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TOTAL ANOMALOUS PULMONARY VENOUS
CONNECTIONS
Defination
 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.
 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.
TOTAL ANOMALOUS PULMONARY VENOUS CONNECTIONS
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 (Hemodynamic
vise)
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.
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.
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
Total anomalous pulmonary venous connections seminar ppt.
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 smaller sum of pulmonary
vein diameters is associated
with higher surgical
mortality after repair of
TAPVC.
Jenkins KJ et al.J Am Coll Cardiol 22:201-206,1993.
Echocardiography cont.
 Size and orientation (horizontal or vertical) of the pulmonary
venous confluence and its relation with the left atrium are
important for surgical planning.
 Generally,the venous channel in supracardiac TAPVC is best
imaged from the precordial windows,and the venous channel
in infradiaphragmatic TAPVC is best evaluated from the
subcostal view.
Echocardiography cont.
 In supracardiac TAPVC,the site of connection with systemic
vein (most frequently the left innominate vein) is a common
site of narrowing.
 In infradiaphragmatic TAPVC,the most common site of
obstruction is its connection with the portal or hepatic vein.
 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)
Echocardiography cont.
 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.
Echocardiography cont.
 Prenatal diagnosis of TAPVC is important for counselling,
prognosis and planning for delivery at a center expertise in
pediatric cardiology.
 As fetal pulmonary blood flow is less in utero,so to identify
anomalous pulmonary venous connections is difficult.
 If RV and PA are enlarged out of proportion to the LV and aorta
in utero then TAPVC is considered if other causes like CoA,left
sided obstructive lesions,AVM,are excluded.
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
coronary sinus bulging into the
left atrium.
Modified A4C view- Pulmonary
venous confluence (star) draining
into coronary sinus
Echocardiography cont.
Subcostal view showing pulmonary
venous confluence (star) traced to
descending vein (arrow) that
drains into portal vein.
Continuous,turbulant and non-phasic
pulmonary venous flow indicating
pulmonary venous obstruction
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 catheterization
 In obstructive type RV and PA pressures are increased and may
be equal or more than systemic pressure.
 Hence,patients whose systemic arterial saturation is ˃70% and
PAP at or above systemic levels are likely to have significant
pulmonary venous obstruction.
 RAP ≥2 mmHg in excess of LAP is more reliable in predicting a
restrictive interatrial communication as compare to presence
of equal pressures in two atria in predicting nonobstructive
interatrial communication (compliance and filling pressures of
both ventricles are comparable).
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
Differential diagnosis
 Non obstructive TAPVC-
Conditions producing high pulmonary flow with cyanosis
like TGA,Taussitrig Bing anomaly, persistent truncus arteriosus
and common aum.
 Obstructive type of TAPVC-
Conditions producing PAH without shunt lesion like
congenital mitral stenosis,cor-triatriatum,pulmonary venous
stenosis and persistent fetal circulation.
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.
Emergency medical management
 Functions of failing heart augmented with the inotropes and
diuretics.
 Isoproterenol has special merit for inotropic support in
obstructed TAPVC because it has pulmonary vasodilatory
properties (0.1 microgm/kg/min for 24-48 hrs).
 PGE1 infusion given to maintain patency of ductus venosus
to decompress the pulmonary veins in obstructed TAPVC.
Serraf et al.J Thorac Cardiovasc Surg 1991;101:601-606
 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.
Total anomalous pulmonary venous connections seminar ppt.
 Balloon dilatation was unsuccessful in a study by Lock et al.
Lock et al.Circulation 1984;70:457-464
Total anomalous pulmonary venous connections seminar ppt.
Case reports
 9 year old girl f/c supracardiac TAPVC and stenting done in
stenotic innominate vein at age of 7 years.
Michel-Behnke et al. Pediatr Cardiol 23;221-223,2002
Total anomalous pulmonary venous connections seminar ppt.
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
Kirklin and Barratt-Boyes Cardiac Surgery 3rd ed.
Sabiston & Spencer Surgery of the Chest 7th ed.
De leval Surgery for CHD 3rd 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
Patency of vertical veins
 34 pts.less than 3 months of age operated from 1993 to 2000 for
TAPVC were followed.22 pts.(65%) had obstructed venous
drainage.
Total anomalous pulmonary venous connections seminar ppt.
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.
