Total anomalous pulmonary venous connections seminar ppt.


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Total anomalous pulmonary venous connections seminar ppt.

  2. 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. 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;346,1798  MULLER-first successful open repair in 1951.
  4. 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. 5. Incidence cont.  Sex ratio with supradiaphragmatic TAPVC is about equal.  Male predominance (4:1) is seen in infradiaphragmatic type of TAPVC.
  6. 6. Associated cardiac anomalies  TGA  TOF  Single ventricle  Truncus arteriosus  Tricuspid atresia  HLHS  CoA  Asplenia or polysplenia
  7. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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 Lab Invest.1957;6:44-64 Karamlou T Circulation 2007;115:1591-1598
  17. 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. 18. Classification cont.  Smith classification 1. Supradiaphragmatic (without pulmonary venous obstruction) 2. Infradiaphragmatic (with pulmonary venous obstruction) Smith Am J Dis Child 1961;101:41-51
  19. 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 Thorac Surg 69(suppl):S56,2000
  20. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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 Am J Roentgenol.86;561,1961)  Newborns with infradiaphragmatic connections and asplenia may have major esophageal varices.
  30. 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. 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. 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. 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. 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. 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. 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
  37. 37. 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
  38. 38. 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
  39. 39. 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.
  40. 40. 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.
  41. 41. 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)
  42. 42. 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.
  43. 43. 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.
  44. 44. 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.
  45. 45. Echocardiography cont. Parasternal LAX with dilated coronary sinus bulging into the left atrium. Modified A4C view- Pulmonary venous confluence (star) draining into coronary sinus
  46. 46. 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
  47. 47. 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
  48. 48. 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
  49. 49. Cardiac catheterization  Diagnostic catheterization is rarely performed for TAPVC diagnosis.  Cardiac 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.
  50. 50. 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).
  51. 51. 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.
  52. 52. 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.
  53. 53. 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.
  54. 54. 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
  55. 55. 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.
  56. 56. Complications 1. Congestive cardiac failure 2. Growth and developmental delay 3. Frequent respiratory infections 4. Pulmonary vascular disease 5. Pulmonary edema in obstructive type.
  57. 57. Management  Corrective surgery is the definitive treatment.
  58. 58. 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
  59. 59. 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.
  60. 60. 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
  61. 61.  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.
  62. 62.  Balloon dilatation was unsuccessful in a study by Lock et al. Lock et al.Circulation 1984;70:457-464
  63. 63. 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
  64. 64. 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.
  65. 65. 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.
  66. 66. Posterior approach  Posterior approach is applicable to all types of TAPVC.
  67. 67. 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.
  68. 68. 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.
  69. 69. Right atrial approach
  70. 70. 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.
  71. 71. 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.
  72. 72. Repair of Cardiac type TAPVC
  73. 73. 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
  74. 74. 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.
  75. 75. 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.
  76. 76. Surgical outcomes risk factors  377 patients of operated TAPVC were followed retrospectively.pulmonary venous obstruction was in 48% of patients.
  77. 77. 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%).
  78. 78.  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%).
  79. 79. 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).
  80. 80. Complications of surgery EARLY-  Pulmonary edema  Pulmonary hypertensive crisis  Phrenic nerve damage  Rhythm disorders LATE-  Pulmonary venous obstruction  Anastomotic stricture  Pulmonary venous stenosis
  81. 81.  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.
  82. 82. 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
  83. 83. 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”.
  84. 84. 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.
  85. 85. 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.
  86. 86.  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 al. Ann Thorac Surg 2011;92:666-72
  87. 87. 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.
  88. 88. 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.
  89. 89. Thanks