with pulmonary
venous obstruction
 Diffuse reticular
pattern
 Cardiac size is normal
 Kerley B lines may be
present
 This pattern also seen
in other causes of
pulmonary venous
obstruction.

42. Echocardiography
 Echocardiography is the cornerstone
investigation for diagnosis of TAPVC.
 The accuracy of echocardiography in diagnosing
TAPVC is 100%.
 The key to the diagnosis of TAPVC is identifying
the anatomic connections of all four pulmonary
veins

43.  Cardiac total anomalous pulmonary venous
connection (type II). Gray-scale and color Doppler
echocardiographic images show common
pulmonary vein confluence (PVC) posterior to heart
that empties into the right atrium (RA).

44. Suprasternal: A. and apical B
echocardiographic planes
demonstrating a pulmonary
venous confluence (PVC)
posterior to the left atrium
(LA) that is being drained by
a vertical vein (VV). The
apical four-chamber view; B.
More clearly shows the VV
arising from the PVC and
heading cephalad.

45.  A. Infracardiac total anomalous pulmonary venous
connection (type III). Common pulmonary vein
confluence is seen posterior to the heart; B. Right
and left pulmonary veins form a confluence posterior
to the left atrium. Descending common pulmonary
vein arising from the confluence joins the portal vein.

46.  Doppler
echocardiogram of a
patient with mixed
TAPVC showing left
upper pulmonary vein
(LUPV) entering the
left atrial appendage
(LAA)

47. CT
 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.

48. MRI
 MRI 3D reconstructed
image showing mixed
total anomalous
pulmonary venous
connection (type IV).

49. 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%).

50. Cardiac Catheterization
without Pulmonary
Venous Obstruction
with 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
 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.

51. Cardiac Catheterization
without Pulmonary
Venous Obstruction
Catheter angiography of totally
anomalous pulmonary venous
connection. Levophase of a right
pulmonary artery angiogram
showing supracardiac TAPVC to
the left innominate vein.
 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

52. Natural History
 Among patients of TAPVC of all types,50% die at
3 months and almost 80% die by the age of 1
year.
 Patients withinadequate interatrial communication
had an even poorer prognosis
 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.

53. Management
 Corrective surgery is the
definitive treatment.

54. Management
 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.

55. Management
Unobstructed Type Obstructed Type
 Compensating right
Heart failure
–Inotropic
support
–Diuresis
 TRUE SURGICAL
EMERGENCY
 Limited role
 Intubation and
hyperventilation
 Correction of acidosis

56. Interrupt the Connections
with the systemic venous
circulation
close the atrial septal
defect
An
unobstructed
communicatio
n between the
Pulmonary
venous
confluence
and the left
atrium
Surgery

57. Goal Of Surgery
 To create a communication between LA and the
pulmonary venous.
 Closure of the anomalous pulmonary venous
connections to systemic circulation
 Closure of ASD

58.  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. Supracardiac
TAPVC

59.  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. Infracardiac
TAPVC

60. 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.
cardiac TAPVC

61. The repair of mixed type TAPVC involves a
combination of the above approaches as dictated
by the specific anatomy of the
lesion Mixed Type
TAPVC

62. Complications of Surgery
Early Late
 Pulmonary edema
 Pulmonary
hypertensive crisis
 Phrenic nerve
damage
 Rhythm disorders
 Pulmonary venous
obstruction
 Anastomotic stricture
 Pulmonary venous
stenosis

63. 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.
 Various surgical techniques-
-Revision of common pulmonary vein to LA
anastomosis,
-Patch angioplasty of stenotic pulmonary veins,
-Suturing of individual pulmonary veins directly to
the LA,

64. Outcome of Surgery
 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.

65. 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.
 Improved surgical techniques and hospital care
have led to significantly better outcomes of
TAPVC surgery.
 Suturless repair is safe and effective method to

66. Thank You