Surgical outcomes risk factors
 377 patients of operated TAPVC were followed retrospectively.pulmonary
venous obstruction was in 48% of patients.
Total anomalous pulmonary venous connections seminar ppt.
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%).
 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%).
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).
Total anomalous pulmonary venous connections seminar ppt.
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.
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.
Calderone CA et al. Ann Thorac Surg 1998;66:1514-20
Lacour-Gayet F. Semin Thorac Cardiovasc Surg 2006;4550
Pulmonary venous obstruction
 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,
-Sutureless repair “neo-atrium”.
Sutureless repair
 A longitudinal incision is made in the posterior pericardium,
underlying pulmonary venous confluence & corresponding left
atrium.
 The left atrium is sutured to the pericardium circumferentially
around the pericardial incision avoiding direct suturing to the
pulmonary veins.
 A “neo-atrium” is created into which pulmonary veins bleed in
a controlled fashion into the left atrium and the left ventricle.
Sutureless repair
 Avoidance of direct suturing to the pulmonary veins minimizes surgery
induced trauma and distortion of pulmonary veins as a result of suturing
related alteration in local geometry.
 Retrospective review of 25 infants who had suturless repair from Oct.2002
to April 2010.
 Kaplan-Meir disease free survival was 96% at 30 days and 84% at 1 year.
Anthony A.et al. Ann Thorac Surg 2011;92:666-72
Total anomalous pulmonary venous connections seminar ppt.
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.
Conclusion
 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.
Thanks

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Total anomalous pulmonary venous connections seminar ppt.

  • 1. TOTAL ANOMALOUS PULMONARY VENOUS CONNECTIONS
  • 2. Defination  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.
  • 3. 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.
  • 4. 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.
  • 5. Incidence cont.  Sex ratio with supradiaphragmatic TAPVC is about equal.  Male predominance (4:1) is seen in infradiaphragmatic type of TAPVC.
  • 6. Associated cardiac anomalies  TGA  TOF  Single ventricle  Truncus arteriosus  Tricuspid atresia  HLHS  CoA  Asplenia or polysplenia
  • 7. 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.
  • 8. 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.
  • 9. 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
  • 10. 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
  • 11. 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
  • 12. 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
  • 13. Embryology cont.  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. TOTAL ANOMALOUS PULMONARY VENOUS CONNECTIONS Edwards JE. Mayo Clin Proc 1953;28:441-452
  • 14. 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.
  • 15. 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)
  • 16. 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
  • 17. 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
  • 18. 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
  • 19. 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
  • 20. 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.
  • 21. 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 (Hemodynamic vise)
  • 22. 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.
  • 23. 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.
  • 24. 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).
  • 25. 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.
  • 26. 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.
  • 27. 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.
  • 28. 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.
  • 29. 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.
  • 30. 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.
  • 31. 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.
  • 32. 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.
  • 33. 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.
  • 34. 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.
  • 35. 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.
  • 36. 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
  • 38. 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
  • 39. 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
  • 40. Echocardiography cont.  A smaller sum of pulmonary vein diameters is associated with higher surgical mortality after repair of TAPVC. Jenkins KJ et al.J Am Coll Cardiol 22:201-206,1993.
  • 41. Echocardiography cont.  Size and orientation (horizontal or vertical) of the pulmonary venous confluence and its relation with the left atrium are important for surgical planning.  Generally,the venous channel in supracardiac TAPVC is best imaged from the precordial windows,and the venous channel in infradiaphragmatic TAPVC is best evaluated from the subcostal view.
  • 42. Echocardiography cont.  In supracardiac TAPVC,the site of connection with systemic vein (most frequently the left innominate vein) is a common site of narrowing.  In infradiaphragmatic TAPVC,the most common site of obstruction is its connection with the portal or hepatic vein.  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)
  • 43. Echocardiography cont.  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.
  • 44. Echocardiography cont.  Prenatal diagnosis of TAPVC is important for counselling, prognosis and planning for delivery at a center expertise in pediatric cardiology.  As fetal pulmonary blood flow is less in utero,so to identify anomalous pulmonary venous connections is difficult.  If RV and PA are enlarged out of proportion to the LV and aorta in utero then TAPVC is considered if other causes like CoA,left sided obstructive lesions,AVM,are excluded.
  • 45. 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.
  • 46. Echocardiography cont. Parasternal LAX with dilated coronary sinus bulging into the left atrium. Modified A4C view- Pulmonary venous confluence (star) draining into coronary sinus
  • 47. Echocardiography cont. Subcostal view showing pulmonary venous confluence (star) traced to descending vein (arrow) that drains into portal vein. Continuous,turbulant and non-phasic pulmonary venous flow indicating pulmonary venous obstruction
  • 48. 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
  • 49. 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
  • 50. 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.
  • 51. Cardiac catheterization  In obstructive type RV and PA pressures are increased and may be equal or more than systemic pressure.  Hence,patients whose systemic arterial saturation is ˃70% and PAP at or above systemic levels are likely to have significant pulmonary venous obstruction.  RAP ≥2 mmHg in excess of LAP is more reliable in predicting a restrictive interatrial communication as compare to presence of equal pressures in two atria in predicting nonobstructive interatrial communication (compliance and filling pressures of both ventricles are comparable).
  • 52. 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.
  • 53. 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.
  • 54. 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.
  • 55. 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
  • 56. Differential diagnosis  Non obstructive TAPVC- Conditions producing high pulmonary flow with cyanosis like TGA,Taussitrig Bing anomaly, persistent truncus arteriosus and common aum.  Obstructive type of TAPVC- Conditions producing PAH without shunt lesion like congenital mitral stenosis,cor-triatriatum,pulmonary venous stenosis and persistent fetal circulation.
  • 57. Complications 1. Congestive cardiac failure 2. Growth and developmental delay 3. Frequent respiratory infections 4. Pulmonary vascular disease 5. Pulmonary edema in obstructive type.
  • 58. Management  Corrective surgery is the definitive treatment.
  • 59. 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
  • 60. 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.
  • 61. Emergency medical management  Functions of failing heart augmented with the inotropes and diuretics.  Isoproterenol has special merit for inotropic support in obstructed TAPVC because it has pulmonary vasodilatory properties (0.1 microgm/kg/min for 24-48 hrs).  PGE1 infusion given to maintain patency of ductus venosus to decompress the pulmonary veins in obstructed TAPVC. Serraf et al.J Thorac Cardiovasc Surg 1991;101:601-606
  • 62.  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.
  • 64.  Balloon dilatation was unsuccessful in a study by Lock et al. Lock et al.Circulation 1984;70:457-464
  • 66. Case reports  9 year old girl f/c supracardiac TAPVC and stenting done in stenotic innominate vein at age of 7 years. Michel-Behnke et al. Pediatr Cardiol 23;221-223,2002
  • 68. 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 Kirklin and Barratt-Boyes Cardiac Surgery 3rd ed. Sabiston & Spencer Surgery of the Chest 7th ed. De leval Surgery for CHD 3rd ed.
  • 69. 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.
  • 70. Posterior approach  Posterior approach is applicable to all types of TAPVC.
  • 71. 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.
  • 72. 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.
  • 74. 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.
  • 75. 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.
  • 76. Repair of Cardiac type TAPVC
  • 77. 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
  • 78. Patency of vertical veins  34 pts.less than 3 months of age operated from 1993 to 2000 for TAPVC were followed.22 pts.(65%) had obstructed venous drainage.
  • 80. 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.
  • 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. Calderone CA et al. Ann Thorac Surg 1998;66:1514-20 Lacour-Gayet F. Semin Thorac Cardiovasc Surg 2006;4550
  • 90. Pulmonary venous obstruction  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, -Sutureless repair “neo-atrium”.
  • 91. Sutureless repair  A longitudinal incision is made in the posterior pericardium, underlying pulmonary venous confluence & corresponding left atrium.  The left atrium is sutured to the pericardium circumferentially around the pericardial incision avoiding direct suturing to the pulmonary veins.  A “neo-atrium” is created into which pulmonary veins bleed in a controlled fashion into the left atrium and the left ventricle.
  • 92. Sutureless repair  Avoidance of direct suturing to the pulmonary veins minimizes surgery induced trauma and distortion of pulmonary veins as a result of suturing related alteration in local geometry.
  • 93.  Retrospective review of 25 infants who had suturless repair from Oct.2002 to April 2010.  Kaplan-Meir disease free survival was 96% at 30 days and 84% at 1 year. Anthony A.et al. Ann Thorac Surg 2011;92:666-72
  • 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. Conclusion  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.